-
1
-
-
77956841570
-
Mechanism of bile acid biosynthesis in mammalian liver
-
Danielsson H, Sjovall J, eds., Amsterdam: BV Elsevier Science Publishers
-
Bjorkhem I. Mechanism of bile acid biosynthesis in mammalian liver. In: Danielsson H, Sjovall J, eds. Sterols and bile acids. Amsterdam: BV Elsevier Science Publishers, 1985: 231–77.
-
(1985)
Sterols and Bile Acids
, pp. 231-277
-
-
Bjorkhem, I.1
-
3
-
-
0023233769
-
Inborn errors of bile acid synthesis
-
Setchell KDR, Street JM. Inborn errors of bile acid synthesis. Semin Liver Dis 1987;7: 85–99.
-
(1987)
Semin Liver Dis
, vol.7
, pp. 85-99
-
-
Setchell, K.1
Street, J.M.2
-
4
-
-
0000078354
-
Disorders of bile acid synthesis and metabolism
-
Walker WA, Durie PR, Hamilton JR, et al., Pathophysiology, diagnosis, management. Toronto/Philadelphia: BC Decker, Inc
-
Setchell KDR. Disorders of bile acid synthesis and metabolism. In: Walker WA, Durie PR, Hamilton JR, et al., eds. Pediatric gastrointestinal disease. Pathophysiology, diagnosis, management. Toronto/Philadelphia: BC Decker, Inc., 1996.
-
(1996)
Pediatric Gastrointestinal Disease
-
-
Setchell, K.1
-
6
-
-
0026606455
-
A proposed nomenclature for bile acids
-
Hofmann AF, Sjovall J, Kurz G, et al. A proposed nomenclature for bile acids. J Lipid Res 1992;33: 599–604.
-
(1992)
J Lipid Res
, vol.33
, pp. 599-604
-
-
Hofmann, A.F.1
Sjovall, J.2
Kurz, G.3
-
7
-
-
0025248645
-
Regulation and ligand-binding specificities of two sex-specific bile acid-binding proteins of rat liver cytosol
-
Le Blanc GA, Waxman DJ. Regulation and ligand-binding specificities of two sex-specific bile acid-binding proteins of rat liver cytosol. J Biol Chem 1990;265: 5654–61.
-
(1990)
J Biol Chem
, vol.265
, pp. 5654-5661
-
-
Le Blanc, G.A.1
Waxman, D.J.2
-
8
-
-
0025140903
-
Identification of cytosolic and microsomal bile acid-binding proteins in rat ileal enterocytes
-
Lin MC, Kramer W, Wilson FA. Identification of cytosolic and microsomal bile acid-binding proteins in rat ileal enterocytes. J Biol Chem 1990;265: 14986–95.
-
(1990)
J Biol Chem
, vol.265
, pp. 14986-14995
-
-
Lin, M.C.1
Kramer, W.2
Wilson, F.A.3
-
9
-
-
0037790917
-
The enzymes, regulation, and genetics of bile acid synthesis
-
Russell DW. The enzymes, regulation, and genetics of bile acid synthesis. Annu Rev Biochem 2003;72: 137–74.
-
(2003)
Annu Rev Biochem
, vol.72
, pp. 137-174
-
-
Russell, D.W.1
-
10
-
-
0024442549
-
Molecular cloning of c dna for cholesterol 7a-hydroxylase from rat liver microsomes. Nucleotide sequence and expression
-
Noshiro M, Nishimoto M, Morohashi K, Okuda K. Molecular cloning of c DNA for cholesterol 7a-hydroxylase from rat liver microsomes. Nucleotide sequence and expression. FEBS Lett 1989;257: 97–100.
-
(1989)
FEBS Lett
, vol.257
, pp. 97-100
-
-
Noshiro, M.1
Nishimoto, M.2
Morohashi, K.3
Okuda, K.4
-
11
-
-
0025299346
-
Molecular cloning and sequence analysis of c dna encoding human cholesterol 7a-hydroxylase
-
Noshiro M, Okuda K. Molecular cloning and sequence analysis of c DNA encoding human cholesterol 7a-hydroxylase. FEBS Lett 1990;268: 137–40.
-
(1990)
FEBS Lett
, vol.268
, pp. 137-140
-
-
Noshiro, M.1
Okuda, K.2
-
12
-
-
0025269939
-
Cloning and regulation of cholesterol 7a-hydroxylase, the rate-limiting enzyme in bile acid biosynthesis
-
Jelinek DF, Andersson S, Slaughter CA, Russell DW. Cloning and regulation of cholesterol 7a-hydroxylase, the rate-limiting enzyme in bile acid biosynthesis. J Biol Chem 1990;265: 8190–7.
-
(1990)
J Biol Chem
, vol.265
, pp. 8190-8197
-
-
Jelinek, D.F.1
Ersson, S.2
Slaughter, C.A.3
Russell, D.W.4
-
13
-
-
0026088436
-
The expression of a catalytically active cholesterol 7a-hydroxylase cytochrome p450 in escherichia coli
-
Li YC, Chiang JY. The expression of a catalytically active cholesterol 7a-hydroxylase cytochrome P450 in Escherichia coli. J Biol Chem 1991;266: 19186–91.
-
(1991)
J Biol Chem
, vol.266
, pp. 19186-19191
-
-
Li, Y.C.1
Chiang, J.Y.2
-
14
-
-
0019522805
-
Physiologic cholestasis: Elevation of the primary serum bile acid concentrations in normal infants
-
Suchy FJ, Balistreri WF, Heubi JE, et al. Physiologic cholestasis: elevation of the primary serum bile acid concentrations in normal infants. Gastroenterology 1981;80: 1037–41.
-
(1981)
Gastroenterology
, vol.80
, pp. 1037-1041
-
-
Suchy, F.J.1
Balistreri, W.F.2
Heubi, J.E.3
-
15
-
-
0019362760
-
Postnatal physiologic hypercholemia in both premature and full-term infants
-
Barnes S, Berkowitz G, Hirschowitz BI, et al. Postnatal physiologic hypercholemia in both premature and full-term infants. J Clin Invest 1981;68: 775–82.
-
(1981)
J Clin Invest
, vol.68
, pp. 775-782
-
-
Barnes, S.1
Berkowitz, G.2
Hirschowitz, B.I.3
-
16
-
-
0014112259
-
Effect of biliary drainage on individual reactions in the conversion of cholesterol to taurochlic acid. Bile acids and steroids 180
-
Danielsson H, Einarsson K, Johansson G. Effect of biliary drainage on individual reactions in the conversion of cholesterol to taurochlic acid. Bile acids and steroids 180. Eur J Biochem 1967;2: 44–9.
-
(1967)
Eur J Biochem
, vol.2
, pp. 44-49
-
-
Danielsson, H.1
Einarsson, K.2
Johansson, G.3
-
17
-
-
0014288125
-
7a-hydroxylation of cholestanol by rat liver microsomes
-
Shefer S, Hauser S, Mosbach EH. 7a-Hydroxylation of cholestanol by rat liver microsomes. J Lipid Res 1968;9: 328–33.
-
(1968)
J Lipid Res
, vol.9
, pp. 328-333
-
-
Shefer, S.1
Hauser, S.2
Mosbach, E.H.3
-
18
-
-
0014841283
-
Biochemical site of regulation of bile acid biosynthesis in the rat
-
Shefer S, Hauser S, Bekersky I, Mosbach EH. Biochemical site of regulation of bile acid biosynthesis in the rat. J Lipid Res 1970;11: 404–11.
-
(1970)
J Lipid Res
, vol.11
, pp. 404-411
-
-
Shefer, S.1
Hauser, S.2
Bekersky, I.3
Mosbach, E.H.4
-
21
-
-
0025828283
-
Regulation of cholesterol 7a- hydroxylase by hepatic 7a-hydroxylated bile acid flux and newly synthesized cholesterol supply
-
Shefer S, Nguyen LB, Salen G, et al. Regulation of cholesterol 7a- hydroxylase by hepatic 7a-hydroxylated bile acid flux and newly synthesized cholesterol supply. J Biol Chem 1991;266: 2693–6.
-
(1991)
J Biol Chem
, vol.266
, pp. 2693-2696
-
-
Shefer, S.1
Nguyen, L.B.2
Salen, G.3
-
22
-
-
0023753194
-
Effects of bile acid administration on bile acid synthesis and its circadian rhythm in man
-
Pooler PA, Duane WC. Effects of bile acid administration on bile acid synthesis and its circadian rhythm in man. Hepatology 1988;8: 1140–6.
-
(1988)
Hepatology
, vol.8
, pp. 1140-1146
-
-
Pooler, P.A.1
Duane, W.C.2
-
23
-
-
0010444141
-
Effect of cholestyramine, a bileacid binding polymer on plasma cholesterol and fecal bile acid excretion in the rat
-
Huff JW, Gilfillan JL, Hunt VM. Effect of cholestyramine, a bileacid binding polymer on plasma cholesterol and fecal bile acid excretion in the rat. Proc Soc Exp Biol Med 1963;114: 352–5.
-
(1963)
Proc Soc Exp Biol Med
, vol.114
, pp. 352-355
-
-
Huff, J.W.1
Gilfillan, J.L.2
Hunt, V.M.3
-
24
-
-
0025894157
-
Regulation of cholesterol 7a-hydroxylase m rna and transcriptional activity by taurocholate and cholesterol in the chronic biliary diverted rat
-
Pandak WM, Li YC, Chiang JY, et al. Regulation of cholesterol 7a-hydroxylase m RNA and transcriptional activity by taurocholate and cholesterol in the chronic biliary diverted rat. J Biol Chem 1991;266: 3416–21.
-
(1991)
J Biol Chem
, vol.266
, pp. 3416-3421
-
-
Pandak, W.M.1
Li, Y.C.2
Chiang, J.Y.3
-
25
-
-
0025120211
-
Regulation of themevalonate pathway
-
Goldstein JL, Brown MS. Regulation of themevalonate pathway. Nature 1990;343: 425–30.
-
(1990)
Nature
, vol.343
, pp. 425-430
-
-
Goldstein, J.L.1
Brown, M.S.2
-
26
-
-
0015371179
-
Relationship between diurnal variations in biosynthesis of cholesterol and bile acids
-
Danielsson H. Relationship between diurnal variations in biosynthesis of cholesterol and bile acids. Steroids 1972;20: 63–72.
-
(1972)
Steroids
, vol.20
, pp. 63-72
-
-
Danielsson, H.1
-
27
-
-
0015427501
-
Diurnal variation in the activity of cholesterol 7a-hydroxylase in the livers of fed and fasted rats
-
Mitropoulos KA, Balasubramaniam S, Gibbons GF, Reeves BE. Diurnal variation in the activity of cholesterol 7a-hydroxylase in the livers of fed and fasted rats. FEBS Lett 1972;27: 203–6.
-
(1972)
FEBS Lett
, vol.27
, pp. 203-206
-
-
Mitropoulos, K.A.1
Balasubramaniam, S.2
Gibbons, G.F.3
Reeves, B.E.4
-
28
-
-
0021062922
-
Regulation of bile acid synthesis in man. Presence of a diurnal rhythm
-
Duane WC, Levitt DG, Mueller SM, Behrens JC. Regulation of bile acid synthesis in man. Presence of a diurnal rhythm. J Clin Invest 1983;72: 1930–6.
-
(1983)
J Clin Invest
, vol.72
, pp. 1930-1936
-
-
Duane, W.C.1
Levitt, D.G.2
Mueller, S.M.3
Behrens, J.C.4
-
29
-
-
0021891305
-
Purification from rabbit and rat liver of cytochromes p-450 involved in bile acid biosynthesis
-
Andersson S, Bostrom H, Danielsson H, Wikvall K. Purification from rabbit and rat liver of cytochromes P-450 involved in bile acid biosynthesis. Methods Enzymol 1985;111: 364–77.
-
(1985)
Methods Enzymol
, vol.111
, pp. 364-377
-
-
Andersson, S.1
Bostrom, H.2
Danielsson, H.3
Wikvall, K.4
-
30
-
-
0031444340
-
Hormonal regulation of cholesterol 7a-hydroxylase specific activity, m rna levels, and transcriptional activity in vivo in the rat
-
Pandak WM, Heuman DM, Redford K, et al. Hormonal regulation of cholesterol 7a-hydroxylase specific activity, m RNA levels, and transcriptional activity in vivo in the rat. J Lipid Res 1997;38: 2483–91.
-
(1997)
J Lipid Res
, vol.38
, pp. 2483-2491
-
-
Pandak, W.M.1
Heuman, D.M.2
Redford, K.3
-
31
-
-
0033590121
-
Expression of the human cholesterol 7a-hydroxylase gene in transgenic mice
-
Goodart SA, Huynh C, Chen W, et al. Expression of the human cholesterol 7a-hydroxylase gene in transgenic mice. Biochem Biophys Res Commun 1999;266: 454–9.
-
(1999)
Biochem Biophys Res Commun
, vol.266
, pp. 454-459
-
-
Goodart, S.A.1
Huynh, C.2
Chen, W.3
-
32
-
-
0031751631
-
Transcriptional activation of the cholesterol 7a-hydroxylase gene (Cyp7a) by nuclear hormone receptors
-
Crestani M, Sadeghpour A, Stroup D, et al. Transcriptional activation of the cholesterol 7a-hydroxylase gene (CYP7A) by nuclear hormone receptors. J Lipid Res 1998;39: 2192–200.
-
(1998)
J Lipid Res
, vol.39
, pp. 2192-2200
-
-
Crestani, M.1
Sadeghpour, A.2
Stroup, D.3
-
33
-
-
0030987347
-
Growth hormone and bile acid synthesis. Key role for the activity of hepatic microsomal cholesterol 7a-hydroxylase in the rat
-
Rudling M, Parini P, Angelin B. Growth hormone and bile acid synthesis. Key role for the activity of hepatic microsomal cholesterol 7a-hydroxylase in the rat. J Clin Invest 1997;99: 2239–45.
-
(1997)
J Clin Invest
, vol.99
, pp. 2239-2245
-
-
Rudling, M.1
Parini, P.2
Angelin, B.3
-
34
-
-
0030773526
-
Quantitative estimations of the contribution of different bile acid pathways to total bile acid synthesis in the rat [see comments]
-
Vlahcevic ZR, Stravitz RT, Heuman DM, et al. Quantitative estimations of the contribution of different bile acid pathways to total bile acid synthesis in the rat [see comments]. Gastroenterology 1997;113: 1949–57.
-
(1997)
Gastroenterology
, vol.113
, pp. 1949-1957
-
-
Vlahcevic, Z.R.1
Stravitz, R.T.2
Heuman, D.M.3
-
35
-
-
0029666290
-
Disruption of cholesterol 7a-hydroxylase gene in mice. Ii. bile acid deficiency is overcome by induction of oxysterol 7a-hydroxylase
-
Schwarz M, Lund EG, Setchell KDR, et al. Disruption of cholesterol 7a-hydroxylase gene in mice. II. Bile acid deficiency is overcome by induction of oxysterol 7a-hydroxylase. J Biol Chem 1996;271: 18024–31.
-
(1996)
J Biol Chem
, vol.271
, pp. 18024-18031
-
-
Schwarz, M.1
Lund, E.G.2
Setchell, K.3
-
36
-
-
0031595674
-
Cholesterol 7-hydroxylase knockout mouse: A model for monohydroxy bile acid-related neonatal cholestasis
-
Arnon R, Yoshimura T, Reiss A, et al. Cholesterol 7-hydroxylase knockout mouse: a model for monohydroxy bile acid-related neonatal cholestasis. Gastroenterology 1998;115: 1223–8.
-
(1998)
Gastroenterology
, vol.115
, pp. 1223-1228
-
-
Arnon, R.1
Yoshimura, T.2
Reiss, A.3
-
37
-
-
0029666289
-
Disruption of cholesterol 7a-hydroxylase gene in mice. I. postnatal lethality reversed by bile acid and vitamin supplementation
-
Ishibashi S, Schwarz M, Frykman PK, et al. Disruption of cholesterol 7a-hydroxylase gene in mice. I. Postnatal lethality reversed by bile acid and vitamin supplementation. J Biol Chem 1996;271: 18017–23.
-
(1996)
J Biol Chem
, vol.271
, pp. 18017-18023
-
-
Ishibashi, S.1
Schwarz, M.2
Frykman, P.K.3
-
38
-
-
0030845921
-
Identification and characterization of a mouse oxysterol 7a-hydroxylase c dna
-
Schwarz M, Lund EG, Lathe R, et al. Identification and characterization of a mouse oxysterol 7a-hydroxylase c DNA. J Biol Chem 1997;272: 23995–4001.
-
(1997)
J Biol Chem
, vol.272
, pp. 23995-24001
-
-
Schwarz, M.1
Lund, E.G.2
Lathe, R.3
-
39
-
-
0032211865
-
Identification of a new inborn error in bile acid synthesis: Mutation of the oxysterol 7a-hydroxylase gene causes severe neonatal liver disease
-
Setchell KDR, Schwarz M, O’Connell NC, et al. Identification of a new inborn error in bile acid synthesis: mutation of the oxysterol 7a-hydroxylase gene causes severe neonatal liver disease. J Clin Invest 1998;102: 1690–703.
-
(1998)
J Clin Invest
, vol.102
, pp. 1690-1703
-
-
Setchell, K.1
Schwarz, M.2
O’Connell, N.C.3
-
40
-
-
0033591264
-
Seeking ligands for lonely orphan receptors [comment]
-
Gustafsson JA. Seeking ligands for lonely orphan receptors [comment]. Science 1999;284: 1285–6.
-
(1999)
Science
, vol.284
, pp. 1285-1286
-
-
Gustafsson, J.A.1
-
41
-
-
0033612369
-
Nuclear orphan receptors control cholesterol catabolism
-
Russell DW. Nuclear orphan receptors control cholesterol catabolism. Cell 1999;97: 539–42.
-
(1999)
Cell
, vol.97
, pp. 539-542
-
-
Russell, D.W.1
-
42
-
-
0033617520
-
Orphan nuclear receptors: Shifting endocrinology into reverse
-
Kliewer SA, Lehmann JM, Willson TM. Orphan nuclear receptors: shifting endocrinology into reverse. Science 1999;284: 757–60.
-
(1999)
Science
, vol.284
, pp. 757-760
-
-
Kliewer, S.A.1
Lehmann, J.M.2
Willson, T.M.3
-
43
-
-
0034793718
-
Chemical genomics: Functional analysis of orphan nuclear receptors in the regulation of bile acid metabolism
-
Willson TM, Jones SA, Moore JT, Kliewer SA. Chemical genomics: functional analysis of orphan nuclear receptors in the regulation of bile acid metabolism. Med Res Rev 2001;21: 513–22.
-
(2001)
Med Res Rev
, vol.21
, pp. 513-522
-
-
Willson, T.M.1
Jones, S.A.2
Moore, J.T.3
Kliewer, S.A.4
-
44
-
-
0036668184
-
Bile acid regulation of gene expression: Roles of nuclear hormone receptors
-
Chiang JY. Bile acid regulation of gene expression: roles of nuclear hormone receptors. Endocr Rev 2002;23: 443–63.
-
(2002)
Endocr Rev
, vol.23
, pp. 443-463
-
-
Chiang, J.Y.1
-
45
-
-
0037340125
-
Differential regulation of rat and human cyp7a1 by the nuclear oxysterol receptor liver x receptor-alpha
-
Goodwin B, Watson MA, Kim H, et al. Differential regulation of rat and human CYP7A1 by the nuclear oxysterol receptor liver X receptor-alpha. Mol Endocrinol 2003;17: 386–94.
-
(2003)
Mol Endocrinol
, vol.17
, pp. 386-394
-
-
Goodwin, B.1
Watson, M.A.2
Kim, H.3
-
46
-
-
22944467490
-
Fxr, a therapeutic target for bile acid and lipid disorders
-
Westin S, Heyman RA, Martin R. FXR, a therapeutic target for bile acid and lipid disorders. Mini Rev Med Chem 2005;5: 719–27.
-
(2005)
Mini Rev Med Chem
, vol.5
, pp. 719-727
-
-
Westin, S.1
Heyman, R.A.2
Martin, R.3
-
48
-
-
0033536005
-
Cpf: An orphan nuclear receptor that regulates liver-specific expression of the human cholesterol 7a-hydroxylase gene
-
Nitta M, Ku S, Brown C, et al. CPF: an orphan nuclear receptor that regulates liver-specific expression of the human cholesterol 7a-hydroxylase gene. Proc Natl Acad Sci US A 1999;96: 6660–5.
-
(1999)
Proc Natl Acad Sci US A
, vol.96
, pp. 6660-6665
-
-
Nitta, M.1
Ku, S.2
Brown, C.3
-
49
-
-
0033637121
-
Aregulatory cascade of the nuclear receptors fxr, shp-1, and lrh-1 represses bile acid biosynthesis
-
Goodwin B, Jones SA, Price RR, et al. Aregulatory cascade of the nuclear receptors FXR, SHP-1, and LRH-1 represses bile acid biosynthesis. Mol Cell 2000;6: 517–26.
-
(2000)
Mol Cell
, vol.6
, pp. 517-526
-
-
Goodwin, B.1
Jones, S.A.2
Price, R.R.3
-
51
-
-
9944265844
-
Regulatory network of lipid-sensing nuclear receptors: Roles for car, pxr, lxr, and fxr
-
Handschin C, Meyer UA. Regulatory network of lipid-sensing nuclear receptors: roles for CAR, PXR, LXR, and FXR. Arch Biochem Biophys 2005;433: 387–96.
-
(2005)
Arch Biochem Biophys
, vol.433
, pp. 387-396
-
-
Handschin, C.1
Meyer, U.A.2
-
52
-
-
0029805887
-
An oxysterol signalling pathway mediated by the nuclear receptor lxr alpha
-
Janowski BA, Willy PJ, Devi TR, et al. An oxysterol signalling pathway mediated by the nuclear receptor LXR alpha. Nature 1996;383: 728–31.
-
(1996)
Nature
, vol.383
, pp. 728-731
-
-
Janowski, B.A.1
Willy, P.J.2
Devi, T.R.3
-
53
-
-
0030885520
-
The orphan nuclear receptor lxralpha is positively and negatively regulated by distinct products of mevalonate metabolism
-
Forman BM, Ruan B, Chen J, et al. The orphan nuclear receptor LXRalpha is positively and negatively regulated by distinct products of mevalonate metabolism. Proc Natl Acad Sci U S A 1997;94: 10588–93.
-
(1997)
Proc Natl Acad Sci U S A
, vol.94
, pp. 10588-10593
-
-
Forman, B.M.1
Ruan, B.2
Chen, J.3
-
54
-
-
0032169485
-
The humanorphan nuclear receptor pxr is activated by compounds that regulate cyp3a4 gene expression and cause drug interactions
-
Lehmann JM, Mc Kee DD, Watson MA, et al. The humanorphan nuclear receptor PXR is activated by compounds that regulate CYP3A4 gene expression and cause drug interactions. J Clin Invest 1998;102: 1016–23.
-
(1998)
J Clin Invest
, vol.102
, pp. 1016-1023
-
-
Lehmann, J.M.1
Mc Kee, D.D.2
Watson, M.A.3
-
55
-
-
0033591297
-
Identification of a nuclear receptor for bile acids [see comments]
-
Makishima M, Okamoto AY, Repa JJ, et al. Identification of a nuclear receptor for bile acids [see comments]. Science 1999; 284: 1362–5.
-
(1999)
Science
, vol.284
, pp. 1362-1365
-
-
Makishima, M.1
Okamoto, A.Y.2
Repa, J.J.3
-
56
-
-
0033591387
-
Bile acids: Natural ligands for an orphan nuclear receptor [see comments]
-
Parks DJ, Blanchard SG, Bledsoe RK, et al. Bile acids: natural ligands for an orphan nuclear receptor [see comments]. Science 1999;284: 1365–8.
-
(1999)
Science
, vol.284
, pp. 1365-1368
-
-
Parks, D.J.1
Blanchard, S.G.2
Bledsoe, R.K.3
-
57
-
-
0033026760
-
Endogenous bile acids are ligands for the nuclear receptor fxr/bar
-
Wang H, Chen J, Hollister K, et al. Endogenous bile acids are ligands for the nuclear receptor FXR/BAR. Mol Cell 1999;3: 543–53.
-
(1999)
Mol Cell
, vol.3
, pp. 543-553
-
-
Wang, H.1
Chen, J.2
Hollister, K.3
-
58
-
-
0031024075
-
Bile acid concentrations in human and rat liver tissue and in hepatocyte nuclei
-
Setchell KD, Rodrigues CM, Clerici C, et al. Bile acid concentrations in human and rat liver tissue and in hepatocyte nuclei. Gastroenterology 1997;112: 226–35.
-
(1997)
Gastroenterology
, vol.112
, pp. 226-235
-
-
Setchell, K.D.1
Rodrigues, C.M.2
Clerici, C.3
-
59
-
-
0019876782
-
Purification and properties of a 3ß-hydroxy-Δ5- c27-steroid oxidoreductase from rabbit liver microsomes
-
Wikvall K. Purification and properties of a 3ß-hydroxy-Δ5- C27-steroid oxidoreductase from rabbit liver microsomes. J Biol Chem 1981;256: 3376–80.
-
(1981)
J Biol Chem
, vol.256
, pp. 3376-3380
-
-
Wikvall, K.1
-
60
-
-
0029664964
-
Purification of a 3ß-hydroxydelta Δ5-c27-steroid dehydrogenase from pig liver microsomes active in major and alternative pathways of bile acid biosynthesis
-
Furster C, Zhang J, Toll A. Purification of a 3ß-hydroxydelta Δ5-C27-steroid dehydrogenase from pig liver microsomes active in major and alternative pathways of bile acid biosynthesis. J Biol Chem 1996;271: 20903–7.
-
(1996)
J Biol Chem
, vol.271
, pp. 20903-20907
-
-
Furster, C.1
Zhang, J.2
Toll, A.3
-
61
-
-
0024375357
-
Full length c dna structure and deduced amino acid sequence of human 3ß-hydroxy-5-ene steroid dehydrogenase
-
The VL, Lachance Y, Labrie C, et al. Full length c DNA structure and deduced amino acid sequence of human 3ß-hydroxy-5-ene steroid dehydrogenase. Mol Endocrinol 1989;3: 1310–12.
-
(1989)
Mol Endocrinol
, vol.3
, pp. 1310-1312
-
-
The, V.L.1
Lachance, Y.2
Labrie, C.3
-
62
-
-
0024788274
-
Molecular cloning, c dna structure and predicted amino acid sequence of bovine 3ß- hydroxy-5-ene steroid dehydrogenase/Δ5–4 isomerase
-
Zhao HF, Simard J, Labrie C, et al. Molecular cloning, c DNA structure and predicted amino acid sequence of bovine 3ß- hydroxy-5-ene steroid dehydrogenase/Δ5–4 isomerase. FEBS Lett 1989;259: 153–7.
-
(1989)
FEBS Lett
, vol.259
, pp. 153-157
-
-
Zhao, H.F.1
Simard, J.2
Labrie, C.3
-
63
-
-
0025295774
-
Human 3ß- hydroxysteroid dehydrogenase/Δ5–4 isomerase from placenta: Expression in nonsteroidogenic cells of a protein that catalyzes the dehydrogenation/isomerization of c21 and c19 steroids
-
Lorence MC, Murry BA, Trant JM, Mason JI. Human 3ß- hydroxysteroid dehydrogenase/Δ5–4 isomerase from placenta: expression in nonsteroidogenic cells of a protein that catalyzes the dehydrogenation/isomerization of C21 and C19 steroids. Endocrinology 1990;126: 2493–8.
-
(1990)
Endocrinology
, vol.126
, pp. 2493-2498
-
-
Lorence, M.C.1
Murry, B.A.2
Trant, J.M.3
Mason, J.I.4
-
64
-
-
0026018480
-
Characterization of rat 3ß-hydroxysteroid dehydrogenase/Δ5-Δ4 isomerase c dnas and differential tissue-specific expression of the corresponding m rnas in steroidogenic and peripheral tissues
-
Zhao HF, Labrie C, Simard J, et al. Characterization of rat 3ß-hydroxysteroid dehydrogenase/Δ5-Δ4 isomerase c DNAs and differential tissue-specific expression of the corresponding m RNAs in steroidogenic and peripheral tissues. J Biol Chem 1991;266: 583–93.
-
(1991)
J Biol Chem
, vol.266
, pp. 583-593
-
-
Zhao, H.F.1
Labrie, C.2
Simard, J.3
-
65
-
-
0033766717
-
The bile acid synthetic gene 3ßhydroxy-Δ5-c27-steroid oxidoreductase is mutated in progressive intrahepatic cholestasis
-
Schwarz M, Wright AC, Davis DL, et al. The bile acid synthetic gene 3ßhydroxy-Δ5-C27-steroid oxidoreductase is mutated in progressive intrahepatic cholestasis. J Clin Invest 2000;106: 1175–84.
-
(2000)
J Clin Invest
, vol.106
, pp. 1175-1184
-
-
Schwarz, M.1
Wright, A.C.2
Davis, D.L.3
-
66
-
-
0037762605
-
Molecular genetics of 3ß-hydroxy-Δ5-c27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease
-
Cheng JB, Jacquemin E, Gerhardt M, et al. Molecular genetics of 3ß-hydroxy-Δ5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease. J Clin Endocrinol Metab 2003;88: 1833–41.
-
(2003)
J Clin Endocrinol Metab
, vol.88
, pp. 1833-1841
-
-
Cheng, J.B.1
Jacquemin, E.2
Gerhardt, M.3
-
67
-
-
0026799389
-
Purification and characterization of 7a-hydroxy-4-cholesten-3-one 12a- hydroxylase
-
Ishida H, Noshiro M, Okuda K, Coon MJ. Purification and characterization of 7a-hydroxy-4-cholesten-3-one 12a- hydroxylase. J Biol Chem 1992;267: 21319–23.
-
(1992)
J Biol Chem
, vol.267
, pp. 21319-21323
-
-
Ishida, H.1
Noshiro, M.2
Okuda, K.3
Coon, M.J.4
-
68
-
-
0029758263
-
Molecular cloning and expression of rabbit sterol 12a-hydroxylase
-
Eggertsen G, Olin M, Andersson U, et al. Molecular cloning and expression of rabbit sterol 12a-hydroxylase. J Biol Chem 1996;271: 32269–75.
-
(1996)
J Biol Chem
, vol.271
, pp. 32269-32275
-
-
Eggertsen, G.1
Olin, M.2
Ersson, U.3
-
69
-
-
0033106423
-
Structure and chromosomal assignment of the sterol 12a-hydroxylase gene (Cyp8b1) inhumanandmouse: Eukaryotic cytochrome p-450 gene devoid of introns
-
Gafvels M, Olin M, Chowdhary BP, et al. Structure and chromosomal assignment of the sterol 12a-hydroxylase gene (CYP8B1) inhumanandmouse: eukaryotic cytochrome P-450 gene devoid of introns. Genomics 1999;56: 184–96.
-
(1999)
Genomics
, vol.56
, pp. 184-196
-
-
Gafvels, M.1
Olin, M.2
Chowdhary, B.P.3
-
70
-
-
0026448726
-
Bjorkhem i. 12a- hydroxylase activity inhumanliver and its relation tocholesterol 7a-hydroxylase activity
-
Einarsson K, Akerlund JE, Reihner E, Bjorkhem I. 12a- hydroxylase activity inhumanliver and its relation tocholesterol 7a-hydroxylase activity. J Lipid Res 1992;33: 1591–5.
-
(1992)
J Lipid Res
, vol.33
, pp. 1591-1595
-
-
Einarsson, K.1
Akerlund, J.E.2
Reihner, E.3
-
71
-
-
0014910222
-
Effect of cholestyramine and diet on hydroxylations in the biosynthesis and metabolism of bile acids
-
Johansson G. Effect of cholestyramine and diet on hydroxylations in the biosynthesis and metabolism of bile acids. Eur J Biochem 1970;17: 292–5.
-
(1970)
Eur J Biochem
, vol.17
, pp. 292-295
-
-
Johansson, G.1
-
72
-
-
0023097340
-
Biliary bile acid composition of the human fetus in early gestation
-
Colombo C, Zuliani G, Ronchi M, et al. Biliary bile acid composition of the human fetus in early gestation. Pediatr Res 1987;21: 197–200.
-
(1987)
Pediatr Res
, vol.21
, pp. 197-200
-
-
Colombo, C.1
Zuliani, G.2
Ronchi, M.3
-
73
-
-
0023744184
-
Hepatic bile acid metabolism during early development revealed from the analysis of human fetal gallbladder bile
-
Setchell KDR, Dumaswala R, Colombo C, Ronchi M. Hepatic bile acid metabolism during early development revealed from the analysis of human fetal gallbladder bile. J Biol Chem 1988;263: 16637–44.
-
(1988)
J Biol Chem
, vol.263
, pp. 16637-16644
-
-
Setchell, K.1
Dumaswala, R.2
Colombo, C.3
Ronchi, M.4
-
74
-
-
10044260166
-
On the bile acids in duodenal contents of infants and children
-
Encrantz JC, Sjovall J. On the bile acids in duodenal contents of infants and children. Clin Chim Acta 1959;4: 793–9.
-
(1959)
Clin Chim Acta
, vol.4
, pp. 793-799
-
-
Encrantz, J.C.1
Sjovall, J.2
-
76
-
-
0000340827
-
Bile acid content of gallbladder of infants, children and adults
-
Bongiovanni AM. Bile acid content of gallbladder of infants, children and adults. J Clin Endocrinol Metab 1965;25: 678–85.
-
(1965)
J Clin Endocrinol Metab
, vol.25
, pp. 678-685
-
-
Bongiovanni, A.M.1
-
77
-
-
0020416230
-
The bile acid composition of gastric contents from neonates with high intestinal obstruction
-
Clayton PT, Muller DP, Lawson AM. The bile acid composition of gastric contents from neonates with high intestinal obstruction. Biochem J 1982;206: 489–98.
-
(1982)
Biochem J
, vol.206
, pp. 489-498
-
-
Clayton, P.T.1
Muller, D.P.2
Lawson, A.M.3
-
78
-
-
0000447259
-
Dietary glycine and taurine conjugation in man
-
Sjovall J. Dietary glycine and taurine conjugation in man. Proc Soc Exp Biol Med 1959;100: 676–8.
-
(1959)
Proc Soc Exp Biol Med
, vol.100
, pp. 676-678
-
-
Sjovall, J.1
-
79
-
-
0014145625
-
Bile salt evolution
-
Haslewood GA. Bile salt evolution. J Lipid Res 1967;8: 535–50.
-
(1967)
J Lipid Res
, vol.8
, pp. 535-550
-
-
Haslewood, G.A.1
-
80
-
-
0003130071
-
Allo bile acids
-
Nair PP, Kritchevsky D, eds., New York: Plenum Press
-
Elliott WH. Allo bile acids. In: Nair PP, Kritchevsky D, eds. The bile acids: chemistry, physiology and metabolism. New York: Plenum Press, 1971: 47–93.
-
(1971)
The Bile Acids: Chemistry, Physiology and Metabolism
, pp. 47-93
-
-
Elliott, W.H.1
-
81
-
-
0024446655
-
Expression cloning and regulation of steroid 5a-reductase, an enzymeessential for male sexual differentiation
-
Andersson S, Bishop RW, Russell DW. Expression cloning and regulation of steroid 5a-reductase, an enzymeessential for male sexual differentiation. J Biol Chem 1989;264: 16249–55.
-
(1989)
J Biol Chem
, vol.264
, pp. 16249-16255
-
-
Andersson, S.1
Bishop, R.W.2
Russell, D.W.3
-
82
-
-
0027979898
-
Cloning and expression of c dna of human Δ4–3-oxosteroid 5ß-reductase and substrate specificity of the expressed enzyme
-
Kondo KH, Kai MH, Setoguchi Y, et al. Cloning and expression of c DNA of human Δ4–3-oxosteroid 5ß-reductase and substrate specificity of the expressed enzyme. Eur J Biochem 1994;219: 357–63.
-
(1994)
Eur J Biochem
, vol.219
, pp. 357-363
-
-
Kondo, K.H.1
Kai, M.H.2
Setoguchi, Y.3
-
84
-
-
0025728697
-
The plasma level of 7a-hydroxy-4-cholesten-3-one reflects the activity of hepatic cholesterol 7a-hydroxylase in man
-
Axelson M, Bjorkhem I, Reihner E, Einarsson K. The plasma level of 7a-hydroxy-4-cholesten-3-one reflects the activity of hepatic cholesterol 7a-hydroxylase in man. FEBS Lett 1991;284: 216–18.
-
(1991)
FEBS Lett
, vol.284
, pp. 216-218
-
-
Axelson, M.1
Bjorkhem, I.2
Reihner, E.3
Einarsson, K.4
-
85
-
-
0023935099
-
Lawson am, et al. 3-oxo-Δ4 bile acids in liver disease [letter]
-
Clayton PT, Patel E, Lawson AM, et al. 3-Oxo-Δ4 bile acids in liver disease [letter]. Lancet 1988;1: 1283–4.
-
(1988)
Lancet
, vol.1
, pp. 1283-1284
-
-
Clayton, P.T.1
Patel, E.2
-
86
-
-
0024246224
-
Welsh mb, et al. Δ4–3-oxosteroid 5ß- reductase deficiency described in identical twins with neonatal hepatitis. a new inborn error in bile acid synthesis
-
Setchell KDR, Suchy FJ, Welsh MB, et al. Δ4–3-oxosteroid 5ß- reductase deficiency described in identical twins with neonatal hepatitis. A new inborn error in bile acid synthesis. J Clin Invest 1988;82: 2148–57.
-
(1988)
J Clin Invest
, vol.82
, pp. 2148-2157
-
-
Setchell, K.1
Suchy, F.J.2
-
87
-
-
0141645554
-
Mutations in srd5b1 (Akr1d1), the gene encoding Δ4–3-oxosteroid 5ß- reductase, in hepatitis and liver failure in infancy
-
Lemonde HA, Custard EJ, Bouquet J, et al. Mutations in SRD5B1 (AKR1D1), the gene encoding Δ4–3-oxosteroid 5ß- reductase, in hepatitis and liver failure in infancy. Gut 2003;52: 1494–9.
-
(2003)
Gut
, vol.52
, pp. 1494-1499
-
-
Lemonde, H.A.1
Custard, E.J.2
Bouquet, J.3
-
88
-
-
0025813257
-
Pawlowski je. Affinity labeling of 3a-hydroxysteroid dehydrogenase with 3a- bromoacetoxyandrosterone and 11a-bromoacetoxyprogesterone. isolation and sequence of active site peptides containing reactive cysteines; sequence confirmation using nucleotide sequence fromac dna clone
-
Penning TM, Abrams WR, Pawlowski JE. Affinity labeling of 3a-hydroxysteroid dehydrogenase with 3a- bromoacetoxyandrosterone and 11a-bromoacetoxyprogesterone. Isolation and sequence of active site peptides containing reactive cysteines; sequence confirmation using nucleotide sequence fromac DNA clone. J Biol Chem 1991;266: 8826–34.
-
(1991)
J Biol Chem
, vol.266
, pp. 8826-8834
-
-
Penning, T.M.1
Abrams, W.R.2
-
89
-
-
0025998395
-
Molecular cloning and expression of rat liver 3a-hydroxysteroid dehydrogenase
-
Cheng KC, White PC, Qin KN. Molecular cloning and expression of rat liver 3a-hydroxysteroid dehydrogenase. Mol Endocrinol 1991;5: 823–8.
-
(1991)
Mol Endocrinol
, vol.5
, pp. 823-828
-
-
Cheng, K.C.1
White, P.C.2
Qin, K.N.3
-
90
-
-
0025835877
-
Cloning and sequencing of the c dna for rat liver 3a-hydroxysteroid/dihydrodiol dehydrogenase
-
Pawlowski JE, Huizinga M, Penning TM. Cloning and sequencing of the c DNA for rat liver 3a-hydroxysteroid/dihydrodiol dehydrogenase. J Biol Chem 1991;266: 8820–5.
-
(1991)
J Biol Chem
, vol.266
, pp. 8820-8825
-
-
Pawlowski, J.E.1
Huizinga, M.2
Penning, T.M.3
-
91
-
-
0025773967
-
Molecular structure of rat hepatic 3a-hydroxysteroid dehydrogenase. A member of the oxidoreductase gene family
-
Stolz A, Rahimi-Kiani M, Ameis D, et al. Molecular structure of rat hepatic 3a-hydroxysteroid dehydrogenase. A member of the oxidoreductase gene family. J Biol Chem 1991;266: 15253–7.
-
(1991)
J Biol Chem
, vol.266
, pp. 15253-15257
-
-
Stolz, A.1
Rahimi-Kiani, M.2
Ameis, D.3
-
92
-
-
0023138036
-
Sugiyama y, et al. 3a-hydroxysteroid dehydrogenase activity of the y’ bile acid binders in rat liver cytosol. identification, kinetics, and physiologic significance
-
Stolz A, Takikawa H, Sugiyama Y, et al. 3a-hydroxysteroid dehydrogenase activity of the Y’ bile acid binders in rat liver cytosol. Identification, kinetics, and physiologic significance. J Clin Invest 1987;79: 427–34.
-
(1987)
J Clin Invest
, vol.79
, pp. 427-434
-
-
Stolz, A.1
Takikawa, H.2
-
93
-
-
0026551710
-
Mechanism of degradation of the steroid side chain in the formation of bile acids
-
Bjorkhem I. Mechanism of degradation of the steroid side chain in the formation of bile acids. J Lipid Res 1992;33: 455–71.
-
(1992)
J Lipid Res
, vol.33
, pp. 455-471
-
-
Bjorkhem, I.1
-
94
-
-
0017764999
-
Carbon-13 nmr studies on cholesterol biosynthesized from [13c]mevalonates
-
Popjak G, Edmond J, Anet FA, Easton NR Jr. Carbon-13 NMR studies on cholesterol biosynthesized from [13C]mevalonates. J Am Chem Soc 1977;99: 931–5.
-
(1977)
J am Chem Soc
, vol.99
, pp. 931-935
-
-
Popjak, G.1
Edmond, J.2
Anet, F.A.3
Easton, N.R.4
-
95
-
-
0024394549
-
Cloning, structure, and expression of the mitochondrial cytochrome p-450 sterol 26-hydroxylase, a bile acid biosynthetic enzyme
-
Andersson S, Davis DL, Dahlback H, et al. Cloning, structure, and expression of the mitochondrial cytochrome P-450 sterol 26-hydroxylase, a bile acid biosynthetic enzyme. J Biol Chem 1989;264: 8222–9.
-
(1989)
J Biol Chem
, vol.264
, pp. 8222-8229
-
-
Andersson, S.1
Davis, D.L.2
Dahlback, H.3
-
96
-
-
0025209795
-
Molecular cloning of c dna for vitamin d3 25-hydroxylase from rat liver mitochondria
-
Usui E, Noshiro M, Okuda K. Molecular cloning of c DNA for vitamin D3 25-hydroxylase from rat liver mitochondria. FEBS Lett 1990;262: 135–8.
-
(1990)
FEBS Lett
, vol.262
, pp. 135-138
-
-
Usui, E.1
Noshiro, M.2
Okuda, K.3
-
97
-
-
0025149281
-
A c dna encoding a rat mitochondrial cytochrome p450 catalyzing both the 26- hydroxylation of cholesterol and 25-hydroxylation of vitamin d3: Gonadotropic regulation of the cognate m rna in ovaries
-
Su P, Rennert H, Shayiq RM, et al. A c DNA encoding a rat mitochondrial cytochrome P450 catalyzing both the 26- hydroxylation of cholesterol and 25-hydroxylation of vitamin D3: gonadotropic regulation of the cognate m RNA in ovaries. DNA Cell Biol 1990;9: 657–67.
-
(1990)
DNA Cell Biol
, vol.9
, pp. 657-667
-
-
Su, P.1
Rennert, H.2
Shayiq, R.M.3
-
98
-
-
0025914556
-
Mutations in the bile acid biosynthetic enzyme sterol 27-hydroxylase underlie cerebrotendinous xanthomatosis
-
Cali JJ, Hsieh CL, Francke U, Russell DW. Mutations in the bile acid biosynthetic enzyme sterol 27-hydroxylase underlie cerebrotendinous xanthomatosis. J Biol Chem 1991;266: 7779–83.
-
(1991)
J Biol Chem
, vol.266
, pp. 7779-7783
-
-
Cali, J.J.1
Hsieh, C.L.2
Francke, U.3
Russell, D.W.4
-
99
-
-
0022505085
-
Demonstration of 26- hydroxylation of c27-steroids in human skin fibroblasts, and a deficiency of this activity in cerebrotendinous xanthomatosis
-
Skrede S, Bjorkhem I, Kvittingen EA, et al. Demonstration of 26- hydroxylation of C27-steroids in human skin fibroblasts, and a deficiency of this activity in cerebrotendinous xanthomatosis. J Clin Invest 1986;78: 729–35.
-
(1986)
J Clin Invest
, vol.78
, pp. 729-735
-
-
Skrede, S.1
Bjorkhem, I.2
Kvittingen, E.A.3
-
100
-
-
0032511035
-
Markedly reduced bile acid synthesis but maintained levels of cholesterol and vitamin d metabolites in mice with disrupted sterol 27-hydroxylase gene
-
Rosen H, Reshef A, Maeda N, et al. Markedly reduced bile acid synthesis but maintained levels of cholesterol and vitamin D metabolites in mice with disrupted sterol 27-hydroxylase gene. J Biol Chem 1998;273: 14805–12.
-
(1998)
J Biol Chem
, vol.273
, pp. 14805-14812
-
-
Rosen, H.1
Reshef, A.2
Maeda, N.3
-
101
-
-
0017228402
-
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat
-
Shefer S, Cheng FW, Dayal B, et al. A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat. J Clin Invest 1976;57: 897–903.
-
(1976)
J Clin Invest
, vol.57
, pp. 897-903
-
-
Shefer, S.1
Cheng, F.W.2
Dayal, B.3
-
102
-
-
0023801335
-
Quantitative importance of the 25-hydroxylation pathway for bile acid biosynthesis in the rat
-
Duane WC, Bjorkhem I, Hamilton JN, Mueller SM. Quantitative importance of the 25-hydroxylation pathway for bile acid biosynthesis in the rat. Hepatology 1988;8: 613–18.
-
(1988)
Hepatology
, vol.8
, pp. 613-618
-
-
Duane, W.C.1
Bjorkhem, I.2
Hamilton, J.N.3
Mueller, S.M.4
-
103
-
-
0023797178
-
Bile acid synthesis in man. In vivo activity of the 25-hydroxylation pathway
-
Duane WC, Pooler PA, Hamilton JN. Bile acid synthesis in man. In vivo activity of the 25-hydroxylation pathway. J Clin Invest 1988;82: 82–5.
-
(1988)
J Clin Invest
, vol.82
, pp. 82-85
-
-
Duane, W.C.1
Pooler, P.A.2
Hamilton, J.N.3
-
104
-
-
0021320638
-
Hydroxylations in biosynthesis of bile acids. Isolation of a cytochrome p-450 from rabbit liver mitochondria catalyzing 26-hydroxylation of c27- steroids
-
Wikvall K. Hydroxylations in biosynthesis of bile acids. Isolation of a cytochrome P-450 from rabbit liver mitochondria catalyzing 26-hydroxylation of C27- steroids. J Biol Chem 1984;259: 3800–4.
-
(1984)
J Biol Chem
, vol.259
, pp. 3800-3804
-
-
Wikvall, K.1
-
105
-
-
0026028331
-
Expression of rat liver vitamin d3 25-hydroxylase c dna in saccharomyces cerevisiae
-
Akiyoshi-Shibata M, Usui E, Sakaki T, et al. Expression of rat liver vitamin D3 25-hydroxylase c DNA in Saccharomyces cerevisiae. FEBS Lett 1991;280: 367–70.
-
(1991)
FEBS Lett
, vol.280
, pp. 367-370
-
-
Akiyoshi-Shibata, M.1
Usui, E.2
Sakaki, T.3
-
106
-
-
0024995969
-
26-hydroxycholesterol: Synthesis, metabolism, and biologic activities
-
Javitt NB. 26-Hydroxycholesterol: synthesis, metabolism, and biologic activities. J Lipid Res 1990;31: 1527–33.
-
(1990)
J Lipid Res
, vol.31
, pp. 1527-1533
-
-
Javitt, N.B.1
-
107
-
-
0025326171
-
Oxidation of 5ß-cholestane-3a, 7a, 12a-triol into 3a, 7a, 12a-trihydroxy-5ß-cholestanoic acid by cytochrome p-450(26) from rabbit liver mitochondria
-
Dahlback H, Holmberg I. Oxidation of 5ß-cholestane-3a, 7a, 12a-triol into 3a, 7a, 12a-trihydroxy-5ß-cholestanoic acid by cytochrome P-450(26) from rabbit liver mitochondria. Biochem Biophys Res Commun 1990;167: 391–5.
-
(1990)
Biochem Biophys Res Commun
, vol.167
, pp. 391-395
-
-
Dahlback, H.1
Holmberg, I.2
-
108
-
-
0028584429
-
Bile acid synthesis from cholesterol: Regulatory and auxiliary pathways
-
Javitt NB. Bile acid synthesis from cholesterol: regulatory and auxiliary pathways. FASEB J 1994;8: 1308–11.
-
(1994)
FASEB J
, vol.8
, pp. 1308-1311
-
-
Javitt, N.B.1
-
109
-
-
0028068796
-
Atherosclerosis and sterol 27-hydroxylase: Evidence for a role of this enzyme in elimination of cholesterol from human macrophages
-
Bjorkhem I, Andersson O, Diczfalusy U, et al. Atherosclerosis and sterol 27-hydroxylase: evidence for a role of this enzyme in elimination of cholesterol from human macrophages. Proc Natl Acad Sci U S A 1994;91: 8592–6.
-
(1994)
Proc Natl Acad Sci U S A
, vol.91
, pp. 8592-8596
-
-
Bjorkhem, I.1
Ersson, O.2
Diczfalusy, U.3
-
110
-
-
0028216504
-
Sterol 27-hydroxylase: High levels of activity in vascular endothelium
-
Reiss AB, Martin KO, Javitt NB, et al. Sterol 27-hydroxylase: high levels of activity in vascular endothelium. J Lipid Res 1994; 35: 1026–30.
-
(1994)
J Lipid Res
, vol.35
, pp. 1026-1030
-
-
Reiss, A.B.1
Martin, K.O.2
Javitt, N.B.3
-
111
-
-
0030062778
-
Importance of a novel oxidativemechanism for elimination of intracellular cholesterol in humans
-
Lund E, Andersson O, Zhang J, et al. Importance of a novel oxidativemechanism for elimination of intracellular cholesterol in humans. Arterioscler Thromb Vasc Biol 1996;16: 208–12.
-
(1996)
Arterioscler Thromb Vasc Biol
, vol.16
, pp. 208-212
-
-
Lund, E.1
Ersson, O.2
Zhang, J.3
-
112
-
-
0030720329
-
Elimination of cholesterol in macrophages and endothelial cells by the sterol 27-hydroxylase mechanism. Comparison with high density lipoprotein-mediated reverse cholesterol transport
-
Babiker A, Andersson O, Lund E, et al. Elimination of cholesterol in macrophages and endothelial cells by the sterol 27-hydroxylase mechanism. Comparison with high density lipoprotein-mediated reverse cholesterol transport. JBiol Chem 1997;272: 26253–61.
-
(1997)
Jbiol Chem
, vol.272
, pp. 26253-26261
-
-
Babiker, A.1
Ersson, O.2
Lund, E.3
-
113
-
-
0020570422
-
Physiological function and kinetic mechanism of human liver alcohol dehydrogenase as 5ß- cholestane-3a, 7a, 12a, 26-tetrol dehydrogenase
-
Okuda A, Okuda K. Physiological function and kinetic mechanism of human liver alcohol dehydrogenase as 5ß- cholestane-3a, 7a, 12a, 26-tetrol dehydrogenase. J Biol Chem 1983;258: 2899–905.
-
(1983)
J Biol Chem
, vol.258
, pp. 2899-2905
-
-
Okuda, A.1
Okuda, K.2
-
114
-
-
0023740620
-
Subcellular localization of 3a, 7a-dihydroxy- and 3a, 7a, 12a-trihydroxy-5ß- cholestanoyl-coenzyme a ligase(S) in rat liver
-
Prydz K, Kase BF, Bjorkhem I, Pedersen JI. Subcellular localization of 3a, 7a-dihydroxy- and 3a, 7a, 12a-trihydroxy-5ß- cholestanoyl-coenzyme A ligase(s) in rat liver. J Lipid Res 1988;29: 997–1004.
-
(1988)
J Lipid Res
, vol.29
, pp. 997-1004
-
-
Prydz, K.1
Kase, B.F.2
Bjorkhem, I.3
Pedersen, J.I.4
-
116
-
-
0025257335
-
Presence of three acyl-co a oxidases in rat liver peroxisomes. An inducible fatty acyl-co a oxidase, a noninducible fatty acyl-co a oxidase, and a noninducible trihydroxycoprostanoyl-co a oxidase
-
Schepers L, Van Veldhoven PP, Casteels M, et al. Presence of three acyl-Co A oxidases in rat liver peroxisomes. An inducible fatty acyl-Co A oxidase, a noninducible fatty acyl-Co A oxidase, and a noninducible trihydroxycoprostanoyl-Co A oxidase. J Biol Chem 1990;265: 5242–6.
-
(1990)
J Biol Chem
, vol.265
, pp. 5242-5246
-
-
Schepers, L.1
Van Veldhoven, P.P.2
Casteels, M.3
-
117
-
-
0027159784
-
The co a esters of 2-methyl-branched chain fatty acids and of the bile acid intermediates di- and trihydroxycoprostanic acids are oxidized by one single peroxisomal branched chain acyl-co a oxidase in human liver and kidney
-
Vanhove GF, Van Veldhoven PP, Fransen M, et al. The Co A esters of 2-methyl-branched chain fatty acids and of the bile acid intermediates di- and trihydroxycoprostanic acids are oxidized by one single peroxisomal branched chain acyl-Co A oxidase in human liver and kidney. J Biol Chem 1993;268: 10335–44.
-
(1993)
J Biol Chem
, vol.268
, pp. 10335-10344
-
-
Vanhove, G.F.1
Van Veldhoven, P.P.2
Fransen, M.3
-
118
-
-
84914641764
-
Peroxisomal proteins involved in bile salt biosynthesis
-
Paumgartner G, Stiehl A, Gerok W, eds., Hangham, MA: Kluwer Academic Publishers
-
Gengenbacher T, Gerok W, Giese U, et al. Peroxisomal proteins involved in bile salt biosynthesis. In: Paumgartner G, Stiehl A, Gerok W, eds. Bile acids as therapeutic agents. Hangham, MA: Kluwer Academic Publishers, 1991: 63–76.
-
(1991)
Bile Acids as Therapeutic Agents
, pp. 63-76
-
-
Gengenbacher, T.1
Gerok, W.2
Giese, U.3
-
120
-
-
0019209725
-
An in vivo evaluation of the quantitative significance of several potential pathways to cholic and chenodeoxycholic acids from cholesterol in man
-
Swell L, Gustafsson J, Schwartz CC, et al. An in vivo evaluation of the quantitative significance of several potential pathways to cholic and chenodeoxycholic acids from cholesterol in man. J Lipid Res 1980;21: 455–66.
-
(1980)
J Lipid Res
, vol.21
, pp. 455-466
-
-
Swell, L.1
Gustafsson, J.2
Schwartz, C.C.3
-
121
-
-
0025045782
-
Potential bile acid precursors in plasma – possible indicators of biosynthetic pathways to cholic and chenodeoxycholic acids inman
-
Axelson M, Sjovall J. Potential bile acid precursors in plasma – possible indicators of biosynthetic pathways to cholic and chenodeoxycholic acids inman. J Steroid Biochem 1990;36: 631–40.
-
(1990)
J Steroid Biochem
, vol.36
, pp. 631-640
-
-
Axelson, M.1
Sjovall, J.2
-
122
-
-
0018209681
-
Alternative biogenetic pathways of c24-bile acids with special reference to chenodeoxycholic acid
-
Yamasaki K. Alternative biogenetic pathways of C24-bile acids with special reference to chenodeoxycholic acid. Kawasaki Med J 1978;4: 227–64.
-
(1978)
Kawasaki Med J
, vol.4
, pp. 227-264
-
-
Yamasaki, K.1
-
123
-
-
0018884141
-
Biosynthesis of bile acids in man. Multiple pathways to cholic acid and chenodeoxycholic acid
-
Vlahcevic ZR, Schwartz CC, Gustafsson J, et al. Biosynthesis of bile acids in man. Multiple pathways to cholic acid and chenodeoxycholic acid. J Biol Chem 1980;255: 2925–33.
-
(1980)
J Biol Chem
, vol.255
, pp. 2925-2933
-
-
Vlahcevic, Z.R.1
Schwartz, C.C.2
Gustafsson, J.3
-
124
-
-
0015251672
-
Bile acid synthesis in man: Metabolism of 7a-hydroxycholesterol-14 c and 26- hydroxycholesterol-3 h
-
Anderson KE, Kok E, Javitt NB. Bile acid synthesis in man: metabolism of 7a-hydroxycholesterol-14 C and 26- hydroxycholesterol-3 H. J Clin Invest 1972;51: 112–17.
-
(1972)
J Clin Invest
, vol.51
, pp. 112-117
-
-
Anderson, K.E.1
Kok, E.2
Javitt, N.B.3
-
125
-
-
0029589217
-
A novel cytochrome p450expressedprimarily inbrain
-
Stapleton G, Steel M, Richardson M, et al. A novel cytochrome P450expressedprimarily inbrain. J Biol Chem 1995;270: 29739–45.
-
(1995)
J Biol Chem
, vol.270
, pp. 29739-29745
-
-
Stapleton, G.1
Steel, M.2
Richardson, M.3
-
126
-
-
0029143894
-
7a-hydroxylation of 27-hydroxycholesterol in rat brain microsomes
-
Zhang J, Akwa Y, Baulieu EE, Sjovall J. 7a-Hydroxylation of 27-hydroxycholesterol in rat brain microsomes. C R Acad Sci III 1995;318: 345–9.
-
(1995)
C R Acad Sci III
, vol.318
, pp. 345-349
-
-
Zhang, J.1
Akwa, Y.2
Baulieu, E.E.3
Sjovall, J.4
-
127
-
-
0028989206
-
7a hydroxylation of 25- hydroxycholesterol and 27-hydroxycholesterol in human fibroblasts
-
Zhang J, Larsson O, Sjovall J. 7a Hydroxylation of 25- hydroxycholesterol and 27-hydroxycholesterol in human fibroblasts. Biochim Biophys Acta 1995;1256: 353–9.
-
(1995)
Biochim Biophys Acta
, vol.1256
, pp. 353-359
-
-
Zhang, J.1
Larsson, O.2
Sjovall, J.3
-
128
-
-
0028891885
-
Structural specificity in the suppressionof hmg-co areductase inhuman fibroblasts by intermediates in bile acid biosynthesis
-
Axelson M, Larsson O, Zhang J, et al. Structural specificity in the suppressionof HMG-Co Areductase inhuman fibroblasts by intermediates in bile acid biosynthesis. J Lipid Res 1995;36: 290–8.
-
(1995)
J Lipid Res
, vol.36
, pp. 290-298
-
-
Axelson, M.1
Larsson, O.2
Zhang, J.3
-
129
-
-
0031576838
-
Studies on the relationships between 7 alpha-hydroxylation and the ability of 25- and 27- hydroxycholesterol to suppress the activity of hmg-co areductase
-
Zhang J, Dricu A, Sjovall J. Studies on the relationships between 7 alpha-hydroxylation and the ability of 25- and 27- hydroxycholesterol to suppress the activity of HMG-Co Areductase. Biochim Biophys Acta 1997;1344: 241–9.
-
(1997)
Biochim Biophys Acta
, vol.1344
, pp. 241-249
-
-
Zhang, J.1
Dricu, A.2
Sjovall, J.3
-
130
-
-
0026500434
-
7a-hydroxylation of 26- hydroxycholesterol, 3ß-hydroxy-5-cholestenoic acid and 3ß- hydroxy-5-cholenoic acid by cytochrome p- 450 in pig liver microsomes
-
Toll A, Shoda J, Axelson M, et al. 7a-hydroxylation of 26- hydroxycholesterol, 3ß-hydroxy-5-cholestenoic acid and 3ß- hydroxy-5-cholenoic acid by cytochrome P- 450 in pig liver microsomes. FEBS Lett 1992;296: 73–6.
-
(1992)
FEBS Lett
, vol.296
, pp. 73-76
-
-
Toll, A.1
Shoda, J.2
Axelson, M.3
-
131
-
-
0026738208
-
7a-hydroxylation of 27- hydroxycholesterol in human liver microsomes
-
Bjorkhem I, Nyberg B, Einarsson K. 7a-Hydroxylation of 27- hydroxycholesterol in human liver microsomes. Biochim Biophys Acta 1992;1128: 73–6.
-
(1992)
Biochim Biophys Acta
, vol.1128
, pp. 73-76
-
-
Bjorkhem, I.1
Nyberg, B.2
Einarsson, K.3
-
132
-
-
0028168279
-
7a-hydroxylation of 25-hydroxycholesterol in liver microsomes. Evidence that the enzyme involved is different from cholesterol 7a-hydroxylase
-
Toll A, Wikvall K, Sudjana-Sugiaman E, et al. 7a-hydroxylation of 25-hydroxycholesterol in liver microsomes. Evidence that the enzyme involved is different from cholesterol 7a-hydroxylase. Eur J Biochem 1994;224: 309–16.
-
(1994)
Eur J Biochem
, vol.224
, pp. 309-316
-
-
Toll, A.1
Wikvall, K.2
Sudjana-Sugiaman, E.3
-
133
-
-
0023805311
-
Regulation of bile acid synthesis. Ii. effect of bile acid feeding on enzymes regulating hepatic cholesterol and bile acid synthesis in the rat
-
Heuman DM, Vlahcevic ZR, Bailey ML, Hylemon PB. Regulation of bile acid synthesis. II. Effect of bile acid feeding on enzymes regulating hepatic cholesterol and bile acid synthesis in the rat. Hepatology 1988;8: 892–7.
-
(1988)
Hepatology
, vol.8
, pp. 892-897
-
-
Heuman, D.M.1
Vlahcevic, Z.R.2
Bailey, M.L.3
Hylemon, P.B.4
-
134
-
-
0033386688
-
Structure and functions of human oxysterol 7a-hydroxylase c dnas and gene cyp7b1
-
Wu Z, Martin KO, Javitt NB, Chiang JY. Structure and functions of human oxysterol 7a-hydroxylase c DNAs and gene CYP7B1. J Lipid Res 1999;40: 2195–203.
-
(1999)
J Lipid Res
, vol.40
, pp. 2195-2203
-
-
Wu, Z.1
Martin, K.O.2
Javitt, N.B.3
Chiang, J.Y.4
-
135
-
-
0014216154
-
Metabolism of taurolithocholic acid in the hamster
-
Emerman S, Javitt NB. Metabolism of taurolithocholic acid in the hamster. J Biol Chem 1967;242: 661–4.
-
(1967)
J Biol Chem
, vol.242
, pp. 661-664
-
-
Emerman, S.1
Javitt, N.B.2
-
136
-
-
0014287967
-
Effect of sodium taurolithocholate on bile flow and bile acid exeretion
-
Javitt NB, Emerman S. Effect of sodium taurolithocholate on bile flow and bile acid exeretion. J Clin Invest 1968;47: 1002–14.
-
(1968)
J Clin Invest
, vol.47
, pp. 1002-1014
-
-
Javitt, N.B.1
Emerman, S.2
-
137
-
-
0020562698
-
Monohydroxy bile salt sulfates: Tauro-3ß-hydroxy-5-cholenoate-3-sulfate induces intrahepatic cholestasis in rats
-
Mathis U, Karlaganis G, Preisig R. Monohydroxy bile salt sulfates: tauro-3ß-hydroxy-5-cholenoate-3-sulfate induces intrahepatic cholestasis in rats. Gastroenterology 1983;85: 674–81.
-
(1983)
Gastroenterology
, vol.85
, pp. 674-681
-
-
Mathis, U.1
Karlaganis, G.2
Preisig, R.3
-
138
-
-
0023838547
-
Bile acid synthesis: Down-regulation by monohydroxy bile acids
-
Hall R, Kok E, Javitt NB. Bile acid synthesis: down-regulation by monohydroxy bile acids. FASEB J 1988;2: 152–6.
-
(1988)
FASEB J
, vol.2
, pp. 152-156
-
-
Hall, R.1
Kok, E.2
Javitt, N.B.3
-
139
-
-
0018348127
-
Cholic acid biosynthesis: The enzymatic defect in cerebrotendinous xanthomatosis
-
Salen G, Shefer S, Cheng FW, et al. Cholic acid biosynthesis: the enzymatic defect in cerebrotendinous xanthomatosis. J Clin Invest 1979;63: 38–44.
-
(1979)
J Clin Invest
, vol.63
, pp. 38-44
-
-
Salen, G.1
Shefer, S.2
Cheng, F.W.3
-
140
-
-
0015423157
-
The formation of cholest-5-ene-3, 26-diol as an intermediate in the conversion of cholesterol into bile acids by liver mitochondria
-
Mitropoulos KA, Avery MD, Myant NB, Gibbons GF. The formation of cholest-5-ene-3, 26-diol as an intermediate in the conversion of cholesterol into bile acids by liver mitochondria. Biochem J 1972;130: 363–71.
-
(1972)
Biochem J
, vol.130
, pp. 363-371
-
-
Mitropoulos, K.A.1
Avery, M.D.2
Myant, N.B.3
Gibbons, G.F.4
-
141
-
-
0014085664
-
The formation of lithocholic acid, chenodeoxycholic acid and a- and ß-muricholic acids from cholesterol incubated with rat-liver mitochondria
-
Mitropoulos KA, Myant NB. The formation of lithocholic acid, chenodeoxycholic acid and a- and ß-muricholic acids from cholesterol incubated with rat-liver mitochondria. Biochem J 1967;103: 472–9.
-
(1967)
Biochem J
, vol.103
, pp. 472-479
-
-
Mitropoulos, K.A.1
Myant, N.B.2
-
142
-
-
0014409946
-
Metabolism of cholest-5-ene- 3ß, 26-diol in the rat and hamster
-
Wachtel N, Emerman S, Javitt NB. Metabolism of cholest-5-ene- 3ß, 26-diol in the rat and hamster. J Biol Chem 1968;243: 5207–12.
-
(1968)
J Biol Chem
, vol.243
, pp. 5207-5212
-
-
Wachtel, N.1
Emerman, S.2
Javitt, N.B.3
-
143
-
-
0019481106
-
Bile acid synthesis. Metabolism of 3ß-hydroxy-5-cholenoic acid in the hamster
-
Kok E, Burstein S, Javitt NB, et al. Bile acid synthesis. Metabolism of 3ß-hydroxy-5-cholenoic acid in the hamster. J Biol Chem 1981;256: 6155–9.
-
(1981)
J Biol Chem
, vol.256
, pp. 6155-6159
-
-
Kok, E.1
Burstein, S.2
Javitt, N.B.3
-
144
-
-
0020260530
-
Chenodeoxycholic acid synthesis in the hamster: A metabolic pathway via 3ß, 7a-dihydroxy-5-cholen- 24-oic acid
-
Kulkarni B, Javitt NB. Chenodeoxycholic acid synthesis in the hamster: a metabolic pathway via 3ß, 7a-dihydroxy-5-cholen- 24-oic acid. Steroids 1982;40: 581–9.
-
(1982)
Steroids
, vol.40
, pp. 581-589
-
-
Kulkarni, B.1
Javitt, N.B.2
-
145
-
-
0022357561
-
Bile acid synthesis in rat liver peroxisomes: Metabolism of 26-hydroxycholesterol to 3ß-hydroxy-5-cholenoic acid
-
Krisans SK, Thompson SL, Pena LA, et al. Bile acid synthesis in rat liver peroxisomes: metabolism of 26-hydroxycholesterol to 3ß-hydroxy-5-cholenoic acid. J Lipid Res 1985;26: 1324–32.
-
(1985)
J Lipid Res
, vol.26
, pp. 1324-1332
-
-
Krisans, S.K.1
Thompson, S.L.2
Pena, L.A.3
-
146
-
-
0014283873
-
Dietary ß-sitosterol as an internal standard to correct for cholesterol losses in sterol balance studies
-
Grundy SM, Ahrens EH Jr, Salen G. Dietary ß-sitosterol as an internal standard to correct for cholesterol losses in sterol balance studies. J Lipid Res 1968;9: 374–87.
-
(1968)
J Lipid Res
, vol.9
, pp. 374-387
-
-
Grundy, S.M.1
Ahrens, E.H.2
Salen, G.3
-
149
-
-
0019497586
-
Identification of acidic steroids in feces of monkeys fed beta-sitoserol
-
Kritchevsky D, Davidson LM, Mosbach EH, Cohen BI. Identification of acidic steroids in feces of monkeys fed beta-sitoserol. Lipids 1981;16: 77–8.
-
(1981)
Lipids
, vol.16
, pp. 77-78
-
-
Kritchevsky, D.1
Davidson, L.M.2
Mosbach, E.H.3
Cohen, B.I.4
-
150
-
-
0021931152
-
The presence of 5a- sitostanol in the serumof a patientwith phytosterolemia, and its biosynthesis fromplant steroids in ratswith bile fistula
-
Skrede B, Bjorkhem I, Bergesen O, et al. The presence of 5a- sitostanol in the serumof a patientwith phytosterolemia, and its biosynthesis fromplant steroids in ratswith bile fistula. Biochim Biophys Acta 1985;836: 368–75.
-
(1985)
Biochim Biophys Acta
, vol.836
, pp. 368-375
-
-
Skrede, B.1
Bjorkhem, I.2
Bergesen, O.3
-
151
-
-
0025325566
-
Apparent lack of conversion of sitosterol into c24-bile acids in humans
-
Boberg KM, Einarsson K, Bjorkhem I. Apparent lack of conversion of sitosterol into C24-bile acids in humans. J Lipid Res 1990;31: 1083–8.
-
(1990)
J Lipid Res
, vol.31
, pp. 1083-1088
-
-
Boberg, K.M.1
Einarsson, K.2
Bjorkhem, I.3
-
152
-
-
0025195190
-
Formationof c21bile acids from plant sterols in the rat
-
Boberg KM, Lund E, Olund J, Bjorkhem I. Formationof C21bile acids from plant sterols in the rat. J Biol Chem 1990;265: 7967–75.
-
(1990)
J Biol Chem
, vol.265
, pp. 7967-7975
-
-
Boberg, K.M.1
Lund, E.2
Olund, J.3
Bjorkhem, I.4
-
153
-
-
0025922165
-
Formation of novel c21-bile acids from cholesterol in the rat. Structure identification of the major di- and trihydroxylated species
-
Lund E, Boberg KM, Bystrom S, et al. Formation of novel C21-bile acids from cholesterol in the rat. Structure identification of the major di- and trihydroxylated species. J Biol Chem 1991;266: 4929–37.
-
(1991)
J Biol Chem
, vol.266
, pp. 4929-4937
-
-
Lund, E.1
Boberg, K.M.2
Bystrom, S.3
-
154
-
-
0019432243
-
Assay of intermediates in bile acid biosynthesis using isotope dilution- -mass spectrometry: Hepatic levels in the normal state and in cerebrotendinous xanthomatosis
-
Bjorkhem I, Oftebro H, Skrede S, Pedersen JI. Assay of intermediates in bile acid biosynthesis using isotope dilution- -mass spectrometry: hepatic levels in the normal state and in cerebrotendinous xanthomatosis. J Lipid Res 1981;22: 191–200.
-
(1981)
J Lipid Res
, vol.22
, pp. 191-200
-
-
Bjorkhem, I.1
Oftebro, H.2
Skrede, S.3
Pedersen, J.I.4
-
155
-
-
0026007201
-
Bile acid synthesis in cultured human hepatoblastoma cells
-
Axelson M, Mork B, Everson GT. Bile acid synthesis in cultured human hepatoblastoma cells. J Biol Chem 1991;266: 17770–7.
-
(1991)
J Biol Chem
, vol.266
, pp. 17770-17777
-
-
Axelson, M.1
Mork, B.2
Everson, G.T.3
-
156
-
-
78651173223
-
Bile acids. Xxii. allocholic acid, a metabolite of 5a-cholestan-3ß-ol in the rat
-
Karavolas HJ, Elliott WH, Hsia SL, et al. Bile acids. XXII. Allocholic acid, a metabolite of 5a-cholestan-3ß-ol in the rat. J Biol Chem 1965;240: 1568–77.
-
(1965)
J Biol Chem
, vol.240
, pp. 1568-1577
-
-
Karavolas, H.J.1
Elliott, W.H.2
Hsia, S.L.3
-
157
-
-
68949151196
-
Identification of allodeoxycholic acid as the major component of gallstones induced in the rabbit by 5a-cholestan-3ß-ol
-
Hofmann AF, Mosbach EH. Identification of allodeoxycholic acid as the major component of gallstones induced in the rabbit by 5a-cholestan-3ß-ol. J Biol Chem 1964;239: 2813–21.
-
(1964)
J Biol Chem
, vol.239
, pp. 2813-2821
-
-
Hofmann, A.F.1
Mosbach, E.H.2
-
158
-
-
0014984408
-
On the conversion of cholestanol into allocholic acid in rat liver
-
Bjorkhem I, Gustafsson J. On the conversion of cholestanol into allocholic acid in rat liver. Eur J Biochem 1971;18: 207–13.
-
(1971)
Eur J Biochem
, vol.18
, pp. 207-213
-
-
Bjorkhem, I.1
Gustafsson, J.2
-
159
-
-
0015239040
-
Allocholic acid, a metabolite of allochenodeoxycholic acid in bile fistula rat
-
Mui MM, Elliott WH. Bile acids. XXXII. Allocholic acid, a metabolite of allochenodeoxycholic acid in bile fistula rats. J Biol Chem 1971;246: 302–4.
-
(1971)
J Biol Che
, vol.246
, pp. 302-304
-
-
Mui, M.M.1
Bile Xxxi, E.W.A.2
-
160
-
-
0016220217
-
Bile acids. Xli. hepatic microsomal 12a-hydroxylation of allochenodeoxycholate to allocholate
-
Blaskiewicz RJ, O’Neil GJ Jr, Elliott WH. Bile acids. XLI. Hepatic microsomal 12a-hydroxylation of allochenodeoxycholate to allocholate. Proc Soc Exp Biol Med 1974;146: 92–5.
-
(1974)
Proc Soc Exp Biol Med
, vol.146
, pp. 92-95
-
-
Blaskiewicz, R.J.1
O’Neil, G.J.2
Elliott, W.H.3
-
161
-
-
0017118662
-
Bile acids. Li. formation of 12a-hydroxyl derivatives and companions from 5a-sterols by rabbit liver microsome
-
Ali SS, Elliott WH. Bile acids. LI. Formation of 12a-hydroxyl derivatives and companions from 5a-sterols by rabbit liver microsomes. J Lipid Res 1976;17: 386–92.
-
(1976)
J Lipid Re
, vol.17
, pp. 386-389
-
-
Ali, S.S.1
Elliott, W.H.2
-
162
-
-
0027234897
-
Tissue distribution and ontogeny of steroid 5a-reductase isozymeexpression
-
Thigpen AE, Silver RI, Guileyardo JM, et al. Tissue distribution and ontogeny of steroid 5a-reductase isozymeexpression. JClin Invest 1993;92: 903–10.
-
(1993)
Jclin Invest
, vol.92
, pp. 903-910
-
-
Thigpen, A.E.1
Silver, R.I.2
Guileyardo, J.M.3
-
163
-
-
0023675927
-
Similarityofunusual bile acids in human umbilical cord blood and amniotic fluid from newborns and in sera and urine from adult patients with cholestatic liver diseases
-
Shoda J, Mahara R, Osuga T, et al. Similarityofunusual bile acids in human umbilical cord blood and amniotic fluid from newborns and in sera and urine from adult patients with cholestatic liver diseases. J Lipid Res 1988;29: 847–58.
-
(1988)
J Lipid Res
, vol.29
, pp. 847-858
-
-
Shoda, J.1
Mahara, R.2
Osuga, T.3
-
164
-
-
0021958537
-
Bile acid synthesis during development. Mitochondrial 12a-hydroxylation in human fetal liver
-
Gustafsson J. Bile acid synthesis during development. Mitochondrial 12a-hydroxylation in human fetal liver. J Clin Invest 1985;75: 604–7.
-
(1985)
J Clin Invest
, vol.75
, pp. 604-607
-
-
Gustafsson, J.1
-
165
-
-
0023096924
-
Bile acid metabolism during development: Metabolism of lithocholic acid in human fetal liver
-
Gustafsson J, Anderson S, Sjovall J. Bile acid metabolism during development: metabolism of lithocholic acid in human fetal liver. Pediatr Res 1987;21: 99–103.
-
(1987)
Pediatr Res
, vol.21
, pp. 99-103
-
-
Gustafsson, J.1
Erson, S.2
Sjovall, J.3
-
166
-
-
0024360299
-
Identification of 3a, 4ß, 7a-trihydroxy-5ß-cholanoic acid in human bile: Reflection of a new pathway in bile acid metabolism in humans
-
Dumaswala R, Setchell KDR, Zimmer-Nechemias L, et al. Identification of 3a, 4ß, 7a-trihydroxy-5ß-cholanoic acid in human bile: reflection of a new pathway in bile acid metabolism in humans. J Lipid Res 1989;30: 847–56.
-
(1989)
J Lipid Res
, vol.30
, pp. 847-856
-
-
Dumaswala, R.1
Setchell, K.2
Zimmer-Nechemias, L.3
-
167
-
-
0020607713
-
Immaturity of the enterohepatic circulation in early life: Factors predisposing to “physiologic” maldigestion and cholestasis
-
Balistreri WF, Heubi JE, Suchy FJ. Immaturity of the enterohepatic circulation in early life: factors predisposing to “physiologic” maldigestion and cholestasis. J Pediatr Gastroenterol Nutr 1983;2: 346–54.
-
(1983)
J Pediatr Gastroenterol Nutr
, vol.2
, pp. 346-354
-
-
Balistreri, W.F.1
Heubi, J.E.2
Suchy, F.J.3
-
168
-
-
0008674355
-
The biological utility of bile acid conjugationwith glycine or taurine
-
Matern S, Bock KW, Gerok W, eds, Lancaster U. K.: MTP Press
-
Hofmann AF, Palmer KR, Yoon Y-B. The biological utility of bile acid conjugationwith glycine or taurine. In: Matern S, Bock KW, Gerok W, eds. Advances in glucuronide conjugation. Lancaster U. K.: MTP Press, 1985: 245–64.
-
(1985)
Advances in Glucuronide Conjugation
, pp. 245-264
-
-
Hofmann, A.F.1
Palmer, K.R.2
Yoon, Y.-B.3
-
169
-
-
84862665891
-
Studies on the conjugation of cholic acidwith taurine in cell subfractions
-
Bremer J, Gloor V. Studies on the conjugation of cholic acidwith taurine in cell subfractions. Acta Chem Scand 1955;9: 689–98.
-
(1955)
Acta Chem Scand
, vol.9
, pp. 689-698
-
-
Bremer, J.1
Gloor, V.2
-
170
-
-
0017854406
-
Measurement and subcellular distribution of choloyl-co a synthetase and bile acid-co a: Amino acid nacyltransferase activities in rat liver
-
Killenberg PG. Measurement and subcellular distribution of choloyl-Co A synthetase and bile acid-Co A: amino acid Nacyltransferase activities in rat liver. J Lipid Res 1978;19: 24–31.
-
(1978)
J Lipid Res
, vol.19
, pp. 24-31
-
-
Killenberg, P.G.1
-
171
-
-
0017410286
-
Characterization of microsomal choloylcoenzyme a synthetase
-
Vessey DA, Zakim D. Characterization of microsomal choloylcoenzyme A synthetase. Biochem J 1977;163: 357–62.
-
(1977)
Biochem J
, vol.163
, pp. 357-362
-
-
Vessey, D.A.1
Zakim, D.2
-
172
-
-
0031543350
-
Purification and characterization of a rat liver bile acid coenzyme a ligase from rat liver microsomes
-
Wheeler JB, Shaw DR, Barnes S. Purification and characterization of a rat liver bile acid coenzyme A ligase from rat liver microsomes. Arch Biochem Biophys 1997;348: 15–24.
-
(1997)
Arch Biochem Biophys
, vol.348
, pp. 15-24
-
-
Wheeler, J.B.1
Shaw, D.R.2
Barnes, S.3
-
173
-
-
0025866940
-
Purification and characterization of bile acid-co a: Amino acid n-acyltransferase from human liver
-
Johnson MR, Barnes S, Kwakye JB, Diasio RB. Purification and characterization of bile acid-Co A: amino acid N-acyltransferase from human liver. J Biol Chem 1991;266: 10227–33.
-
(1991)
J Biol Chem
, vol.266
, pp. 10227-10233
-
-
Johnson, M.R.1
Barnes, S.2
Kwakye, J.B.3
Diasio, R.B.4
-
175
-
-
0024374648
-
Peroxisomal bile acid-co a: Amino-acid n-acyltransferase in rat liver
-
Kase BF, Bjorkhem I. Peroxisomal bile acid-Co A: amino-acid N-acyltransferase in rat liver. J Biol Chem 1989;264: 9220–3.
-
(1989)
J Biol Chem
, vol.264
, pp. 9220-9223
-
-
Kase, B.F.1
Bjorkhem, I.2
-
176
-
-
30144444322
-
Bile acid coenzyme a: Amino acid n-acyltransferase in the amino acid conjugation of bile acids
-
Shonsey EM, Sfakianos M, Johnson M, et al. Bile acid coenzyme A: amino acid N-acyltransferase in the amino acid conjugation of bile acids. Methods Enzymol 2005;400: 374–94.
-
(2005)
Methods Enzymol
, vol.400
, pp. 374-394
-
-
Shonsey, E.M.1
Sfakianos, M.2
Johnson, M.3
-
177
-
-
0028108409
-
Glycine and taurine conjugation of bile acids by a single enzyme. Molecular cloning and expression of human liver bile acid co a: Amino acid n-acyltransferase
-
Falany CN, Johnson MR, Barnes S, Diasio RB. Glycine and taurine conjugation of bile acids by a single enzyme. Molecular cloning and expression of human liver bile acid Co A: amino acid N-acyltransferase. J Biol Chem 1994;269: 19375–9.
-
(1994)
J Biol Chem
, vol.269
, pp. 19375-19379
-
-
Falany, C.N.1
Johnson, M.R.2
Barnes, S.3
Diasio, R.B.4
-
178
-
-
0030962690
-
Cloning, expression, and chromosomal localization of mouse liver bile acid co a: Amino acid n-acyltransferase
-
Falany CN, Fortinberry H, Leiter EH, Barnes S. Cloning, expression, and chromosomal localization of mouse liver bile acid Co A: amino acid N-acyltransferase. J Lipid Res 1997;38: 1139–48.
-
(1997)
J Lipid Res
, vol.38
, pp. 1139-1148
-
-
Falany, C.N.1
Fortinberry, H.2
Leiter, E.H.3
Barnes, S.4
-
179
-
-
0036910989
-
Molecular cloning and expression of rat liver bile acid co a ligase
-
Falany CN, Xie X, Wheeler JB, et al. Molecular cloning and expression of rat liver bile acid Co A ligase. J Lipid Res 2002;43: 2062–72.
-
(2002)
J Lipid Res
, vol.43
, pp. 2062-2072
-
-
Falany, C.N.1
Xie, X.2
Wheeler, J.B.3
-
180
-
-
0019501566
-
Subcellular distribution of hepatic bile acid-conjugating enzymes
-
Lim WC, Jordan TW. Subcellular distribution of hepatic bile acid-conjugating enzymes. Biochem J 1981;197: 611–18.
-
(1981)
Biochem J
, vol.197
, pp. 611-618
-
-
Lim, W.C.1
Jordan, T.W.2
-
181
-
-
0141780836
-
The human bile acid- co a: Amino acid n-acyltransferase functions in the conjugation of fatty acids to glycine
-
O’Byrne J, Hunt MC, Rai DK, et al. The human bile acid- Co A: amino acid N-acyltransferase functions in the conjugation of fatty acids to glycine. J Biol Chem 2003;278: 34237–44.
-
(2003)
J Biol Chem
, vol.278
, pp. 34237-34244
-
-
O’Byrne, J.1
Hunt, M.C.2
Rai, D.K.3
-
182
-
-
0023883486
-
Effect of side chain length on bile acid conjugation: Glucuronidation, sulfation and coenzyme a formation of nor-bile acids and their natural c24 homologs by human and rat liver fractions
-
Kirkpatrick RB, Green MD, Hagey LR, et al. Effect of side chain length on bile acid conjugation: glucuronidation, sulfation and coenzyme A formation of nor-bile acids and their natural C24 homologs by human and rat liver fractions. Hepatology 1988;8: 353–7.
-
(1988)
Hepatology
, vol.8
, pp. 353-357
-
-
Kirkpatrick, R.B.1
Green, M.D.2
Hagey, L.R.3
-
183
-
-
0019918861
-
The effect of bile acid structure on the activity of bile acid–co a: Glycine/taurine-n-acetyltransferase
-
Czuba B, Vessey DA. The effect of bile acid structure on the activity of bile acid–Co A: glycine/taurine-N-acetyltransferase. J Biol Chem 1982;257: 8761–5.
-
(1982)
J Biol Chem
, vol.257
, pp. 8761-8765
-
-
Czuba, B.1
Vessey, D.A.2
-
184
-
-
0029128368
-
Reduced expression of kan-1 (Encoding putative bile acid-co a-amino acid nacyltransferase) m rnain livers of rats after partial hepatectomy and during sepsis
-
Furutani M, Arii S, Higashitsuji H, et al. Reduced expression of kan-1 (encoding putative bile acid-Co A-amino acid Nacyltransferase) m RNAin livers of rats after partial hepatectomy and during sepsis. Biochem J 1995;311: 203–8.
-
(1995)
Biochem J
, vol.311
, pp. 203-208
-
-
Furutani, M.1
Arii, S.2
Higashitsuji, H.3
-
185
-
-
0013800926
-
Acute taurine depletion and maximal ratesof hepatic conjugation and secretion of cholic acid in the dog
-
O’Maille ER, Richards TG, Short AH. Acute taurine depletion and maximal ratesof hepatic conjugation and secretion of cholic acid in the dog. J Physiol (Lond) 1965;180: 67–79.
-
(1965)
J Physiol (Lond)
, vol.180
, pp. 67-79
-
-
O’Maille, E.R.1
Richards, T.G.2
Short, A.H.3
-
186
-
-
0016724125
-
Taurine in the brain and liver of the developing human and monkey
-
Sturman JA, Gaull GE. Taurine in the brain and liver of the developing human and monkey. J Neurochem 1975;25: 831–5.
-
(1975)
J Neurochem
, vol.25
, pp. 831-835
-
-
Sturman, J.A.1
Gaull, G.E.2
-
187
-
-
0014134175
-
The formation of bile acid sulfates: A new pathway of bile acid metabolism in humans
-
Palmer RH. The formation of bile acid sulfates: a new pathway of bile acid metabolism in humans. Proc Natl Acad Sci U S A 1967;58: 1047–50.
-
(1967)
Proc Natl Acad Sci U S A
, vol.58
, pp. 1047-1050
-
-
Palmer, R.H.1
-
188
-
-
0022975338
-
Metabolism of 24-norlithocholic acid in the rat: Formation of hydroxyl- and carboxyl-linked glucuronides and effect on bile flow
-
Shattuck KE, Radominska-Pyrek A, Zimniak P, et al. Metabolism of 24-norlithocholic acid in the rat: formation of hydroxyl- and carboxyl-linked glucuronides and effect on bile flow. Hepatology 1986;6: 869–73.
-
(1986)
Hepatology
, vol.6
, pp. 869-873
-
-
Shattuck, K.E.1
Radominska-Pyrek, A.2
Zimniak, P.3
-
189
-
-
0022530992
-
Glucuronides of monohydroxylated bile acids: Specificity of microsomal glucuronyltransferase for the glucuronidation site, c-3 configuration, and side chain length
-
Radominska-Pyrek A, Zimniak P, Chari M, et al. Glucuronides of monohydroxylated bile acids: specificity of microsomal glucuronyltransferase for the glucuronidation site, C-3 configuration, and side chain length. J Lipid Res 1986;27: 89–101.
-
(1986)
J Lipid Res
, vol.27
, pp. 89-101
-
-
Radominska-Pyrek, A.1
Zimniak, P.2
Chari, M.3
-
190
-
-
0345308545
-
Formation of bile acid glucosides by a sugar nucleotide-independent glucosyltransferase isolated fromhumanliver microsomes
-
Matern H, Matern S, Gerok W. Formation of bile acid glucosides by a sugar nucleotide-independent glucosyltransferase isolated fromhumanliver microsomes. Proc Natl Acad Sci USA 1984;81: 7036–40.
-
(1984)
Proc Natl Acad Sci USA
, vol.81
, pp. 7036-7040
-
-
Matern, H.1
Matern, S.2
Gerok, W.3
-
191
-
-
0023108885
-
Evidence for bile acid glucosides as normal constituents in human urine
-
Marschall HU, Egestad B, Matern H, et al. Evidence for bile acid glucosides as normal constituents in human urine. FEBS Lett 1987;213: 411–14.
-
(1987)
FEBS Lett
, vol.213
, pp. 411-414
-
-
Marschall, H.U.1
Egestad, B.2
Matern, H.3
-
192
-
-
0024307536
-
N-acetylglucosaminides. A new type of bile acid conjugate in man
-
Marschall HU, Egestad B, Matern H, et al. N-acetylglucosaminides. A new type of bile acid conjugate in man. J Biol Chem 1989;264: 12989–93.
-
(1989)
J Biol Chem
, vol.264
, pp. 12989-12993
-
-
Marschall, H.U.1
Egestad, B.2
Matern, H.3
-
193
-
-
0023390432
-
Metabolism of 5-fluorouracil to an n-cholyl-2-fluoro-ß-alanine conjugate: Previously unrecognized role for bile acids in drug conjugation
-
Sweeny DJ, Barnes S, Heggie GD, Diasio RB. Metabolism of 5-fluorouracil to an N-cholyl-2-fluoro-ß-alanine conjugate: previously unrecognized role for bile acids in drug conjugation. Proc Natl Acad Sci U S A 1987;84: 5439–43.
-
(1987)
Proc Natl Acad Sci U S A
, vol.84
, pp. 5439-5443
-
-
Sweeny, D.J.1
Barnes, S.2
Heggie, G.D.3
Diasio, R.B.4
-
194
-
-
0023934756
-
Formation of conjugates of 2-fluoro-ß-alanine and bile acids during the metabolism of 5-fluorouracil and 5-fluoro-2-deoxyuridine in the isolated perfused rat liver
-
Sweeny DJ, Barnes S, Diasio RB. Formation of conjugates of 2-fluoro-ß-alanine and bile acids during the metabolism of 5-fluorouracil and 5-fluoro-2-deoxyuridine in the isolated perfused rat liver. Cancer Res 1988;48: 2010–14.
-
(1988)
Cancer Res
, vol.48
, pp. 2010-2014
-
-
Sweeny, D.J.1
Barnes, S.2
Diasio, R.B.3
-
195
-
-
0023818907
-
19f nmr spectrometry evidence for bile acid conjugates of a- fluoro-ß-alanine as the main biliary metabolites of antineoplastic fluoropyrimidines in humans
-
Malet-Martino MC, Bernadou J, Martino R, Armand JP. 19F NMR spectrometry evidence for bile acid conjugates of a- fluoro-ß-alanine as the main biliary metabolites of antineoplastic fluoropyrimidines in humans. Drug Metab Dispos 1988; 16: 78–84.
-
(1988)
Drug Metab Dispos
, vol.16
, pp. 78-84
-
-
Malet-Martino, M.C.1
Bernadou, J.2
Martino, R.3
Armand, J.P.4
-
196
-
-
0021739393
-
Physicochemical properties of bile acids and their relationship to biological properties: An overview of the problem
-
Hofmann AF, Roda A. Physicochemical properties of bile acids and their relationship to biological properties: an overview of the problem. J Lipid Res 1984;25: 1477–89.
-
(1984)
J Lipid Res
, vol.25
, pp. 1477-1489
-
-
Hofmann, A.F.1
Roda, A.2
-
197
-
-
0021178162
-
Influence of hydroxylation and conjugation of bile salts on their membranedamaging properties – studies on isolated hepatocytes and lipid membrane vesicles
-
Scholmerich J, Becher MS, Schmidt K, et al. Influence of hydroxylation and conjugation of bile salts on their membranedamaging properties – studies on isolated hepatocytes and lipid membrane vesicles. Hepatology 1984;4: 661–6.
-
(1984)
Hepatology
, vol.4
, pp. 661-666
-
-
Scholmerich, J.1
Becher, M.S.2
Schmidt, K.3
-
198
-
-
0004636015
-
Metabolism of ursodeoxycholic acid in normal subjects and in patients with cholestatic liver disease: Biotransformation by conjugation and urinary excretion
-
Paumgartner G, Stiehl A, Gerok W, eds., Dordrecht/Boston/London: Kluwer Academic Publishers
-
Setchell KDR, Balistreri WF, Lin Q, et al. Metabolism of ursodeoxycholic acid in normal subjects and in patients with cholestatic liver disease: biotransformation by conjugation and urinary excretion. In: Paumgartner G, Stiehl A, Gerok W, eds. Bile acids and the hepatobiliary system. From basic science to clinical practice. Dordrecht/Boston/London: Kluwer Academic Publishers, 1993: 245–9.
-
(1993)
Bile Acids and the Hepatobiliary System. from Basic Science to Clinical Practice
, pp. 245-249
-
-
Setchell, K.1
Balistreri, W.F.2
Lin, Q.3
-
199
-
-
0026691374
-
Bile acid nacetylglucosaminidation. In vivo and in vitro evidence for a selective conjugation reaction of 7ß-hydroxylated bile acids in humans
-
Marschall HU, Matern H, Wietholtz H, et al. Bile acid Nacetylglucosaminidation. In vivo and in vitro evidence for a selective conjugation reaction of 7ß-hydroxylated bile acids in humans. J Clin Invest 1992;89: 1981–7.
-
(1992)
J Clin Invest
, vol.89
, pp. 1981-1987
-
-
Marschall, H.U.1
Matern, H.2
Wietholtz, H.3
-
200
-
-
0017121608
-
Synthesis of bile acid monosulphates by the isolated perfused rat kidney
-
Summerfield JA, Gollan JL, Billing BH. Synthesis of bile acid monosulphates by the isolated perfused rat kidney. Biochem J 1976;156: 339–45.
-
(1976)
Biochem J
, vol.156
, pp. 339-345
-
-
Summerfield, J.A.1
Gollan, J.L.2
Billing, B.H.3
-
201
-
-
0021134465
-
Hepatic and extrahepatic glucuronidation of bile acids in man. Characterization of bile acid uridine 5′-diphosphate glucuronosyltransferase in hepatic, renal, and intestinal microsomes
-
Matern S, Matern H, Farthmann EH, Gerok W. Hepatic and extrahepatic glucuronidation of bile acids in man. Characterization of bile acid uridine 5′-diphosphate glucuronosyltransferase in hepatic, renal, and intestinal microsomes. J Clin Invest 1984;74: 402–10.
-
(1984)
J Clin Invest
, vol.74
, pp. 402-410
-
-
Matern, S.1
Matern, H.2
Farthmann, E.H.3
Gerok, W.4
-
202
-
-
0017594533
-
Enzymatic sulfation of bile salts. Partial purification and characterization of an enzyme from rat liver that catalyzes the sulfation of bile salts
-
Chen LJ, Bolt RJ, Admirand WH. Enzymatic sulfation of bile salts. Partial purification and characterization of an enzyme from rat liver that catalyzes the sulfation of bile salts. Biochim Biophys Acta 1977;480: 219–27.
-
(1977)
Biochim Biophys Acta
, vol.480
, pp. 219-227
-
-
Chen, L.J.1
Bolt, R.J.2
Admirand, W.H.3
-
203
-
-
0018859812
-
Partial purification of a human liver sulphotransferase active towards bile salts
-
Loof L, Hjerten S. Partial purification of a human liver sulphotransferase active towards bile salts. Biochim Biophys Acta 1980;617: 192–204.
-
(1980)
Biochim Biophys Acta
, vol.617
, pp. 192-204
-
-
Loof, L.1
Hjerten, S.2
-
204
-
-
0018614873
-
Enzymatic sulfation of glycochenodeoxycholic acid by tissue fractions fromadult hamsters
-
Barnes S, Burhol PG, Zander R, et al. Enzymatic sulfation of glycochenodeoxycholic acid by tissue fractions fromadult hamsters. J Lipid Res 1979;20: 952–9.
-
(1979)
J Lipid Res
, vol.20
, pp. 952-959
-
-
Barnes, S.1
Burhol, P.G.2
Zander, R.3
-
205
-
-
6844255153
-
Sulfation of bile acids in the fetus
-
Presig R, Bircher J, eds., Gstaad, Switzerland: Edito Cantor Aulendorf
-
Watkins JB, Goldstein E, Coryer R. Sulfation of bile acids in the fetus. In: Presig R, Bircher J, eds. The liver, quantitative aspects of structure and function. Gstaad, Switzerland: Edito Cantor Aulendorf, 1974: 249–54.
-
(1974)
The Liver, Quantitative Aspects of Structure and Function
, pp. 249-254
-
-
Watkins, J.B.1
Goldstein, E.2
Coryer, R.3
-
206
-
-
0020511602
-
Placental transport: Bile acid conjugation and sulfation in the fetus
-
Watkins JB. Placental transport: bile acid conjugation and sulfation in the fetus. J Pediatr Gastroenterol Nutr 1983;2: 365–73.
-
(1983)
J Pediatr Gastroenterol Nutr
, vol.2
, pp. 365-373
-
-
Watkins, J.B.1
-
207
-
-
0017580907
-
Evidence for renal control of urinary excretion of bile acids and bile acid sulphates in the cholestatic syndrome
-
Summerfield JA, Cullen J, Barnes S, Billing BH. Evidence for renal control of urinary excretion of bile acids and bile acid sulphates in the cholestatic syndrome. Clin Sci Mol Med 1977;52: 51–65.
-
(1977)
Clin Sci Mol Med
, vol.52
, pp. 51-65
-
-
Summerfield, J.A.1
Cullen, J.2
Barnes, S.3
Billing, B.H.4
-
208
-
-
0001288457
-
Urinary bile acids
-
Setchell KDR, Kritchevsky D, Nair PP, eds., New York: Plenum Press
-
Back P. Urinary bile acids. In: Setchell KDR, Kritchevsky D, Nair PP, eds. The bile acids. New York: Plenum Press, 1988: 405–40.
-
(1988)
The Bile Acids
, pp. 405-440
-
-
Back, P.1
-
209
-
-
0025193141
-
Comprehensive study of the biliarybile acid compositionof patientswith cystic fibrosis and associated liver disease before and after udca administration
-
Nakagawa M, Colombo C, Setchell KDR. Comprehensive study of the biliarybile acid compositionof patientswith cystic fibrosis and associated liver disease before and after UDCA administration. Hepatology 1990;12: 322–34.
-
(1990)
Hepatology
, vol.12
, pp. 322-334
-
-
Nakagawa, M.1
Colombo, C.2
Setchell, K.3
-
210
-
-
0023263549
-
Glucuronidation of 6a-hydroxy bile acids by human liver microsomes
-
Radominska-Pyrek A, Zimniak P, Irshaid YM, et al. Glucuronidation of 6a-hydroxy bile acids by human liver microsomes. J Clin Invest 1987;80: 234–41.
-
(1987)
J Clin Invest
, vol.80
, pp. 234-241
-
-
Radominska-Pyrek, A.1
Zimniak, P.2
Irshaid, Y.M.3
-
211
-
-
0023845861
-
Effective glucuronidation of 6a-hydroxylated bile acids by human hepatic and renal microsomes
-
Parquet M, Pessah M, Sacquet E, et al. Effective glucuronidation of 6a-hydroxylated bile acids by human hepatic and renal microsomes. Eur J Biochem 1988;171: 329–34.
-
(1988)
Eur J Biochem
, vol.171
, pp. 329-334
-
-
Parquet, M.1
Pessah, M.2
Sacquet, E.3
-
212
-
-
0026058319
-
Stable expression of two human udp-glucuronosyltransferase c dnas in v79 cell cultures
-
Fournel-Gigleux S, Sutherland L, Sabolovic N, et al. Stable expression of two human UDP-glucuronosyltransferase c DNAs in V79 cell cultures. Mol Pharmacol 1991;39: 177–83.
-
(1991)
Mol Pharmacol
, vol.39
, pp. 177-183
-
-
Fournel-Gigleux, S.1
Sutherland, L.2
Sabolovic, N.3
-
213
-
-
0023686122
-
Isolationofbile acid glucosides and n-acetylglucosaminides from human urine by ion-exchange chromatography and reversed-phase high- performance liquid chromatography
-
Marschall HU, Green G, Egestad B, Sjovall J. Isolationofbile acid glucosides and N-acetylglucosaminides from human urine by ion-exchange chromatography and reversed-phase high- performance liquid chromatography. J Chromatogr 1988;452: 459–68.
-
(1988)
J Chromatogr
, vol.452
, pp. 459-468
-
-
Marschall, H.U.1
Green, G.2
Egestad, B.3
Sjovall, J.4
-
214
-
-
0025847114
-
Urinary excretion of bile acid glucosides and glucuronides in extrahepatic cholestasis
-
Wietholtz H, Marschall HU, Reuschenbach R, et al. Urinary excretion of bile acid glucosides and glucuronides in extrahepatic cholestasis. Hepatology 1991;13: 656–62.
-
(1991)
Hepatology
, vol.13
, pp. 656-662
-
-
Wietholtz, H.1
Marschall, H.U.2
Reuschenbach, R.3
-
215
-
-
0023251004
-
Formation of bile acid glucosides and dolichyl phosphoglucose by microsomal glucosyltransferases in liver, kidney and intestine of man
-
Matern H, Matern S. Formation of bile acid glucosides and dolichyl phosphoglucose by microsomal glucosyltransferases in liver, kidney and intestine of man. Biochim Biophys Acta 1987;921: 1–6.
-
(1987)
Biochim Biophys Acta
, vol.921
, pp. 1-6
-
-
Matern, H.1
Matern, S.2
-
216
-
-
0001270186
-
Fecal bile acids
-
Setchell KDR, Kritchevsky D, Nair PP, eds., New York: Plenum Press
-
Setchell KDR, Street JM, Sjovall J. Fecal bile acids. In: Setchell KDR, Kritchevsky D, Nair PP, eds. The bile acids: methods and applications. New York: Plenum Press, 1988: 441–570.
-
(1988)
The Bile Acids: Methods and Applications
, pp. 441-570
-
-
Setchell, K.1
Street, J.M.2
Sjovall, J.3
-
217
-
-
0014251125
-
Degradation of bile salts by humanintestinal bacteria
-
Hill MJ, Drasar BS. Degradation of bile salts by humanintestinal bacteria. Gut 1968;9: 22–7.
-
(1968)
Gut
, vol.9
, pp. 22-27
-
-
Hill, M.J.1
Drasar, B.S.2
-
218
-
-
0015693607
-
Microbiological transformation of bile acids
-
Hayakawa S. Microbiological transformation of bile acids. Adv Lipid Res 1973;11: 143–92.
-
(1973)
Adv Lipid Res
, vol.11
, pp. 143-192
-
-
Hayakawa, S.1
-
220
-
-
77956838415
-
Metabolism of bile acids in intestinal microflora
-
Danielsson H, Sjovall J, eds., Amsterdam: Elsevier Science
-
Hylemon PB. Metabolism of bile acids in intestinal microflora. In: Danielsson H, Sjovall J, eds. Sterols and bile acids. Amsterdam: Elsevier Science, 1985: 331–43.
-
(1985)
Sterols and Bile Acids
, pp. 331-343
-
-
Hylemon, P.B.1
-
221
-
-
84927979966
-
Intraluminal bile acid composition along the entire length of the humanintestine
-
Setchell KDR, O’Connell NC, Cummings J. Intraluminal bile acid composition along the entire length of the humanintestine. FASEB J. 1993;A722.
-
(1993)
FASEB J
-
-
Setchell, K.1
O’Connell, N.C.2
Cummings, J.3
-
222
-
-
0015551136
-
Savage d. Patterns of bile acids and microflora in the human small intestine. i. bile acids
-
Mallory A, Kern F Jr, Smith J, Savage D. Patterns of bile acids and microflora in the human small intestine. I. Bile acids. Gastroenterology 1973;64: 26–33.
-
(1973)
Gastroenterology
, vol.64
, pp. 26-33
-
-
Mallory, A.1
Kern, F.2
Smith, J.3
-
223
-
-
0015538186
-
Patterns of bile acids and microflora in the human small intestine. Ii. microflora
-
Mallory A, Savage D, Kern F Jr, Smith JG. Patterns of bile acids and microflora in the human small intestine. II. Microflora. Gastroenterology 1973;64: 34–42.
-
(1973)
Gastroenterology
, vol.64
, pp. 34-42
-
-
Mallory, A.1
Savage, D.2
Kern, F.3
Smith, J.G.4
-
224
-
-
0015847127
-
Postprandial concentrations of free and conjugated bile acids down the length of the normal human small intestine
-
Northfield TC, McColl I. Postprandial concentrations of free and conjugated bile acids down the length of the normal human small intestine. Gut 1973;14: 513–18.
-
(1973)
Gut
, vol.14
, pp. 513-518
-
-
Northfield, T.C.1
McColl, I.2
-
225
-
-
0022466827
-
Influence of the amino acid moiety on deconjugation of bile acid amidates by cholylglycine hydrolase or human fecal cultures
-
Huijghebaert SM, Hofmann AF. Influence of the amino acid moiety on deconjugation of bile acid amidates by cholylglycine hydrolase or human fecal cultures. J Lipid Res 1986;27: 742–52.
-
(1986)
J Lipid Res
, vol.27
, pp. 742-752
-
-
Huijghebaert, S.M.1
Hofmann, A.F.2
-
226
-
-
0021742094
-
Substrate specificity of cholylglycine hydrolase for the hydrolysis of bile acid conjugates
-
Batta AK, Salen G, Shefer S. Substrate specificity of cholylglycine hydrolase for the hydrolysis of bile acid conjugates. J Biol Chem 1984;259: 15035–9.
-
(1984)
J Biol Chem
, vol.259
, pp. 15035-15039
-
-
Batta, A.K.1
Salen, G.2
Shefer, S.3
-
227
-
-
0024397473
-
Mechanism of intestinal formation of deoxycholic acid from cholic acid in humans: Evidence for a 3-oxo-Δ4-steroid intermediate
-
Bjorkhem I, Einarsson K, Melone P, Hylemon P. Mechanism of intestinal formation of deoxycholic acid from cholic acid in humans: evidence for a 3-oxo-Δ4-steroid intermediate. J Lipid Res 1989;30: 1033–9.
-
(1989)
J Lipid Res
, vol.30
, pp. 1033-1039
-
-
Bjorkhem, I.1
Einarsson, K.2
Melone, P.3
Hylemon, P.4
-
228
-
-
84889541936
-
Biochemistry and genetics of intestinal bile salt metabolism
-
Paumgartner G, Gerok W, eds, Dordrecht/ Boston/London: Kluwer Academic Publishers
-
Hylemon PB. Biochemistry and genetics of intestinal bile salt metabolism. In: Paumgartner G, Gerok W, eds. Bile acids as therapeutic agents. From basic science to clinical practice. Dordrecht/ Boston/London: Kluwer Academic Publishers, 1990: 1–11.
-
(1990)
Bile Acids as Therapeutic Agents. from Basic Science to Clinical Practice
, pp. 1-11
-
-
Hylemon, P.B.1
-
229
-
-
0027288794
-
Characterization of the bai h gene encoding a bile acid-inducible nadh: Flavin oxidoreductase from eubacterium sp. strain vpi 12708
-
Franklund CV, Baron SF, Hylemon PB. Characterization of the bai H gene encoding a bile acid-inducible NADH: flavin oxidoreductase from Eubacterium sp. strain VPI 12708. J Bacteriol 1993;175: 3002–12.
-
(1993)
J Bacteriol
, vol.175
, pp. 3002-3012
-
-
Franklund, C.V.1
Baron, S.F.2
Hylemon, P.B.3
-
230
-
-
0024373642
-
Regulation of bile acid synthesis. Iii. correlation between biliary bile salt hydrophobicity index and the activities of enzymes regulating cholesterol and bile acid synthesis in the rat
-
Heuman DM, Hylemon PB, Vlahcevic ZR. Regulation of bile acid synthesis. III. Correlation between biliary bile salt hydrophobicity index and the activities of enzymes regulating cholesterol and bile acid synthesis in the rat. J Lipid Res 1989;30: 1161–71.
-
(1989)
J Lipid Res
, vol.30
, pp. 1161-1171
-
-
Heuman, D.M.1
Hylemon, P.B.2
Vlahcevic, Z.R.3
-
231
-
-
0024361959
-
Role of primary and secondary bile acids as feedback inhibitors of bile acid synthesis in the rat in vivo
-
Stange EF, Scheibner J, Ditschuneit H. Role of primary and secondary bile acids as feedback inhibitors of bile acid synthesis in the rat in vivo. J Clin Invest 1989;84: 173–80.
-
(1989)
J Clin Invest
, vol.84
, pp. 173-180
-
-
Stange, E.F.1
Scheibner, J.2
Ditschuneit, H.3
-
232
-
-
0019979314
-
Diurnal changes in serum unconjugated bile acids in normal man
-
Setchell KDR, Lawson AM, Blackstock EJ, Murphy GM. Diurnal changes in serum unconjugated bile acids in normal man. Gut 1982;23: 637–42.
-
(1982)
Gut
, vol.23
, pp. 637-642
-
-
Setchell, K.1
Lawson, A.M.2
Blackstock, E.J.3
Murphy, G.M.4
-
233
-
-
0022377742
-
Serum unconjugated bile acids: Qualitative and quantitative profiles in ileal resection and bacterial overgrowth
-
Setchell KDR, Harrison DL, Gilbert JM, Mupthy GM. Serum unconjugated bile acids: qualitative and quantitative profiles in ileal resection and bacterial overgrowth. Clin Chim Acta 1985; 152: 297–306.
-
(1985)
Clin Chim Acta
, vol.152
, pp. 297-306
-
-
Setchell, K.1
Harrison, D.L.2
Gilbert, J.M.3
Mupthy, G.M.4
-
234
-
-
0031888585
-
A method for the quantitation of conjugated bile acids in dried blood spots using electrospray ionization-mass spectrometry
-
Mills KA, Mushtaq I, Johnson AW, et al. A method for the quantitation of conjugated bile acids in dried blood spots using electrospray ionization-mass spectrometry. Pediatr Res 1998;43: 361–8.
-
(1998)
Pediatr Res
, vol.43
, pp. 361-368
-
-
Mills, K.A.1
Mushtaq, I.2
Johnson, A.W.3
-
235
-
-
0023193394
-
Familial giant cell hepatitis associated with synthesis of 3ß, 7a-dihydroxyand 3ß, 7a, 12a-trihydroxy-5-cholenoic acids
-
Clayton PT, Leonard JV, Lawson AM, et al. Familial giant cell hepatitis associated with synthesis of 3ß, 7a-dihydroxyand 3ß, 7a, 12a-trihydroxy-5-cholenoic acids. J Clin Invest 1987;79: 1031–8.
-
(1987)
J Clin Invest
, vol.79
, pp. 1031-1038
-
-
Clayton, P.T.1
Leonard, J.V.2
Lawson, A.M.3
-
236
-
-
0028942689
-
Familial giant cell hepatitis with low bile acid concentrations and increased urinary excretion of specific bile alcohols: A new inborn error of bile acid synthesis?
-
Clayton PT, Casteels M, Mieli-Vergani G, Lawson AM. Familial giant cell hepatitis with low bile acid concentrations and increased urinary excretion of specific bile alcohols: a new inborn error of bile acid synthesis? Pediatr Res 1995;37: 424–31.
-
(1995)
Pediatr Res
, vol.37
, pp. 424-431
-
-
Clayton, P.T.1
Casteels, M.2
Mieli-Vergani, G.3
Lawson, A.M.4
-
237
-
-
0002731325
-
Identification of a unique inborn error in bile acid conjugation involving a deficiency in amidation
-
Paumgartner G, Stiehl A, Gerok W, eds., Dordrecht/Boston/London: Kluwer Academic Publishers
-
Setchell KDR, Heubi JE, O’Connell NC, et al. Identification of a unique inborn error in bile acid conjugation involving a deficiency in amidation. In: Paumgartner G, Stiehl A, Gerok W, eds. Bile acids in hepatobiliary diseases: basic research and clinical application. Dordrecht/Boston/London: Kluwer Academic Publishers, 1997: 43–7.
-
(1997)
Bile Acids in Hepatobiliary Diseases: Basic Research and Clinical Application
, pp. 43-47
-
-
Setchell, K.1
Heubi, J.E.2
O’Connell, N.C.3
-
238
-
-
4344711835
-
Congenital defects in bile acid synthesis cause a spectrum of diseases manifest as severe cholestasis, neurological disease, and fat-soluble vitamin malabsorption
-
Northfield TC, Ahmed H, Jazwari R, Zentler-Munro P, eds., Dordrecht: Kluwer Academic Publishers
-
Setchell KDR, O’Connell NC, Squires RH, Heubi JE. Congenital defects in bile acid synthesis cause a spectrum of diseases manifest as severe cholestasis, neurological disease, and fat-soluble vitamin malabsorption. In: Northfield TC, Ahmed H, Jazwari R, Zentler-Munro P, eds. Bile acids in hepatobiliary disease. Dordrecht: Kluwer Academic Publishers, 1999: 55–63.
-
(1999)
Bile Acids in Hepatobiliary Disease
, pp. 55-63
-
-
Setchell, K.1
O’Connell, N.C.2
Squires, R.H.3
Heubi, J.E.4
-
239
-
-
85047684249
-
Human cholesterol 7a- hydroxylase (Cyp7a1) deficiency has a hypercholesterolemic phenotype
-
Pullinger CR, Eng C, Salen G, et al. Human cholesterol 7a- hydroxylase (CYP7A1) deficiency has a hypercholesterolemic phenotype. J Clin Invest 2002;110: 109–17.
-
(2002)
J Clin Invest
, vol.110
, pp. 109-117
-
-
Pullinger, C.R.1
Eng, C.2
Salen, G.3
-
240
-
-
0001803448
-
Congenital bile salt deficiency associated with 28 years of intractable constipation
-
Paumgartner G, Stiehl A, eds, Lancaster, U. K.: MTP Press
-
Iser J, Dowling R, Murphy G. Congenital bile salt deficiency associated with 28 years of intractable constipation. In: Paumgartner G, Stiehl A, eds. Bile acid metabolism in health and disease. Lancaster, U. K.: MTP Press, 1977: 231–4.
-
(1977)
Bile Acid Metabolism in Health and Disease
, pp. 231-234
-
-
Iser, J.1
Dowling, R.2
Murphy, G.3
-
242
-
-
4243461511
-
Chronic hepatitis in a 10 yr old due to an inborn error in bile acid synthesis – diagnosis and treatment with oral bile acid
-
Setchell KDR, Flick R, Watkins JB, Piccoli DA. Chronic hepatitis in a 10 yr old due to an inborn error in bile acid synthesis – diagnosis and treatment with oral bile acid. Gastroenterology 1990;98: A578.
-
(1990)
Gastroenterology
, vol.98
-
-
Setchell, K.1
Flick, R.2
Watkins, J.B.3
Piccoli, D.A.4
-
243
-
-
33745740400
-
Defects in bile acid synthesis – diagnosis and treatment
-
Setchell KDR, Heubi J. Defects in bile acid synthesis – diagnosis and treatment. J Pediatr Gastroenterol Nutr 2006;43(suppl 1): S17–22.
-
(2006)
J Pediatr Gastroenterol Nutr
, vol.43
, Issue.1
, pp. S17-S22
-
-
Setchell, K.1
Heubi, J.2
-
244
-
-
0027964355
-
A new cause of progressive intrahepatic cholestasis: 3ß-hydroxy-c27- steroid dehydrogenase/isomerase deficiency
-
Jacquemin E, Setchell KDR, O’Connell NC, et al. A new cause of progressive intrahepatic cholestasis: 3ß-hydroxy-C27- steroid dehydrogenase/isomerase deficiency. J Pediatr 1994;125: 379–84.
-
(1994)
J Pediatr
, vol.125
, pp. 379-384
-
-
Jacquemin, E.1
Setchell, K.2
O’Connell, N.C.3
-
245
-
-
0008699862
-
Oral bile acid therapy in the treatment of inborn errors in bile acid synthesis associated with liver disease
-
Paumgartner G, Stiehl A, Gerok W, eds, Freiburg, Germany: Kluwer Academic Publishers
-
Setchell KDR, Balistreri WF, Piccoli DA, Clerici C. Oral bile acid therapy in the treatment of inborn errors in bile acid synthesis associated with liver disease. In: Paumgartner G, Stiehl A, Gerok W, eds. Falk symposium no. 58. Bile acids as therapeutic agents: from basic science to clinical practice. Freiburg, Germany: Kluwer Academic Publishers, 1990: 367–73.
-
(1990)
Falk Symposium No. 58. Bile Acids as Therapeutic Agents: From Basic Science to Clinical Practice
, pp. 367-373
-
-
Setchell, K.1
Balistreri, W.F.2
Piccoli, D.A.3
Clerici, C.4
-
247
-
-
0026508548
-
3ß-hydroxy- Δ5-c27-steroid dehydrogenase deficiency; effect of chenodeoxycholic acid therapy on liver histology
-
Horslen SP, Lawson AM, Malone M, Clayton PT. 3ß-hydroxy- Δ5-C27-steroid dehydrogenase deficiency; Effect of chenodeoxycholic acid therapy on liver histology. J Inherit Metab Dis 1992;15: 38–46.
-
(1992)
J Inherit Metab Dis
, vol.15
, pp. 38-46
-
-
Horslen, S.P.1
Lawson, A.M.2
Malone, M.3
Clayton, P.T.4
-
250
-
-
0025834646
-
Bile acids and bile alcohols in a child with hepatic 3ß-hydroxy-Δ5-c27-steroid dehydrogenase deficiency: Effects of chenodeoxycholic acid treatment
-
Ichimiya H, Egestad B, Nazer H, et al. Bile acids and bile alcohols in a child with hepatic 3ß-hydroxy-Δ5-C27-steroid dehydrogenase deficiency: effects of chenodeoxycholic acid treatment. J Lipid Res 1991;32: 829–41.
-
(1991)
J Lipid Res
, vol.32
, pp. 829-841
-
-
Ichimiya, H.1
Egestad, B.2
Nazer, H.3
-
251
-
-
0022354303
-
Fast atom bombardment mass spectrometry in the diagnosis of cerebrotendinous xanthomatosis
-
Egestad B, Pettersson P, Skrede S, Sjovall J. Fast atom bombardment mass spectrometry in the diagnosis of cerebrotendinous xanthomatosis. Scand J Clin Lab Invest 1985;45: 443–6.
-
(1985)
Scand J Clin Lab Invest
, vol.45
, pp. 443-446
-
-
Egestad, B.1
Pettersson, P.2
Skrede, S.3
Sjovall, J.4
-
252
-
-
0025602665
-
Lack of 3ß- hydroxy-Δ5-c27-steroid dehydrogenase/isomerase in fibroblasts from a child with urinary excretion of 3ß-hydroxy-Δ5- bile acids. A new inborn error of metabolism
-
Buchmann MS, Kvittingen EA, Nazer H, et al. Lack of 3ß- hydroxy-Δ5-C27-steroid dehydrogenase/isomerase in fibroblasts from a child with urinary excretion of 3ß-hydroxy-Δ5- bile acids. A new inborn error of metabolism. J Clin Invest 1990;86: 2034–7.
-
(1990)
J Clin Invest
, vol.86
, pp. 2034-2037
-
-
Buchmann, M.S.1
Kvittingen, E.A.2
Nazer, H.3
-
253
-
-
0010633655
-
Transport of taurine conjugates of 7a-hydroxy-3-oxo-4-cholenoic acid and 3ß, 7a- dihydroxy-5-cholenoic acid in rat liver plasma membrane vesicles
-
Van Berge-Henegouwen GP, Van Hock B, De Groote J, et al., eds, Dordrecht/Boston/London: Kluwer Academic Press
-
Stieger B, Zhang J, O’Neill B, et al. Transport of taurine conjugates of 7a-hydroxy-3-oxo-4-cholenoic acid and 3ß, 7a- dihydroxy-5-cholenoic acid in rat liver plasma membrane vesicles. In: Van Berge-Henegouwen GP, Van Hock B, De Groote J, et al., eds. Cholestatic liver diseases. Dordrecht/Boston/London: Kluwer Academic Press, 1994: 82–7.
-
(1994)
Cholestatic Liver Diseases
, pp. 82-87
-
-
Stieger, B.1
Zhang, J.2
O’Neill, B.3
-
254
-
-
0031055345
-
Differential interaction of bile acids from patients with inborn errors of bile acid synthesis with hepatocellular bile acid transporters
-
Stieger B, Zhang J, O’Neill B, et al. Differential interaction of bile acids from patients with inborn errors of bile acid synthesis with hepatocellular bile acid transporters. Eur J Biochem 1997;244: 39–44.
-
(1997)
Eur J Biochem
, vol.244
, pp. 39-44
-
-
Stieger, B.1
Zhang, J.2
O’Neill, B.3
-
255
-
-
0025186139
-
Treatmentof chronic liver disease caused by 3ß-hydroxy-Δ5-c27- steroid dehydrogenase deficiency with chenodeoxycholic acid
-
Ichimiya H, Nazer H, Gunasekaran T, et al. Treatmentof chronic liver disease caused by 3ß-hydroxy-Δ5-C27- steroid dehydrogenase deficiency with chenodeoxycholic acid. Arch Dis Child 1990;65: 1121–4.
-
(1990)
Arch Dis Child
, vol.65
, pp. 1121-1124
-
-
Ichimiya, H.1
Nazer, H.2
Gunasekaran, T.3
-
256
-
-
0026571062
-
Oral bile acid treatment and the patient with zellweger syndrome
-
Setchell KDR, Bragetti P, Zimmer-Nechemias L, et al. Oral bile acid treatment and the patient with Zellweger syndrome. Hepatology 1992;15: 198–207.
-
(1992)
Hepatology
, vol.15
, pp. 198-207
-
-
Setchell, K.1
Bragetti, P.2
Zimmer-Nechemias, L.3
-
257
-
-
0027370660
-
Resolution of liver biopsy alterations in three siblings with bile acid treatment of an inborn error of bile acid metabolism (Δ4–3- oxosteroid 5ß-reductase deficiency)
-
Daugherty CC, Setchell KDR, Heubi JE, Balistreri WF. Resolution of liver biopsy alterations in three siblings with bile acid treatment of an inborn error of bile acid metabolism (Δ4–3- oxosteroid 5ß-reductase deficiency). Hepatology 1993;18: 1096–101.
-
(1993)
Hepatology
, vol.18
, pp. 1096-1101
-
-
Daugherty, C.C.1
Setchell, K.2
Heubi, J.E.3
Balistreri, W.F.4
-
258
-
-
0014156005
-
Enzymatic conversion of a Δ4–3- ketosteroid into a 3a-hydroxy- 5ß steroid: Mechanism and stereochemistry of hydrogen transfer from nadph. bile acids and steroids 190
-
Berseus O, Bjorkhem I. Enzymatic conversion of a Δ4–3- ketosteroid into a 3a-hydroxy- 5ß steroid: mechanism and stereochemistry of hydrogen transfer from NADPH. Bile acids and steroids 190. Eur J Biochem 1967;2: 503–7.
-
(1967)
Eur J Biochem
, vol.2
, pp. 503-507
-
-
Berseus, O.1
Bjorkhem, I.2
-
259
-
-
0014155528
-
Conversion of cholesterol to bile acids in rat: Purification and properties of aΔ4–3-ketosteroid 5ß-reductase and a 3a-hydroxysteroid dehydrogenase
-
Berseus O. Conversion of cholesterol to bile acids in rat: purification and properties of aΔ4–3-ketosteroid 5ß-reductase and a 3a-hydroxysteroid dehydrogenase. Eur J Biochem 1967;2: 493–502.
-
(1967)
Eur J Biochem
, vol.2
, pp. 493-502
-
-
Berseus, O.1
-
260
-
-
0342547204
-
Gene analysis in Δ4–3-oxosteroid 5ß-reductase deficiency [letter]
-
Sumazaki R, Nakamura N, Shoda J, et al. Gene analysis in Δ4–3-oxosteroid 5ß-reductase deficiency [letter]. Lancet 1997; 349: 329.
-
(1997)
Lancet
, vol.349
, pp. 329
-
-
Sumazaki, R.1
Nakamura, N.2
Shoda, J.3
-
261
-
-
0024843429
-
Ketonic bile acids in urine of infants during the neonatal period
-
Wahlen E, Egestad B, Strandvik B, Sjoovall J. Ketonic bile acids in urine of infants during the neonatal period. J Lipid Res 1989;30: 1847–57.
-
(1989)
J Lipid Res
, vol.30
, pp. 1847-1857
-
-
Wahlen, E.1
Egestad, B.2
Strandvik, B.3
Sjoovall, J.4
-
262
-
-
0028177674
-
Whitington pf, et al. Δ4–3- oxosteroid 5ß-reductase deficiency causing neonatal liver failure and hemochromatosis [see comments]
-
Shneider BL, Setchell KD, Whitington PF, et al. Δ4–3- Oxosteroid 5ß-reductase deficiency causing neonatal liver failure and hemochromatosis [see comments]. J Pediatr 1994;124: 234–8.
-
(1994)
J Pediatr
, vol.124
, pp. 234-238
-
-
Shneider, B.L.1
Setchell, K.D.2
-
263
-
-
0026039668
-
Acute infusions of bile salts increase biliary excretion of iron in iron-loaded rats [see comments]
-
Levy P, Dumont M, Brissot P, et al. Acute infusions of bile salts increase biliary excretion of iron in iron-loaded rats [see comments]. Gastroenterology 1991;101: 1673–9.
-
(1991)
Gastroenterology
, vol.101
, pp. 1673-1679
-
-
Levy, P.1
Dumont, M.2
Brissot, P.3
-
264
-
-
0142015659
-
Inborn errors of bile acid biosynthesis: Update on biochemical aspects
-
Hofmann AF, Paumgartner G, Stiehl A, eds., Dordrecht/Boston/London: Kluwer Academic Publishers
-
Setchell KDR, O’Connell NC. Inborn errors of bile acid biosynthesis: update on biochemical aspects. In: Hofmann AF, Paumgartner G, Stiehl A, eds. Bile acids in gastroenterology. Basic and clinical advances. Dordrecht/Boston/London: Kluwer Academic Publishers, 1995: 129–36.
-
(1995)
Bile Acids in Gastroenterology. Basic and Clinical Advances
, pp. 129-136
-
-
Setchell, K.1
O’Connell, N.C.2
-
265
-
-
0023933622
-
Occurrence of 3ß-hydroxy-5- cholestenoic acid, 3ß, 7a-dihydroxy-5-cholestenoic acid, and 7a-hydroxy-3-oxo-4-cholestenoic acid as normal constituents in human blood
-
Axelson M, Mork B, Sjovall J. Occurrence of 3ß-hydroxy-5- cholestenoic acid, 3ß, 7a-dihydroxy-5-cholestenoic acid, and 7a-hydroxy-3-oxo-4-cholestenoic acid as normal constituents in human blood. J Lipid Res 1988;29: 629–41.
-
(1988)
J Lipid Res
, vol.29
, pp. 629-641
-
-
Axelson, M.1
Mork, B.2
Sjovall, J.3
-
266
-
-
0022480435
-
Bile acid biosynthesis during development: Hydroxylation of c27-sterols in human fetal liver
-
Gustafsson J. Bile acid biosynthesis during development: hydroxylation of C27-sterols in human fetal liver. J Lipid Res 1986;27: 801–6.
-
(1986)
J Lipid Res
, vol.27
, pp. 801-806
-
-
Gustafsson, J.1
-
267
-
-
0013418793
-
Bile acids in constipation and diarrhoea
-
Barbara L, Dowling RH, Hofmann AF, eds, Lancaster, U. K.: MTP Press
-
Dowling RH. Bile acids in constipation and diarrhoea. In: Barbara L, Dowling RH, Hofmann AF, eds. Bile acids in gastroenterology. Lancaster, U. K.: MTP Press, 1982: 157–71.
-
(1982)
Bile Acids in Gastroenterology
, pp. 157-171
-
-
Dowling, R.H.1
-
268
-
-
0025883377
-
Abnormallowratio of cholic acid to chenodeoxycholic acid in a cholestatic infantwith severe hypoglycemia
-
Kimura A, Yuge K, Yukizane S, et al. Abnormallowratio of cholic acid to chenodeoxycholic acid in a cholestatic infantwith severe hypoglycemia. J Pediatr Gastroenterol Nutr 1991;12: 383–7.
-
(1991)
J Pediatr Gastroenterol Nutr
, vol.12
, pp. 383-387
-
-
Kimura, A.1
Yuge, K.2
Yukizane, S.3
-
269
-
-
0002436809
-
Bile formation, mechanisms and development
-
Suchy FJ, ed., Philadelphia: Mosby- Year Book
-
Suchy FJ. Bile formation, mechanisms and development. In: Suchy FJ, ed. Liver disease in children. Philadelphia: Mosby- Year Book, 1994.
-
(1994)
Liver Disease in Children
-
-
Suchy, F.J.1
-
272
-
-
0016223896
-
A biochemical abnormality in cerebrotendinous xanthomatosis. Impairment of bile acid biosynthesis associatedwith incomplete degradation of the cholesterol side chain
-
Setoguchi T, Salen G, Tint GS, Mosbach EH. A biochemical abnormality in cerebrotendinous xanthomatosis. Impairment of bile acid biosynthesis associatedwith incomplete degradation of the cholesterol side chain. J Clin Invest 1974;53: 1393–401.
-
(1974)
J Clin Invest
, vol.53
, pp. 1393-1401
-
-
Setoguchi, T.1
Salen, G.2
Tint, G.S.3
Mosbach, E.H.4
-
273
-
-
0025936676
-
Juvenile cataract associated with chronic diarrhea in pediatric cerebrotendinous xanthomatosis [letter]
-
Cruysberg JR, Wevers RA, Tolboom JJ. Juvenile cataract associated with chronic diarrhea in pediatric cerebrotendinous xanthomatosis [letter]. Am J Ophthalmol 1991;112: 606–7.
-
(1991)
Am J Ophthalmol
, vol.112
, pp. 606-607
-
-
Cruysberg, J.R.1
Wevers, R.A.2
Tolboom, J.J.3
-
275
-
-
0025891185
-
Cerebrotendinous xanthomatosis: Clinical and biochemical evaluation of eight patients and review of the literature
-
Kuriyama M, Fujiyama J, Yoshidome H, et al. Cerebrotendinous xanthomatosis: clinical and biochemical evaluation of eight patients and review of the literature. J Neurol Sci 1991;102: 225–32.
-
(1991)
J Neurol Sci
, vol.102
, pp. 225-232
-
-
Kuriyama, M.1
Fujiyama, J.2
Yoshidome, H.3
-
277
-
-
12244306965
-
Mutations in the sterol 27-hydroxylase gene (Cyp27a) cause hepatitis of infancy as well as cerebrotendinous xanthomatosis
-
Clayton PT, Verrips A, Sistermans E, et al. Mutations in the sterol 27-hydroxylase gene (CYP27A) cause hepatitis of infancy as well as cerebrotendinous xanthomatosis. J Inherit Metab Dis 2002;25: 501–13.
-
(2002)
J Inherit Metab Dis
, vol.25
, pp. 501-513
-
-
Clayton, P.T.1
Verrips, A.2
Sistermans, E.3
-
278
-
-
16844368489
-
Mutation in the sterol 27-hydroxylase gene associated with fatal cholestasis in infancy
-
von Bahr S, Bjorkhem I, Van’t Hooft F, et al. Mutation in the sterol 27-hydroxylase gene associated with fatal cholestasis in infancy. J Pediatr Gastroenterol Nutr 2005;40: 481–6.
-
(2005)
J Pediatr Gastroenterol Nutr
, vol.40
, pp. 481-486
-
-
Von Bahr, S.1
Bjorkhem, I.2
Van’T Hooft, F.3
-
279
-
-
0015910239
-
The metabolism of cholestanol, cholesterol, and bile acids in cerebrotendinous xanthomatosis
-
Salen G, Grundy SM. The metabolism of cholestanol, cholesterol, and bile acids in cerebrotendinous xanthomatosis. J Clin Invest 1973;52: 2822–35.
-
(1973)
J Clin Invest
, vol.52
, pp. 2822-2835
-
-
Salen, G.1
Grundy, S.M.2
-
280
-
-
0016546665
-
Chenodeoxycholic acid inhibits increased cholesterol and cholestanol synthesis in patients with cerebrotendinous xanthomatosis
-
Salen G, Meriwether TW, Nicolau G. Chenodeoxycholic acid inhibits increased cholesterol and cholestanol synthesis in patients with cerebrotendinous xanthomatosis. Biochem Med 1975;14: 57–74.
-
(1975)
Biochem Med
, vol.14
, pp. 57-74
-
-
Salen, G.1
Meriwether, T.W.2
Nicolau, G.3
-
281
-
-
0018886395
-
Cerebrotendinous xanthomatosis: A defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid
-
Oftebro H, Bjorkhem I, Skrede S, et al. Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid. J Clin Invest 1980;65: 1418–30.
-
(1980)
J Clin Invest
, vol.65
, pp. 1418-1430
-
-
Oftebro, H.1
Bjorkhem, I.2
Skrede, S.3
-
282
-
-
0020287360
-
Cerebrotendinous xanthomatosis: Reduced serum 26-hydroxycholesterol
-
Javitt NB, Kok E, Cohen B, Burstein S. Cerebrotendinous xanthomatosis: reduced serum 26-hydroxycholesterol. J Lipid Res 1982;23: 627–30.
-
(1982)
J Lipid Res
, vol.23
, pp. 627-630
-
-
Javitt, N.B.1
Kok, E.2
Cohen, B.3
Burstein, S.4
-
283
-
-
0020586748
-
Diagnosis of cerebrotendinous xanthomatosis (Ctx) and effect of chenodeoxycholic acid therapy by analysis of urine using capillary gas chromatography
-
Wolthers BG, Volmer M, van der Molen J, et al. Diagnosis of cerebrotendinous xanthomatosis (CTX) and effect of chenodeoxycholic acid therapy by analysis of urine using capillary gas chromatography. Clin Chim Acta 1983;131: 53–65.
-
(1983)
Clin Chim Acta
, vol.131
, pp. 53-65
-
-
Wolthers, B.G.1
Volmer, M.2
Van Der Molen, J.3
-
284
-
-
0022641883
-
Bile alcohol profiles in bile, urine, and feces of a patient with cerebrotendinous xanthomatosis
-
Shimazu K, Kuwabara M, Yoshii M, et al. Bile alcohol profiles in bile, urine, and feces of a patient with cerebrotendinous xanthomatosis. J Biochem (Tokyo) 1986;99: 477–83.
-
(1986)
J Biochem (Tokyo)
, vol.99
, pp. 477-483
-
-
Shimazu, K.1
Kuwabara, M.2
Yoshii, M.3
-
285
-
-
0021810422
-
Biosynthesis of bile acids in cerebrotendinous xanthomatosis. Relationship of bile acid pool sizes and synthesis rates to hydroxylations at c-12, c-25, and c-26
-
Salen G, Shefer S, Tint GS, et al. Biosynthesis of bile acids in cerebrotendinous xanthomatosis. Relationship of bile acid pool sizes and synthesis rates to hydroxylations at C-12, C-25, and C-26. J Clin Invest 1985;76: 744–51.
-
(1985)
J Clin Invest
, vol.76
, pp. 744-751
-
-
Salen, G.1
Shefer, S.2
Tint, G.S.3
-
286
-
-
0028227211
-
Identification of new mutations in sterol 27-hydroxylase gene in japanese patients with cerebrotendinous xanthomatosis (Ctx)
-
Kim KS, Kubota S, Kuriyama M, et al. Identification of new mutations in sterol 27-hydroxylase gene in Japanese patients with cerebrotendinous xanthomatosis (CTX). J Lipid Res 1994;35: 1031–9.
-
(1994)
J Lipid Res
, vol.35
, pp. 1031-1039
-
-
Kim, K.S.1
Kubota, S.2
Kuriyama, M.3
-
287
-
-
0028097330
-
Cerebrotendinous xanthomatosis in the israeli druze: Molecular genetics and phenotypic characteristics
-
Leitersdorf E, Safadi R, Meiner V, et al. Cerebrotendinous xanthomatosis in the Israeli Druze: molecular genetics and phenotypic characteristics. Am J Hum Genet 1994;55: 907–15.
-
(1994)
Am J Hum Genet
, vol.55
, pp. 907-915
-
-
Leitersdorf, E.1
Safadi, R.2
Meiner, V.3
-
288
-
-
0028013348
-
Premature termination codon at the sterol 27-hydroxylase gene causes cerebrotendinous xanthomatosis in an afrikaner family
-
Meiner V, Marais DA, Reshef A, et al. Premature termination codon at the sterol 27-hydroxylase gene causes cerebrotendinous xanthomatosis in an Afrikaner family. Hum Mol Genet 1994;3: 193–4.
-
(1994)
Hum Mol Genet
, vol.3
, pp. 193-194
-
-
Meiner, V.1
Marais, D.A.2
Reshef, A.3
-
289
-
-
0028206666
-
Molecular genetics of cerebrotendinous xanthomatosis in jews of north african origin
-
Reshef A, Meiner V, Berginer VM, Leitersdorf E. Molecular genetics of cerebrotendinous xanthomatosis in Jews of North African origin. J Lipid Res 1994;35: 478–83.
-
(1994)
J Lipid Res
, vol.35
, pp. 478-483
-
-
Reshef, A.1
Meiner, V.2
Berginer, V.M.3
Leitersdorf, E.4
-
290
-
-
0028798653
-
Premature termination codon at the sterol 27-hydroxylase gene causes cerebrotendinous xanthomatosis in a french family
-
Segev H, Reshef A, Clavey V, et al. Premature termination codon at the sterol 27-hydroxylase gene causes cerebrotendinous xanthomatosis in a French family. Hum Genet 1995;95: 238–40.
-
(1995)
Hum Genet
, vol.95
, pp. 238-240
-
-
Segev, H.1
Reshef, A.2
Clavey, V.3
-
291
-
-
0030023244
-
Cerebrotendinous xanthomatosis: A family study of sterol 27-hydroxylase mutations and pharmacotherapy
-
Watts GF, Mitchell WD, Bending JJ, et al. Cerebrotendinous xanthomatosis: a family study of sterol 27-hydroxylase mutations and pharmacotherapy. Q J Med 1996;89: 55–63.
-
(1996)
Q J Med
, vol.89
, pp. 55-63
-
-
Watts, G.F.1
Mitchell, W.D.2
Bending, J.J.3
-
292
-
-
0030589062
-
Genetic analysis of a japanese cerebrotendinous xanthomatosis family: Identification of a novel mutation in the adrenodoxin binding region of the cyp27 gene
-
Chen W, Kubota S, Nishimura Y, et al. Genetic analysis of a Japanese cerebrotendinous xanthomatosis family: identification of a novel mutation in the adrenodoxin binding region of the CYP27 gene. Biochim Biophys Acta 1996;1317: 119–26.
-
(1996)
Biochim Biophys Acta
, vol.1317
, pp. 119-126
-
-
Chen, W.1
Kubota, S.2
Nishimura, Y.3
-
293
-
-
8944245008
-
Cerebrotendinous xanthomatosis caused by two new mutations of the sterol-27- hydroxylase gene that disrupt m rna splicing
-
Garuti R, Lelli N, Barozzini M, et al. Cerebrotendinous xanthomatosis caused by two new mutations of the sterol-27- hydroxylase gene that disrupt m RNA splicing. J Lipid Res 1996;37: 1459–67.
-
(1996)
J Lipid Res
, vol.37
, pp. 1459-1467
-
-
Garuti, R.1
Lelli, N.2
Barozzini, M.3
-
294
-
-
0029971502
-
Japanese tripletswith cerebrotendinous xanthomatosis are homozygous for a mutant gene coding for the sterol 27-hydroxylase (Arg441trp)
-
Nagai Y, Hirano M, Mori T, et al. Japanese tripletswith cerebrotendinous xanthomatosis are homozygous for a mutant gene coding for the sterol 27-hydroxylase (Arg441Trp). Neurology 1996;46: 571–4.
-
(1996)
Neurology
, vol.46
, pp. 571-574
-
-
Nagai, Y.1
Hirano, M.2
Mori, T.3
-
295
-
-
0029961657
-
A novel mutation in the cytochrome p450(27) (cyp27) gene caused cerebrotendinous xanthomatosis in a japanese family
-
Okuyama E, Tomita S, Takeuchi H, Ichikawa Y. A novel mutation in the cytochrome P450(27) (CYP27) gene caused cerebrotendinous xanthomatosis in a Japanese family. J Lipid Res 1996;37: 631–9.
-
(1996)
J Lipid Res
, vol.37
, pp. 631-639
-
-
Okuyama, E.1
Tomita, S.2
Takeuchi, H.3
Ichikawa, Y.4
-
296
-
-
0029908729
-
Two new mutations in the sterol 27-hydroxylase gene in two families lead to cerebrotendinous xanthomatosis
-
Verrips A, Steenbergen-Spanjers GC, Luyten JA, et al. Two new mutations in the sterol 27-hydroxylase gene in two families lead to cerebrotendinous xanthomatosis. Hum Genet 1996;98: 735–7.
-
(1996)
Hum Genet
, vol.98
, pp. 735-737
-
-
Verrips, A.1
Steenbergen-Spanjers, G.C.2
Luyten, J.A.3
-
297
-
-
0030842375
-
Exon skipping in the sterol 27-hydroxylase gene leads to cerebrotendinous xanthomatosis
-
Verrips A, Steenbergen-Spanjers GC, Luyten JA, et al. Exon skipping in the sterol 27-hydroxylase gene leads to cerebrotendinous xanthomatosis. Hum Genet 1997;100: 284–6.
-
(1997)
Hum Genet
, vol.100
, pp. 284-286
-
-
Verrips, A.1
Steenbergen-Spanjers, G.C.2
Luyten, J.A.3
-
298
-
-
0030957251
-
Novel homozygous and compound heterozygous mutations of sterol 27-hydroxylase gene (Cyp27) cause cerebrotendinous xanthomatosis in three japanese patients from two unrelated families
-
Chen W, Kubota S, Kim KS, et al. Novel homozygous and compound heterozygous mutations of sterol 27-hydroxylase gene (CYP27) cause cerebrotendinous xanthomatosis in three Japanese patients from two unrelated families. J Lipid Res 1997;38: 870–9.
-
(1997)
J Lipid Res
, vol.38
, pp. 870-879
-
-
Chen, W.1
Kubota, S.2
Kim, K.S.3
-
299
-
-
0031037412
-
A novel mutation in the sterol 27-hydroxylase gene of a pakistani family with autosomal recessive cerebrotendinous xanthomatosis
-
Ahmed MS, Afsar S, Hentati A, et al. A novel mutation in the sterol 27-hydroxylase gene of a Pakistani family with autosomal recessive cerebrotendinous xanthomatosis. Neurology 1997;48: 258–60.
-
(1997)
Neurology
, vol.48
, pp. 258-260
-
-
Ahmed, M.S.1
Afsar, S.2
Hentati, A.3
-
300
-
-
0031921532
-
Alternative pre-m rna splicing of the sterol 27-hydroxylase gene (Cyp27) caused by a g to a mutation at the last nucleotide of exon 6 in a patient with cerebrotendinous xanthomatosis (ctx)
-
Chen W, Kubota S, Seyama Y. Alternative pre-m RNA splicing of the sterol 27-hydroxylase gene (CYP27) caused by a G to A mutation at the last nucleotide of exon 6 in a patient with cerebrotendinous xanthomatosis (CTX). J Lipid Res 1998;39: 509–17.
-
(1998)
J Lipid Res
, vol.39
, pp. 509-517
-
-
Chen, W.1
Kubota, S.2
Seyama, Y.3
-
301
-
-
0031721752
-
Genetic analysis enables definite and rapid diagnosis of cerebrotendinous xanthomatosis
-
Chen W, Kubota S, Teramoto T, et al. Genetic analysis enables definite and rapid diagnosis of cerebrotendinous xanthomatosis. Neurology 1998;51: 865–7.
-
(1998)
Neurology
, vol.51
, pp. 865-867
-
-
Chen, W.1
Kubota, S.2
Teramoto, T.3
-
302
-
-
0032584276
-
Silent nucleotide substitution in the sterol 27-hydroxylase gene (Cyp27) leads to alternative pre-m rna splicing by activating a cryptic 5′ splice site at the mutant codon in cerebrotendinous xanthomatosis patients
-
Chen W, Kubota S, Teramoto T, et al. Silent nucleotide substitution in the sterol 27-hydroxylase gene (CYP27) leads to alternative pre-m RNA splicing by activating a cryptic 5′ splice site at the mutant codon in cerebrotendinous xanthomatosis patients. Biochemistry 1998;37: 4420–8.
-
(1998)
Biochemistry
, vol.37
, pp. 4420-4428
-
-
Chen, W.1
Kubota, S.2
Teramoto, T.3
-
303
-
-
0032774671
-
Mutations producing premature termination of translation and an amino acid substitution in the sterol 27-hydroxylase gene cause cerebrotendinous xanthomatosis associated with parkinsonism
-
Wakamatsu N, Hayashi M, Kawai H, et al. Mutations producing premature termination of translation and an amino acid substitution in the sterol 27-hydroxylase gene cause cerebrotendinous xanthomatosis associated with parkinsonism. J Neurol Neurosurg Psychiatry 1999;67: 195–8.
-
(1999)
J Neurol Neurosurg Psychiatry
, vol.67
, pp. 195-198
-
-
Wakamatsu, N.1
Hayashi, M.2
Kawai, H.3
-
304
-
-
0032850803
-
Mutation of the sterol 27-hydroxylase gene (Cyp27) results in truncation of m rna expressed in leucocytes in a japanese family with cerebrotendinous xanthomatosis
-
Shiga K, Fukuyama R, Kimura S, et al. Mutation of the sterol 27-hydroxylase gene (CYP27) results in truncation of m RNA expressed in leucocytes in a Japanese family with cerebrotendinous xanthomatosis. J Neurol Neurosurg Psychiatry 1999;67: 675–7.
-
(1999)
J Neurol Neurosurg Psychiatry
, vol.67
, pp. 675-677
-
-
Shiga, K.1
Fukuyama, R.2
Kimura, S.3
-
306
-
-
0014303558
-
Cerebrotendinous xanthomatosis. The storage of cholestanol within the nervous system
-
Menkes JH, Schimschock JR, Swanson PD. Cerebrotendinous xanthomatosis. The storage of cholestanol within the nervous system. Arch Neurol 1968;19: 47–53.
-
(1968)
Arch Neurol
, vol.19
, pp. 47-53
-
-
Menkes, J.H.1
Schimschock, J.R.2
Swanson, P.D.3
-
307
-
-
0015167177
-
Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism
-
Salen G. Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism. Ann Intern Med 1971;75: 843–51.
-
(1971)
Ann Intern Med
, vol.75
, pp. 843-851
-
-
Salen, G.1
-
308
-
-
0019131539
-
Occurrence of bile alcohols in the bile of a patient with cholestasis
-
Kibe A, Nakai S, Kuramoto T, Hoshita T. Occurrence of bile alcohols in the bile of a patient with cholestasis. J Lipid Res 1980;21: 594–9.
-
(1980)
J Lipid Res
, vol.21
, pp. 594-599
-
-
Kibe, A.1
Nakai, S.2
Kuramoto, T.3
Hoshita, T.4
-
309
-
-
0023625930
-
Urinary bile alcohols in liver dysfunction
-
Hiraoka T, Kihira K, Kohda T, et al. Urinary bile alcohols in liver dysfunction. Clin Chim Acta 1987;169: 127–32.
-
(1987)
Clin Chim Acta
, vol.169
, pp. 127-132
-
-
Hiraoka, T.1
Kihira, K.2
Kohda, T.3
-
310
-
-
0024827039
-
Increased urinary excretion of bile alcohol glucuronides in patients with primary biliary cirrhosis
-
Weydert-Huijghebaert S, Karlaganis G, Renner EL, Preisig R. Increased urinary excretion of bile alcohol glucuronides in patients with primary biliary cirrhosis. J Lipid Res 1989;30: 1673–9.
-
(1989)
J Lipid Res
, vol.30
, pp. 1673-1679
-
-
Weydert-Huijghebaert, S.1
Karlaganis, G.2
Renner, E.L.3
Preisig, R.4
-
311
-
-
0021914929
-
A novel pathway for biosynthesis of cholestanol with 7a-hydroxylated c27-steroids as intermediates, and its importance for the accumulation of cholestanol in cerebrotendinous xanthomatosis
-
Skrede S, Bjorkhem I, Buchmann MS, et al. A novel pathway for biosynthesis of cholestanol with 7a-hydroxylated C27-steroids as intermediates, and its importance for the accumulation of cholestanol in cerebrotendinous xanthomatosis. J Clin Invest 1985;75: 448–55.
-
(1985)
J Clin Invest
, vol.75
, pp. 448-455
-
-
Skrede, S.1
Bjorkhem, I.2
Buchmann, M.S.3
-
312
-
-
0023838684
-
Hepatic 7a- dehydroxylation of bile acid intermediates, and its significance for the pathogenesis of cerebrotendinous xanthomatosis
-
Skrede S, Buchmann MS, Bjorkhem I. Hepatic 7a- dehydroxylation of bile acid intermediates, and its significance for the pathogenesis of cerebrotendinous xanthomatosis. J Lipid Res 1988;29: 157–64.
-
(1988)
J Lipid Res
, vol.29
, pp. 157-164
-
-
Skrede, S.1
Buchmann, M.S.2
Bjorkhem, I.3
-
313
-
-
0021336684
-
Capillary gas chromatographic determination of cholestanol/cholesterol ratio in biological fluids. Its potential usefulness for the followup of some liver diseases and its lack of specificity in diagnosing ctx (cerebrotendinous xanthomatosis)
-
Koopman BJ, van der Molen JC, Wolthers BG, et al. Capillary gas chromatographic determination of cholestanol/cholesterol ratio in biological fluids. Its potential usefulness for the followup of some liver diseases and its lack of specificity in diagnosing CTX (cerebrotendinous xanthomatosis). Clin Chim Acta 1984;137: 305–15.
-
(1984)
Clin Chim Acta
, vol.137
, pp. 305-315
-
-
Koopman, B.J.1
Van Der Molen, J.C.2
Wolthers, B.G.3
-
314
-
-
0021707122
-
Long-term treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid
-
Berginer VM, Salen G, Shefer S. Long-term treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid. N Engl J Med 1984;311: 1649–52.
-
(1984)
N Engl J Med
, vol.311
, pp. 1649-1652
-
-
Berginer, V.M.1
Salen, G.2
Shefer, S.3
-
316
-
-
0022373413
-
Treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid
-
Pedley TA, Emerson RG, Warner CL, et al. Treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid. Ann Neurol 1985;18: 517–18.
-
(1985)
Ann Neurol
, vol.18
, pp. 517-518
-
-
Pedley, T.A.1
Emerson, R.G.2
Warner, C.L.3
-
318
-
-
0025782611
-
Cerebrotendinous xanthomatosis: Treatments with simvastatin, lovastatin, and chenodeoxycholic acid in 3 siblings [see comments]
-
Peynet J, Laurent A, De Liege P, et al. Cerebrotendinous xanthomatosis: treatments with simvastatin, lovastatin, and chenodeoxycholic acid in 3 siblings [see comments]. Neurology 1991;41: 434–6.
-
(1991)
Neurology
, vol.41
, pp. 434-436
-
-
Peynet, J.1
Laurent, A.2
De Liege, P.3
-
319
-
-
0020957614
-
Cerebrotendinous xanthomatosis: Biochemical response to inhibition of cholesterol synthesis
-
Lewis B, Mitchell WD, Marenah CB, et al. Cerebrotendinous xanthomatosis: biochemical response to inhibition of cholesterol synthesis. Br Med J (Clin Res Ed) 1983;287: 21–2.
-
(1983)
Br Med J (Clin Res Ed)
, vol.287
, pp. 21-22
-
-
Lewis, B.1
Mitchell, W.D.2
Marenah, C.B.3
-
320
-
-
0025895604
-
Combined treatment with chenodeoxycholic acid and pravastatin improves plasma cholestanol levels associated with marked regression of tendon xanthomas in cerebrotendinous xanthomatosis
-
Nakamura T, Matsuzawa Y, Takemura K, et al. Combined treatment with chenodeoxycholic acid and pravastatin improves plasma cholestanol levels associated with marked regression of tendon xanthomas in cerebrotendinous xanthomatosis. Metabolism 1991;40: 741–6.
-
(1991)
Metabolism
, vol.40
, pp. 741-746
-
-
Nakamura, T.1
Matsuzawa, Y.2
Takemura, K.3
-
321
-
-
0037219301
-
Liver disease caused by failure to racemize trihydroxycholestanoic acid: Gene mutation and effect of bile acid therapy
-
Setchell KDR, Heubi JE, Bove KE, et al. Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy. Gastroenterology 2003;124: 217–32.
-
(2003)
Gastroenterology
, vol.124
, pp. 217-232
-
-
Setchell, K.1
Heubi, J.E.2
Bove, K.E.3
-
322
-
-
0033973970
-
Mutations in the gene encoding peroxisomal 2-methyl-acyl-co a racemase cause adult-onset sensory motor neuropathy
-
Ferdinandusse S, Denis S, Clayton PT, et al. Mutations in the gene encoding peroxisomal 2-methyl-acyl-Co A racemase cause adult-onset sensory motor neuropathy. Nat Genet 2000;24: 188–191.
-
(2000)
Nat Genet
, vol.24
, pp. 188-191
-
-
Ferdinandusse, S.1
Denis, S.2
Clayton, P.T.3
-
323
-
-
0028176487
-
Purification and properties of an alpha-methylacyl-co a racemase from rat liver
-
Schmitz W, Fingerhut R, Conzelmann E. Purification and properties of an alpha-methylacyl-Co A racemase from rat liver. Eur J Biochem 1994;222: 313–23.
-
(1994)
Eur J Biochem
, vol.222
, pp. 313-323
-
-
Schmitz, W.1
Fingerhut, R.2
Conzelmann, E.3
-
324
-
-
0028982895
-
Purification and characterizationof an alpha-methylacyl-co aracemase from human liver
-
Schmitz W, Albers C, Fingerhut R, Conzelmann E. Purification and characterizationof an alpha-methylacyl-Co Aracemase from human liver. Eur J Biochem 1995;231: 815–22.
-
(1995)
Eur J Biochem
, vol.231
, pp. 815-822
-
-
Schmitz, W.1
Albers, C.2
Fingerhut, R.3
Conzelmann, E.4
-
325
-
-
0036566440
-
The role of alphamethylacyl- co a racemase in bile acid synthesis
-
Cuebas DA, Phillips C, Schmitz W, et al. The role of alphamethylacyl- Co A racemase in bile acid synthesis. Biochem J 2002;363: 801–7.
-
(2002)
Biochem J
, vol.363
, pp. 801-807
-
-
Cuebas, D.A.1
Phillips, C.2
Schmitz, W.3
-
326
-
-
0034864582
-
Fibroblast studies documenting a case of peroxisomal 2-methylacyl-co a racemase deficiency; possible link between racemase deficiency and malabsorption and vitamin k deficiency
-
Van Veldhoven PP, Meyhi E, Squires J, et al. Fibroblast studies documenting a case of peroxisomal 2-methylacyl-Co A racemase deficiency; possible link between racemase deficiency and malabsorption and vitamin K deficiency. Eur J Clin Invest 2001;31: 714–22.
-
(2001)
Eur J Clin Invest
, vol.31
, pp. 714-722
-
-
Van Veldhoven, P.P.1
Meyhi, E.2
Squires, J.3
-
327
-
-
2442529920
-
A mouse model for alpha-methylacyl-co a racemase deficiency: Adjustment of bile acid synthesis and intolerance to dietary methyl-branched lipids
-
Savolainen K, Kotti TJ, Schmitz W, et al. A mouse model for alpha-methylacyl-Co A racemase deficiency: adjustment of bile acid synthesis and intolerance to dietary methyl-branched lipids. Hum Mol Genet 2004;13: 955–65.
-
(2004)
Hum Mol Genet
, vol.13
, pp. 955-965
-
-
Savolainen, K.1
Kotti, T.J.2
Schmitz, W.3
-
328
-
-
0000228425
-
Disorders of peroxisome biogenesis
-
Scriver CR, Beaudet AL, Sly WS, Valle D, eds, New York: Mc Graw-Hill
-
Lazarow PB, Moser HW. Disorders of peroxisome biogenesis. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The metabolic basis of inherited disease. New York: Mc Graw-Hill, 1989: 1479–509.
-
(1989)
The Metabolic Basis of Inherited Disease
, pp. 1479-1509
-
-
Lazarow, P.B.1
Moser, H.W.2
-
329
-
-
0021047742
-
Review: The cerebrohepatorenal syndrome of zellweger, morphologic and metabolic aspects
-
Kelley RI. Review: the cerebrohepatorenal syndrome of Zellweger, morphologic and metabolic aspects. Am J Med Genet 1983;16: 503–17.
-
(1983)
Am J Med Genet
, vol.16
, pp. 503-517
-
-
Kelley, R.I.1
-
330
-
-
0023832444
-
Clinical biochemistry of peroxisomal disorders
-
Kaiser E, Kramar R. Clinical biochemistry of peroxisomal disorders. Clin Chim Acta 1988;173: 57–80.
-
(1988)
Clin Chim Acta
, vol.173
, pp. 57-80
-
-
Kaiser, E.1
Kramar, R.2
-
333
-
-
49249087095
-
Peroxisomal diseases
-
Suchy FJ, Sokol RJ, Balistreri WF, eds., 3rd ed. Philadelphia: Lippincott Williams &Wilkins
-
Watkins PA, Schwarz KB. Peroxisomal diseases. In: Suchy FJ, Sokol RJ, Balistreri WF, eds. Liver disease in children. 3rd ed. Philadelphia: Lippincott Williams &Wilkins, 2007: 840–57.
-
(2007)
Liver Disease in Children
, pp. 840-857
-
-
Watkins, P.A.1
Schwarz, K.B.2
-
334
-
-
0022917472
-
Rapid diagnosis of zellweger syndrome and infantile refsum’s disease by fast atom bombardment–mass spectrometry of urine bile salts
-
Lawson AM, Madigan MJ, Shortland D, Clayton PT. Rapid diagnosis of Zellweger syndrome and infantile Refsum’s disease by fast atom bombardment–mass spectrometry of urine bile salts. Clin Chim Acta 1986;161: 221–31.
-
(1986)
Clin Chim Acta
, vol.161
, pp. 221-231
-
-
Lawson, A.M.1
Madigan, M.J.2
Shortland, D.3
Clayton, P.T.4
-
335
-
-
0025800057
-
Bile acids in amniotic fluid: Promising metabolites for the prenatal diagnosis of peroxisomal disorders
-
Stellaard F, Kleijer WJ, Wanders RJ, et al. Bile acids in amniotic fluid: promising metabolites for the prenatal diagnosis of peroxisomal disorders. J Inherit Metab Dis 1991;14: 353–6.
-
(1991)
J Inherit Metab Dis
, vol.14
, pp. 353-356
-
-
Stellaard, F.1
Kleijer, W.J.2
Wanders, R.J.3
-
336
-
-
0018762194
-
Metabolism of 5ß- cholestane-3a, 7a, 12a, 26-tetrol and 5ß-cholestane-3a, 7a, 12a, 25-tetrol into cholic acid in normal human subjects
-
Hanson RF, Staples AB, Williams GC. Metabolism of 5ß- cholestane-3a, 7a, 12a, 26-tetrol and 5ß-cholestane-3a, 7a, 12a, 25-tetrol into cholic acid in normal human subjects. J Lipid Res 1979;20: 489–93.
-
(1979)
J Lipid Res
, vol.20
, pp. 489-493
-
-
Hanson, R.F.1
Staples, A.B.2
Williams, G.C.3
-
337
-
-
0018651545
-
C27 bile acids in infants with coprostanic acidemia and occurrence of a 3a, 7a, 12a-trihydroxy-5ß-c29 dicarboxylic bile acid as a major component in their serum
-
Parmentier GG, Janssen GA, Eggermont EA, Eyssen HJ. C27 bile acids in infants with coprostanic acidemia and occurrence of a 3a, 7a, 12a-trihydroxy-5ß-C29 dicarboxylic bile acid as a major component in their serum. Eur J Biochem 1979;102: 173–83.
-
(1979)
Eur J Biochem
, vol.102
, pp. 173-183
-
-
Parmentier, G.G.1
Janssen, G.A.2
Eggermont, E.A.3
Eyssen, H.J.4
-
338
-
-
0021806511
-
Bile acid abnormalities and the diagnosis of cerebro-hepato-renal syndrome (Zellweger syndrome)
-
Eyssen H, Eggermont E, van Eldere J, et al. Bile acid abnormalities and the diagnosis of cerebro-hepato-renal syndrome (Zellweger syndrome). Acta Paediatr Scand 1985;74: 539–44.
-
(1985)
Acta Paediatr Scand
, vol.74
, pp. 539-544
-
-
Eyssen, H.1
Eggermont, E.2
Van Eldere, J.3
-
339
-
-
0021906934
-
Identification of 3a, 7a, 12a-trihydroxy- 5ß-cholestan-26-oic acid, an intermediate in cholic acid synthesis, in the plasma of patients with infantile refsum’s disease
-
Poulos A, Whiting MJ. Identification of 3a, 7a, 12a-trihydroxy- 5ß-cholestan-26-oic acid, an intermediate in cholic acid synthesis, in the plasma of patients with infantile Refsum’s disease. J Inherit Metab Dis 1985;8: 13–17.
-
(1985)
J Inherit Metab Dis
, vol.8
, pp. 13-17
-
-
Poulos, A.1
Whiting, M.J.2
-
340
-
-
0025359856
-
Bile acid profiles in peroxisomal 3-oxoacyl-coenzyme a thiolase deficiency
-
Clayton PT, Patel E, Lawson AM, et al. Bile acid profiles in peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency. J Clin Invest 1990;85: 1267–73.
-
(1990)
J Clin Invest
, vol.85
, pp. 1267-1273
-
-
Clayton, P.T.1
Patel, E.2
Lawson, A.M.3
-
341
-
-
0025875876
-
Inborn errors of bile acid metabolism
-
Clayton PT. Inborn errors of bile acid metabolism. J Inherit Metab Dis 1991;14: 478–96.
-
(1991)
J Inherit Metab Dis
, vol.14
, pp. 478-496
-
-
Clayton, P.T.1
-
342
-
-
0022551325
-
Lignoceroyl-co ash ligase: Enzyme defect in fatty acid beta-oxidation system in x-linked childhood adrenoleukodystrophy
-
Hashmi M, Stanley W, Singh I. Lignoceroyl-Co ASH ligase: enzyme defect in fatty acid beta-oxidation system in X-linked childhood adrenoleukodystrophy. FEBS Lett 1986;196: 247–50.
-
(1986)
FEBS Lett
, vol.196
, pp. 247-250
-
-
Hashmi, M.1
Stanley, W.2
Singh, I.3
-
343
-
-
0001112876
-
Peroxisomal lignoceroyl- co a ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy
-
Lazo O, Contreras M, Hashmi M, et al. Peroxisomal lignoceroyl- Co A ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy. Proc Natl Acad Sci U S A 1988;85: 7647–51.
-
(1988)
Proc Natl Acad Sci U S A
, vol.85
, pp. 7647-7651
-
-
Lazo, O.1
Contreras, M.2
Hashmi, M.3
-
344
-
-
0023886918
-
Direct demonstration that the deficient oxidation of very long chain fatty acids in x-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids
-
Wanders RJ, van Roermund CW, van Wijland MJ, et al. Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids. Biochem Biophys Res Commun 1988;153: 618–24.
-
(1988)
Biochem Biophys Res Commun
, vol.153
, pp. 618-624
-
-
Wanders, R.J.1
Van Roermund, C.W.2
Van Wijland, M.J.3
-
345
-
-
0023878166
-
Anew peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-co a oxidase (Pseudo-neonatal adrenoleukodystrophy)
-
Poll-The BT, Roels F, Ogier H, et al. Anew peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-Co A oxidase (pseudo-neonatal adrenoleukodystrophy). Am J Hum Genet 1988;42: 422–34.
-
(1988)
Am J Hum Genet
, vol.42
, pp. 422-434
-
-
Poll-The, B.T.1
Roels, F.2
Ogier, H.3
-
346
-
-
0021917830
-
Defectiveperoxisomal cleavage of the c27-steroid side chain in the cerebro-hepatorenal syndrome of zellweger
-
Kase BF, Bjorkhem I, Haga P, Pedersen JI. Defectiveperoxisomal cleavage of the C27-steroid side chain in the cerebro-hepatorenal syndrome of Zellweger. J Clin Invest 1985;75: 427–35.
-
(1985)
J Clin Invest
, vol.75
, pp. 427-435
-
-
Kase, B.F.1
Bjorkhem, I.2
Haga, P.3
Pedersen, J.I.4
-
347
-
-
0022373298
-
In vivo and vitro studies on formation of bile acids in patients with zellweger syndrome. Evidence that peroxisomes are of importance in the normal biosynthesis of both cholic and chenodeoxycholic acid
-
Kase BF, Pedersen JI, Strandvik B, Bjorkhem I. In vivo and vitro studies on formation of bile acids in patients with Zellweger syndrome. Evidence that peroxisomes are of importance in the normal biosynthesis of both cholic and chenodeoxycholic acid. J Clin Invest 1985;76: 2393–402.
-
(1985)
J Clin Invest
, vol.76
, pp. 2393-2402
-
-
Kase, B.F.1
Pedersen, J.I.2
Strandvik, B.3
Bjorkhem, I.4
-
348
-
-
0023092778
-
Plasma bile acids in patients with peroxisomal dysfunction syndromes: Analysis by capillary gas chromatography-mass spectrometry
-
Clayton PT, Lake BD, Hall NA, et al. Plasma bile acids in patients with peroxisomal dysfunction syndromes: analysis by capillary gas chromatography-mass spectrometry. Eur J Pediatr 1987;146: 166–73.
-
(1987)
Eur J Pediatr
, vol.146
, pp. 166-173
-
-
Clayton, P.T.1
Lake, B.D.2
Hall, N.A.3
-
349
-
-
0026668028
-
Substrate specificities of rat liver peroxisomal acyl-co a oxidases: Palmitoyl-co a oxidase (inducible acyl-co a oxidase), pristanoyl-co a oxidase (non-inducible acyl-co a oxidase), and trihydroxycoprostanoyl-co a oxidase
-
Van Veldhoven PP, Vanhove G, Assselberghs S, et al. Substrate specificities of rat liver peroxisomal acyl-Co A oxidases: palmitoyl-Co A oxidase (inducible acyl-Co A oxidase), pristanoyl-Co A oxidase (non-inducible acyl-Co A oxidase), and trihydroxycoprostanoyl-Co A oxidase. J Biol Chem 1992;267: 20065–74.
-
(1992)
J Biol Chem
, vol.267
, pp. 20065-20074
-
-
Van Veldhoven, P.P.1
Vanhove, G.2
Assselberghs, S.3
-
350
-
-
0030462605
-
Molecular characterization of the human peroxisomal branched-chain acyl-co a oxidase: C dnacloning, chromosomal assignment, tissue distribution, and evidence for the absence of the protein in zellweger syndrome
-
Baumgart E, Vanhooren JC, Fransen M, et al. Molecular characterization of the human peroxisomal branched-chain acyl-Co A oxidase: c DNAcloning, chromosomal assignment, tissue distribution, and evidence for the absence of the protein in Zellweger syndrome. Proc Natl Acad Sci U S A 1996;93: 13748–53.
-
(1996)
Proc Natl Acad Sci U S A
, vol.93
, pp. 13748-13753
-
-
Baumgart, E.1
Vanhooren, J.C.2
Fransen, M.3
-
351
-
-
0025169032
-
Anew peroxisomal disorder: Di- and trihydroxycholestanaemia due to a presumed trihydroxycholestanoyl-co a oxidase deficiency
-
Christensen E, Van Eldere J, Brandt NJ, et al. Anew peroxisomal disorder: di- and trihydroxycholestanaemia due to a presumed trihydroxycholestanoyl-Co A oxidase deficiency. J Inherit Metab Dis 1990;13: 363–6.
-
(1990)
J Inherit Metab Dis
, vol.13
, pp. 363-366
-
-
Christensen, E.1
Van Eldere, J.2
Brandt, N.J.3
-
354
-
-
0029808744
-
Ataxia associated with increased plasma concentrations of pristanic acid, phytanic acid and c27 bile acids but normal fibroblast branched-chain fatty acid oxidation
-
Clayton PT, Johnson AW, Mills KA, et al. Ataxia associated with increased plasma concentrations of pristanic acid, phytanic acid and C27 bile acids but normal fibroblast branched-chain fatty acid oxidation. J Inher Metab Dis 1996;19: 761–8.
-
(1996)
J Inher Metab Dis
, vol.19
, pp. 761-768
-
-
Clayton, P.T.1
Johnson, A.W.2
Mills, K.A.3
-
355
-
-
2842597516
-
Disorders of peroxisomal metabolism
-
Walker WA, Durie PR, Hamilton JR, eds, Toronto/Philadelphia: BC Decker
-
Kelley RI. Disorders of peroxisomal metabolism. In: Walker WA, Durie PR, Hamilton JR, eds. Pediatric gastrointestinal disease, pathophysiology, diagnosis, management. Toronto/Philadelphia: BC Decker, 1991: 1032–54.
-
(1991)
Pediatric Gastrointestinal Disease, Pathophysiology, Diagnosis, Management
, pp. 1032-1054
-
-
Kelley, R.I.1
-
356
-
-
0342528190
-
Afatty acyl-co aoxidizing systemin rat liver peroxisomes; enhancement by clofibrate, a hypolipidemic drug
-
Lazarow PB, De Duve C. Afatty acyl-Co Aoxidizing systemin rat liver peroxisomes; enhancement by clofibrate, a hypolipidemic drug. Proc Natl Acad Sci U S A 1976;73: 2043–6.
-
(1976)
Proc Natl Acad Sci U S A
, vol.73
, pp. 2043-2046
-
-
Lazarow, P.B.1
De Duve, C.2
-
357
-
-
0021961904
-
Unsuccessful attempts to induce peroxisomes in two cases of zellweger disease by treatmentwith clofibrate
-
Bjorkhem I, Blomstrand S, Glaumann H, Strandvik B. Unsuccessful attempts to induce peroxisomes in two cases of Zellweger disease by treatmentwith clofibrate. Pediatr Res 1985;19: 590–3.
-
(1985)
Pediatr Res
, vol.19
, pp. 590-593
-
-
Bjorkhem, I.1
Blomstrand, S.2
Glaumann, H.3
Strandvik, B.4
-
358
-
-
0019168913
-
Liver in the cerebro-hepato-renal syndrome: Defective bile acid synthesis and abnormal mitochondria
-
Mathis RK, Watkins JB, Szczepanik-Van Leeuwen P, Lott IT. Liver in the cerebro-hepato-renal syndrome: defective bile acid synthesis and abnormal mitochondria. Gastroenterology 1980;79: 1311–17.
-
(1980)
Gastroenterology
, vol.79
, pp. 1311-1317
-
-
Mathis, R.K.1
Watkins, J.B.2
Szczepanik-Van Leeuwen, P.3
Lott, I.T.4
-
359
-
-
0022913264
-
Identification of unconjugated bile acids in human bile
-
Matoba N, Une M, Hoshita T. Identification of unconjugated bile acids in human bile. J Lipid Res 1986;27: 1154–62.
-
(1986)
J Lipid Res
, vol.27
, pp. 1154-1162
-
-
Matoba, N.1
Une, M.2
Hoshita, T.3
-
360
-
-
0026352217
-
Changes in bile acid composition in patients with primary biliary cirrhosis induced by ursodeoxycholic acid administration
-
Crosignani A, Podda M, Battezzati PM, et al. Changes in bile acid composition in patients with primary biliary cirrhosis induced by ursodeoxycholic acid administration. Hepatology 1991;14: 1000–7.
-
(1991)
Hepatology
, vol.14
, pp. 1000-1007
-
-
Crosignani, A.1
Podda, M.2
Battezzati, P.M.3
-
361
-
-
0037994064
-
Complex inheritance of familial hypercholanemia with associated mutations in tjp2 and baat
-
Carlton VEH, Harris BZ, Puffenberger EG, et al. Complex inheritance of familial hypercholanemia with associated mutations in TJP2 and BAAT. Nature Genetics 2003;34: 91–6.
-
(2003)
Nature Genetics
, vol.34
, pp. 91-96
-
-
Carlton, V.1
Harris, B.Z.2
Puffenberger, E.G.3
-
362
-
-
0025315898
-
Bile acid metabolism in early life: Studies of amniotic fluid
-
Nakagawa M, Setchell KDR. Bile acid metabolism in early life: studies of amniotic fluid. J Lipid Res 1990;31: 1089–98.
-
(1990)
J Lipid Res
, vol.31
, pp. 1089-1098
-
-
Nakagawa, M.1
Setchell, K.2
-
363
-
-
0023801559
-
Defective bile acid amidation: Predicted features of a new inborn error of metabolism
-
Hofmann AF, Strandvik B. Defective bile acid amidation: predicted features of a new inborn error of metabolism. Lancet 1988;2: 311–13.
-
(1988)
Lancet
, vol.2
, pp. 311-313
-
-
Hofmann, A.F.1
Strandvik, B.2
-
364
-
-
0014491370
-
Effects of sulfation of taurolithocholic and glycolithocholic acids on their intestinal transport
-
Low-Beer TS, Tyor MP, Lack L. Effects of sulfation of taurolithocholic and glycolithocholic acids on their intestinal transport. Gastroenterology 1969;56: 721–6.
-
(1969)
Gastroenterology
, vol.56
, pp. 721-726
-
-
Low-Beer, T.S.1
Tyor, M.P.2
Lack, L.3
-
365
-
-
0018618693
-
Properties and biological significance of the ileal bile salt transport system
-
Lack L. Properties and biological significance of the ileal bile salt transport system. Environ Health Perspect 1979;33: 79–90.
-
(1979)
Environ Health Perspect
, vol.33
, pp. 79-90
-
-
Lack, L.1
-
366
-
-
0018850281
-
Effects of sulfation patterns on intestinal transport of bile salt sulfate esters
-
De Witt EH, Lack L. Effects of sulfation patterns on intestinal transport of bile salt sulfate esters. Am J Physiol 1980;238: G34–9.
-
(1980)
Am J Physiol
, vol.238
, pp. G34-G39
-
-
De Witt, E.H.1
Lack, L.2
-
367
-
-
0018350237
-
Refractory infantile diarrhea due to primary bile acid malabsorption
-
Heubi JE, Balistreri WF, Partin JC, et al. Refractory infantile diarrhea due to primary bile acid malabsorption. J Pediatr 1979;94: 546–51.
-
(1979)
J Pediatr
, vol.94
, pp. 546-551
-
-
Heubi, J.E.1
Balistreri, W.F.2
Partin, J.C.3
-
368
-
-
0019962909
-
Primary bile acid malabsorption: Defective in vitro ileal active bile acid transport
-
Heubi JE, Balistreri WF, Fondacaro JD, et al. Primary bile acid malabsorption: defective in vitro ileal active bile acid transport. Gastroenterology 1982;83: 804–11.
-
(1982)
Gastroenterology
, vol.83
, pp. 804-811
-
-
Heubi, J.E.1
Balistreri, W.F.2
Fondacaro, J.D.3
-
369
-
-
0000139419
-
A newly recognized syndrome of multiple congenital anomalies
-
Smith DW, Lemli L, Opitz JM. A newly recognized syndrome of multiple congenital anomalies. J Pediatr 1964;64: 210–17.
-
(1964)
J Pediatr
, vol.64
, pp. 210-217
-
-
Smith, D.W.1
Lemli, L.2
Opitz, J.M.3
-
371
-
-
0141891445
-
Genetic cholestasis, causes and consequences for hepatobiliary transport
-
Jansen PL, Sturm E. Genetic cholestasis, causes and consequences for hepatobiliary transport. Liver Int 2003;23: 315–22.
-
(2003)
Liver Int
, vol.23
, pp. 315-322
-
-
Jansen, P.L.1
Sturm, E.2
-
372
-
-
11144245023
-
Molecular basis of intrahepatic cholestasis
-
Carlton VE, Pawlikowska L, Bull LN. Molecular basis of intrahepatic cholestasis. Ann Med 2004;36: 606–17.
-
(2004)
Ann Med
, vol.36
, pp. 606-617
-
-
Carlton, V.E.1
Pawlikowska, L.2
Bull, L.N.3
-
373
-
-
30144432405
-
Inborn errors of biliary canalicular transport systems
-
Kubitz R, Keitel V, Haussinger D. Inborn errors of biliary canalicular transport systems. Methods Enzymol 2005;400: 558–69.
-
(2005)
Methods Enzymol
, vol.400
, pp. 558-569
-
-
Kubitz, R.1
Keitel, V.2
Haussinger, D.3
-
374
-
-
17844403220
-
Expression and localization of hepatobiliary transport proteins in progressive familial intrahepatic cholestasis
-
Keitel V, Burdelski M, Warskulat U, et al. Expression and localization of hepatobiliary transport proteins in progressive familial intrahepatic cholestasis. Hepatology 2005;41: 1160–72.
-
(2005)
Hepatology
, vol.41
, pp. 1160-1172
-
-
Keitel, V.1
Burdelski, M.2
Warskulat, U.3
-
375
-
-
0014442684
-
Byler disease. Fatal familial intrahepatic cholestasis in an amish kindred
-
Clayton RJ, Iber FL, Ruebner BH, Mc Kusick VA. Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969;117: 112–24.
-
(1969)
Am J Dis Child
, vol.117
, pp. 112-124
-
-
Clayton, R.J.1
Iber, F.L.2
Ruebner, B.H.3
Mc Kusick, V.A.4
-
376
-
-
12644268207
-
Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [pfic-1] and byler syndrome): Evidence for heterogeneity
-
Bull LN, Carlton VE, Stricker NL, et al. Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): evidence for heterogeneity. Hepatology 1997;26: 155–64.
-
(1997)
Hepatology
, vol.26
, pp. 155-164
-
-
Bull, L.N.1
Carlton, V.E.2
Stricker, N.L.3
-
377
-
-
0034005389
-
Progressive familial intrahepatic cholestasis: A personal perspective
-
Knisely AS. Progressive familial intrahepatic cholestasis: a personal perspective. Pediatr Dev Pathol 2000;3: 113–25.
-
(2000)
Pediatr Dev Pathol
, vol.3
, pp. 113-125
-
-
Knisely, A.S.1
-
378
-
-
17344366172
-
A gene encoding a liver-specific abc transporter ismutated in progressive familial intrahepatic cholestasis
-
Strautnieks SS, Bull LN, Knisely AS, et al. A gene encoding a liver-specific ABC transporter ismutated in progressive familial intrahepatic cholestasis. Nat Genet 1998;20: 233–8.
-
(1998)
Nat Genet
, vol.20
, pp. 233-238
-
-
Strautnieks, S.S.1
Bull, L.N.2
Knisely, A.S.3
-
379
-
-
0040284751
-
Mutations in the mdr3 gene cause progressive familial intrahepatic cholestasis
-
De Vree JML, Jacquemin E, Sturm E, et al. Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis. Proc Natl Acad Sci U S A 1998;95: 282–7.
-
(1998)
Proc Natl Acad Sci U S A
, vol.95
, pp. 282-287
-
-
De Vree, J.1
Jacquemin, E.2
Sturm, E.3
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