-
1
-
-
0037790917
-
The enzymes, regulation, and genetics of bile acid synthesis
-
Russell, D. W. 2003. The enzymes, regulation, and genetics of bile acid synthesis. Annu. Rev. Biochem. 72: 137-174.
-
(2003)
Annu. Rev. Biochem
, vol.72
, pp. 137-174
-
-
Russell, D.W.1
-
2
-
-
0037025401
-
Participation of two members of the very long-chain acyl-CoA synthetase family in bile acid synthesis and recycling
-
Mihalik, S. J., S. J. Steinberg, Z. Pei, J. Park, D. G. Kim, A. K. Heinzer, G. Dacremont, R. J. Wanders, D. A. Cuebas, K. D. Smith, and P. A. Watkins. 2002. Participation of two members of the very long-chain acyl-CoA synthetase family in bile acid synthesis and recycling. J. Biol. Chem. 277: 24771-24779.
-
(2002)
J. Biol. Chem
, vol.277
, pp. 24771-24779
-
-
Mihalik, S.J.1
Steinberg, S.J.2
Pei, Z.3
Park, J.4
Kim, D.G.5
Heinzer, A.K.6
Dacremont, G.7
Wanders, R.J.8
Cuebas, D.A.9
Smith, K.D.10
Watkins, P.A.11
-
3
-
-
0034717047
-
The human liver-specifi c homolog of very long-chain acyl-CoA synthetase is cholate:CoA ligase
-
Steinberg, S. J., S. J. Mihalik, D. G. Kim, D. A. Cuebas, and P. A. Watkins. 2000. The human liver-specifi c homolog of very long-chain acyl-CoA synthetase is cholate:CoA ligase. J. Biol. Chem. 275: 15605- 15608.
-
(2000)
J. Biol. Chem
, vol.275
, pp. 15605-15608
-
-
Steinberg, S.J.1
Mihalik, S.J.2
Kim, D.G.3
Cuebas, D.A.4
Watkins, P.A.5
-
4
-
-
0033551122
-
Human very-long-chain acyl-CoA synthetase: Cloning, topography, and relevance to branched-chain fatty acid metabolism
-
Steinberg, S. J., S. J. Wang, D. G. Kim, S. J. Mihalik, and P. A. Watkins. 1999. Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism. Biochem. Biophys. Res. Commun. 257: 615-621.
-
(1999)
Biochem. Biophys. Res. Commun
, vol.257
, pp. 615-621
-
-
Steinberg, S.J.1
Wang, S.J.2
Kim, D.G.3
Mihalik, S.J.4
Watkins, P.A.5
-
5
-
-
0023740620
-
Subcellular localization of 3 alpha, 7 alpha-dihydroxy- and 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoyl-coenzyme A ligase(s) in rat liver
-
Prydz, K., B. F. Kase, I. Bjorkhem, and J. I. Pedersen. 1988. Subcellular localization of 3 alpha, 7 alpha-dihydroxy- and 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoyl-coenzyme A ligase(s) in rat liver. J. Lipid Res. 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
-
6
-
-
0024491504
-
Subcellular distribution and characteristics of trihydroxycoprostanoyl-CoA synthetase in rat liver
-
Schepers, L., M. Casteels, K. Verheyden, G. Parmentier, S. Asselberghs, H. J. Eyssen, and G. P. Mannaerts. 1989. Subcellular distribution and characteristics of trihydroxycoprostanoyl-CoA synthetase in rat liver. Biochem. J. 257: 221-229.
-
(1989)
Biochem. J
, vol.257
, pp. 221-229
-
-
Schepers, L.1
Casteels, M.2
Verheyden, K.3
Parmentier, G.4
Asselberghs, S.5
Eyssen, H.J.6
Mannaerts, G.P.7
-
7
-
-
0141703305
-
ATP-dependent transport of bile acid intermediates across rat liver peroxisomal membranes
-
Une, M., Y. Iguchi, T. Sakamoto, T. Tomita, Y. Suzuki, M. Morita, and T. Imanaka. 2003. ATP-dependent transport of bile acid intermediates across rat liver peroxisomal membranes. J. Biochem. 134: 225-230.
-
(2003)
J. Biochem
, vol.134
, pp. 225-230
-
-
Une, M.1
Iguchi, Y.2
Sakamoto, T.3
Tomita, T.4
Suzuki, Y.5
Morita, M.6
Imanaka, T.7
-
8
-
-
0028982895
-
Purification and characterization of an alpha-methylacyl-CoA racemase from human liver
-
Schmitz, W., C. Albers, R. Fingerhut, and E. Conzelmann. 1995. Purification and characterization of an alpha-methylacyl-CoA racemase from human liver. Eur. J. Biochem. 231: 815-822.
-
(1995)
Eur. J. Biochem
, vol.231
, pp. 815-822
-
-
Schmitz, W.1
Albers, C.2
Fingerhut, R.3
Conzelmann, E.4
-
9
-
-
0028176487
-
Purification and properties of an alpha-methylacyl-CoA racemase from rat liver
-
Schmitz, W., R. Fingerhut, and E. Conzelmann. 1994. Purification and properties of an alpha-methylacyl-CoA racemase from rat liver. Eur. J. Biochem. 222: 313-323.
-
(1994)
Eur. J. Biochem
, vol.222
, pp. 313-323
-
-
Schmitz, W.1
Fingerhut, R.2
Conzelmann, E.3
-
10
-
-
0030570428
-
Substrate stereospecificity in oxidation of (25S)-3 alpha, 7 alpha, 12 alphatrihydroxy-5 beta-cholestanoyl-CoA by peroxisomal trihydroxy-5 beta-cholestanoyl-CoA oxidase
-
Pedersen, J. I., T. Veggan, and I. Bjorkhem. 1996. Substrate stereospecificity in oxidation of (25S)-3 alpha, 7 alpha, 12 alphatrihydroxy-5 beta-cholestanoyl-CoA by peroxisomal trihydroxy-5 beta-cholestanoyl-CoA oxidase. Biochem. Biophys. Res. Commun. 224: 37-42.
-
(1996)
Biochem. Biophys. Res. Commun
, vol.224
, pp. 37-42
-
-
Pedersen, J.I.1
Veggan, T.2
Bjorkhem, I.3
-
11
-
-
0029898658
-
Peroxisomal beta-oxidation of 2-methylbranched acyl-CoA esters: Stereospecific recognition of the 2Smethyl compounds by trihydroxycoprostanoyl-CoA oxidase and pristanoyl-CoA oxidase
-
van Veldhoven, P. P., K. Croes, S. Asselberghs, P. Herdewijn, and G. P. Mannaerts. 1996. Peroxisomal beta-oxidation of 2-methylbranched acyl-CoA esters: stereospecific recognition of the 2Smethyl compounds by trihydroxycoprostanoyl-CoA oxidase and pristanoyl-CoA oxidase. FEBS Lett. 388: 80-84.
-
(1996)
FEBS Lett
, vol.388
, pp. 80-84
-
-
van Veldhoven, P.P.1
Croes, K.2
Asselberghs, S.3
Herdewijn, P.4
Mannaerts, G.P.5
-
12
-
-
0020665789
-
Confi guration at C-25 in 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta- cholestan-26-oic acid isolated from human bile
-
Batta, A. K., G. Salen, S. Shefer, B. Dayal, and G. S. Tint. 1983. Confi guration at C-25 in 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta- cholestan-26-oic acid isolated from human bile. J. Lipid Res. 24: 94-96.
-
(1983)
J. Lipid Res
, vol.24
, pp. 94-96
-
-
Batta, A.K.1
Salen, G.2
Shefer, S.3
Dayal, B.4
Tint, G.S.5
-
13
-
-
0018119868
-
Stereospecific side chain hydroxylations in the biosynthesis of chenodeoxycholic acid
-
Shefer, S., F. W. Cheng, A. K. Batta, B. Dayal, G. S. Tint, G. Salen, and E. H. Mosbach. 1978. Stereospecific side chain hydroxylations in the biosynthesis of chenodeoxycholic acid. J. Biol. Chem. 253: 6386-6392.
-
(1978)
J. Biol. Chem
, vol.253
, pp. 6386-6392
-
-
Shefer, S.1
Cheng, F.W.2
Batta, A.K.3
Dayal, B.4
Tint, G.S.5
Salen, G.6
Mosbach, E.H.7
-
14
-
-
0027159784
-
The CoA 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-CoA oxidase in human liver and kidney
-
Vanhove, G. F., P. P. Van Veldhoven, M. Fransen, S. Denis, H. J. Eyssen, R. J. Wanders, and G. P. Mannaerts. 1993. The CoA 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-CoA oxidase in human liver and kidney. J. Biol. Chem. 268: 10335-10344.
-
(1993)
J. Biol. Chem
, vol.268
, pp. 10335-10344
-
-
Vanhove, G.F.1
Van Veldhoven, P.P.2
Fransen, M.3
Denis, S.4
Eyssen, H.J.5
Wanders, R.J.6
Mannaerts, G.P.7
-
15
-
-
0031003977
-
Physiological role of D-3-hydroxyacyl-CoA dehydratase/ D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein
-
Jiang, L. L., T. Kurosawa, M. Sato, Y. Suzuki, and T. Hashimoto. 1997. Physiological role of D-3-hydroxyacyl-CoA dehydratase/ D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein. J. Biochem. (Tokyo). 121: 506-513.
-
(1997)
J. Biochem. (Tokyo)
, vol.121
, pp. 506-513
-
-
Jiang, L.L.1
Kurosawa, T.2
Sato, M.3
Suzuki, Y.4
Hashimoto, T.5
-
16
-
-
0030889795
-
Stereospecifi c formation of (24R,25R)-3 alpha,7 alpha,12 alpha,24-tetrahydroxy-5 beta-cholestan-26-oic acid catalyzed with a peroxisomal bifunctional D-3-hydroxyacyl-CoA dehydratase/ D-3-hydroxyacyl-CoA dehydrogenase
-
Kurosawa, T., M. Sato, T. Yoshimura, L. L. Jiang, T. Hashimoto, and M. Tohma. 1997. Stereospecifi c formation of (24R,25R)-3 alpha,7 alpha,12 alpha,24-tetrahydroxy-5 beta-cholestan-26-oic acid catalyzed with a peroxisomal bifunctional D-3-hydroxyacyl-CoA dehydratase/ D-3-hydroxyacyl-CoA dehydrogenase. Biol. Pharm. Bull. 20: 295-297.
-
(1997)
Biol. Pharm. Bull
, vol.20
, pp. 295-297
-
-
Kurosawa, T.1
Sato, M.2
Yoshimura, T.3
Jiang, L.L.4
Hashimoto, T.5
Tohma, M.6
-
17
-
-
0030976855
-
The human peroxisomal multifunctional protein involved in bile acid synthesis: Activity measurement, deficiency in Zellweger syndrome and chromosome mapping
-
Novikov, D., M. Dieuaide-Noubhani, J. R. Vermeesch, B. Fournier, G. P. Mannaerts, and P. P. Van Veldhoven. 1997. The human peroxisomal multifunctional protein involved in bile acid synthesis: activity measurement, deficiency in Zellweger syndrome and chromosome mapping. Biochim. Biophys. Acta. 1360: 229-240.
-
(1997)
Biochim. Biophys. Acta
, vol.1360
, pp. 229-240
-
-
Novikov, D.1
Dieuaide-Noubhani, M.2
Vermeesch, J.R.3
Fournier, B.4
Mannaerts, G.P.5
Van Veldhoven, P.P.6
-
18
-
-
0030678022
-
Recombinant 2-enoyl-CoA hydratase derived from rat peroxisomal multifunctional enzyme 2: Role of the hydratase reaction in bile acid synthesis
-
Qin, Y. M., A. M. Haapalainen, D. Conry, D. A. Cuebas, J. K. Hiltunen, and D. K. Novikov. 1997. Recombinant 2-enoyl-CoA hydratase derived from rat peroxisomal multifunctional enzyme 2: role of the hydratase reaction in bile acid synthesis. Biochem. J. 328: 377-382.
-
(1997)
Biochem. J
, vol.328
, pp. 377-382
-
-
Qin, Y.M.1
Haapalainen, A.M.2
Conry, D.3
Cuebas, D.A.4
Hiltunen, J.K.5
Novikov, D.K.6
-
19
-
-
0030855998
-
Substrate specificities of 3-oxoacyl-CoA thiolase A and sterol carrier protein 2/3-oxoacyl-CoA thiolase purified from normal rat liver peroxisomes. Sterol carrier protein 2/3-oxoacyl-CoA thiolase is involved in the metabolism of 2-methylbranched fatty acids and bile acid intermediates
-
Antonenkov, V. D., P. P. Van Veldhoven, E. Waelkens, and G. P. Mannaerts. 1997. Substrate specificities of 3-oxoacyl-CoA thiolase A and sterol carrier protein 2/3-oxoacyl-CoA thiolase purified from normal rat liver peroxisomes. Sterol carrier protein 2/3-oxoacyl-CoA thiolase is involved in the metabolism of 2-methylbranched fatty acids and bile acid intermediates. J. Biol. Chem. 272: 26023-26031.
-
(1997)
J. Biol. Chem
, vol.272
, pp. 26023-26031
-
-
Antonenkov, V.D.1
Van Veldhoven, P.P.2
Waelkens, E.3
Mannaerts, G.P.4
-
20
-
-
0031935558
-
Thiolase involved in bile acid formation
-
Bun-ya, M., M. Maebuchi, T. Kamiryo, T. Kurosawa, M. Sato, M. Tohma, L. L. Jiang, and T. Hashimoto. 1998. Thiolase involved in bile acid formation. J. Biochem. (Tokyo). 123: 347-352.
-
(1998)
J. Biochem. (Tokyo)
, vol.123
, pp. 347-352
-
-
Bun-ya, M.1
Maebuchi, M.2
Kamiryo, T.3
Kurosawa, T.4
Sato, M.5
Tohma, M.6
Jiang, L.L.7
Hashimoto, T.8
-
21
-
-
0031879234
-
Identification of the newly discovered 58 kDa peroxisomal thiolase SCPx as the main thiolase involved in both pristanic acid and trihydroxycholestanoic acid oxidation: Implications for peroxisomal beta-oxidation disorders
-
Wanders, R. J., S. Denis, E. van Berkel, F. Wouters, K. W. Wirtz, and U. Seedorf. 1998. Identification of the newly discovered 58 kDa peroxisomal thiolase SCPx as the main thiolase involved in both pristanic acid and trihydroxycholestanoic acid oxidation: implications for peroxisomal beta-oxidation disorders. J. Inherit. Metab. Dis. 21: 302-305.
-
(1998)
J. Inherit. Metab. Dis
, vol.21
, pp. 302-305
-
-
Wanders, R.J.1
Denis, S.2
van Berkel, E.3
Wouters, F.4
Wirtz, K.W.5
Seedorf, U.6
-
23
-
-
0030962690
-
Cloning, expression, and chromosomal localization of mouse liver bile acid CoA:amino acid N-acyltransferase
-
Falany, C. N., H. Fortinberry, E. H. Leiter, and S. Barnes. 1997. Cloning, expression, and chromosomal localization of mouse liver bile acid CoA:amino acid N-acyltransferase. J. Lipid Res. 38: 1139-1148.
-
(1997)
J. Lipid Res
, vol.38
, pp. 1139-1148
-
-
Falany, C.N.1
Fortinberry, H.2
Leiter, E.H.3
Barnes, S.4
-
24
-
-
0028108409
-
Glycine and taurine conjugation of bile acids by a single enzyme. Molecular cloning and expression of human liver bile acid CoA:amino acid N-acyltransferase
-
Falany, C. N., M. R. Johnson, S. Barnes, and R. B. Diasio. 1994. Glycine and taurine conjugation of bile acids by a single enzyme. Molecular cloning and expression of human liver bile acid CoA:amino acid N-acyltransferase. J. Biol. Chem. 269: 19375-19379.
-
(1994)
J. Biol. Chem
, vol.269
, pp. 19375-19379
-
-
Falany, C.N.1
Johnson, M.R.2
Barnes, S.3
Diasio, R.B.4
-
25
-
-
0345861749
-
Rat liver bile acid CoA:amino acid N-acyltransferase: Expression, characterization, and peroxisomal localization
-
He, D., S. Barnes, and C. N. Falany. 2003. Rat liver bile acid CoA:amino acid N-acyltransferase: expression, characterization, and peroxisomal localization. J. Lipid Res. 44: 2242-2249.
-
(2003)
J. Lipid Res
, vol.44
, pp. 2242-2249
-
-
He, D.1
Barnes, S.2
Falany, C.N.3
-
26
-
-
34247396623
-
Demonstration of bile acid transport across the mammalian peroxisomal membrane
-
Visser, W. F., C. W. van Roermund, L. Ijlst, H. R. Waterham, and R. J. Wanders. 2007. Demonstration of bile acid transport across the mammalian peroxisomal membrane. Biochem. Biophys. Res. Commun. 357: 335-340.
-
(2007)
Biochem. Biophys. Res. Commun
, vol.357
, pp. 335-340
-
-
Visser, W.F.1
van Roermund, C.W.2
Ijlst, L.3
Waterham, H.R.4
Wanders, R.J.5
-
27
-
-
0024374648
-
Peroxisomal bile acid-CoA:amino-acid N-acyltransferase in rat liver
-
Kase, B. F., and I. Bjorkhem. 1989. Peroxisomal bile acid-CoA:amino-acid N-acyltransferase in rat liver. J. Biol. Chem. 264: 9220-9223.
-
(1989)
J. Biol. Chem
, vol.264
, pp. 9220-9223
-
-
Kase, B.F.1
Bjorkhem, I.2
-
28
-
-
33847741406
-
Human and rat bile acid-CoA:amino acid N-acyltransferase are liver-specifi c peroxisomal enzymes: Implications for intracellular bile salt transport
-
Pellicoro, A., F. A. van den Heuvel, M. Geuken, H. Moshage, P. L. Jansen, and K. N. Faber. 2007. Human and rat bile acid-CoA:amino acid N-acyltransferase are liver-specifi c peroxisomal enzymes: implications for intracellular bile salt transport. Hepatology.45: 340-348.
-
(2007)
Hepatology
, vol.45
, pp. 340-348
-
-
Pellicoro, A.1
van den Heuvel, F.A.2
Geuken, M.3
Moshage, H.4
Jansen, P.L.5
Faber, K.N.6
-
29
-
-
0141780836
-
The human bile acid-CoA:amino acid N-acyltransferase functions in the conjugation of fatty acids to glycine
-
O'Byrne, J., M. C. Hunt, D. K. Rai, M. Saeki, and S. E. Alexson. 2003. The human bile acid-CoA:amino acid N-acyltransferase functions in the conjugation of fatty acids to glycine. J. Biol. Chem. 278: 34237-34244.
-
(2003)
J. Biol. Chem
, vol.278
, pp. 34237-34244
-
-
O'Byrne, J.1
Hunt, M.C.2
Rai, D.K.3
Saeki, M.4
Alexson, S.E.5
-
30
-
-
2542429160
-
Differential regulation of cytosolic and peroxisomal bile acid amidation by PPAR alpha activation favors the formation of unconjugated bile acids
-
Solaas, K., B. F. Kase, V. Pham, K. Bamberg, M. C. Hunt, and S. E. Alexson. 2004. Differential regulation of cytosolic and peroxisomal bile acid amidation by PPAR alpha activation favors the formation of unconjugated bile acids. J. Lipid Res. 45: 1051-1060.
-
(2004)
J. Lipid Res
, vol.45
, pp. 1051-1060
-
-
Solaas, K.1
Kase, B.F.2
Pham, V.3
Bamberg, K.4
Hunt, M.C.5
Alexson, S.E.6
-
31
-
-
0033927970
-
Subcellular organization of bile acid amidation in human liver: A key issue in regulating the biosynthesis of bile salts
-
Solaas, K., A. Ulvestad, O. Soreide, and B. F. Kase. 2000. Subcellular organization of bile acid amidation in human liver: a key issue in regulating the biosynthesis of bile salts. J. Lipid Res. 41: 1154-1162.
-
(2000)
J. Lipid Res
, vol.41
, pp. 1154-1162
-
-
Solaas, K.1
Ulvestad, A.2
Soreide, O.3
Kase, B.F.4
-
32
-
-
34548158293
-
Quantifi cation and regulation of the subcellular distribution of bile acid coenzyme A:amino acid N-acyltransferase activity in rat liver
-
Styles, N. A., J. L. Falany, S. Barnes, and C. N. Falany. 2007. Quantifi cation and regulation of the subcellular distribution of bile acid coenzyme A:amino acid N-acyltransferase activity in rat liver. J. Lipid Res. 48: 1305-1315.
-
(2007)
J. Lipid Res
, vol.48
, pp. 1305-1315
-
-
Styles, N.A.1
Falany, J.L.2
Barnes, S.3
Falany, C.N.4
-
33
-
-
0025875876
-
Inborn errors of bile acid metabolism
-
Clayton, P. T. 1991. Inborn errors of bile acid metabolism. J. Inherit. Metab. Dis. 14: 478-496.
-
(1991)
J. Inherit. Metab. Dis
, vol.14
, pp. 478-496
-
-
Clayton, P.T.1
-
34
-
-
0023579798
-
-
van Eldere, J. R., G. G. Parmentier, H. J. Eyssen, R. J. Wanders, R. B. Schutgens, J. Vamecq, F. Van Hoof, B. T. Poll-The, and J. M. Saudubray. 1987. Bile acids in peroxisomal disorders. Eur. J. Clin. Invest. 17: 386-390.
-
van Eldere, J. R., G. G. Parmentier, H. J. Eyssen, R. J. Wanders, R. B. Schutgens, J. Vamecq, F. Van Hoof, B. T. Poll-The, and J. M. Saudubray. 1987. Bile acids in peroxisomal disorders. Eur. J. Clin. Invest. 17: 386-390.
-
-
-
-
35
-
-
0020317314
-
Structure of the side chain of the C29 dicarboxylic bile acid occurring in infants with coprostanic acidemia
-
Janssen, G., S. Toppet, and G. Parmentier. 1982. Structure of the side chain of the C29 dicarboxylic bile acid occurring in infants with coprostanic acidemia. J. Lipid Res. 23: 456-465.
-
(1982)
J. Lipid Res
, vol.23
, pp. 456-465
-
-
Janssen, G.1
Toppet, S.2
Parmentier, G.3
-
36
-
-
0018651545
-
C27 bile acids in infants with coprostanic acidemia and occurrence of a 3 alpha,7 alpha,12 alpha-tridhydroxy-5 beta-C29 dicarboxylic bile acid as a major component in their serum
-
Parmentier, G. G., G. A. Janssen, E. A. Eggermont, and H. J. Eyssen. 1979. C27 bile acids in infants with coprostanic acidemia and occurrence of a 3 alpha,7 alpha,12 alpha-tridhydroxy-5 beta-C29 dicarboxylic bile acid as a major component in their serum. Eur. J. Biochem. 102: 173-183.
-
(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
-
37
-
-
0022917472
-
Rapid diagnosis of Zellweger syndrome and infantile Refsum's disease by fast atom bombardment-mass spectrometry of urine bile salts
-
Lawson, A. M., M. J. Madigan, D. Shortland, and P. T. Clayton. 1986. Rapid diagnosis of Zellweger syndrome and infantile Refsum's disease by fast atom bombardment-mass spectrometry of urine bile salts. Clin. Chim. Acta. 161: 221-231.
-
(1986)
Clin. Chim. Acta
, vol.161
, pp. 221-231
-
-
Lawson, A.M.1
Madigan, M.J.2
Shortland, D.3
Clayton, P.T.4
-
38
-
-
0023487858
-
Occurrence of both (25R)- and (25S)-3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acids in urine from an infant with Zellweger's syndrome
-
Une, M., Y. Tazawa, K. Tada, and T. Hoshita. 1987. Occurrence of both (25R)- and (25S)-3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acids in urine from an infant with Zellweger's syndrome. J. Biochem. (Tokyo). 102: 1525-1530.
-
(1987)
J. Biochem. (Tokyo)
, vol.102
, pp. 1525-1530
-
-
Une, M.1
Tazawa, Y.2
Tada, K.3
Hoshita, T.4
-
39
-
-
0037354742
-
Urinary bile acid profi le in children with inborn errors of bile acid metabolism and chronic cholestasis; screening technique using electrospray tandem mass-spectrometry (ES/MS/MS)
-
Yousef, I. M., S. Perwaiz T. Lamireau, and B. Tuchweber. 2003. Urinary bile acid profi le in children with inborn errors of bile acid metabolism and chronic cholestasis; screening technique using electrospray tandem mass-spectrometry (ES/MS/MS). Med. Sci. Monit. 9: MT21-MT31.
-
(2003)
Med. Sci. Monit
, vol.9
-
-
Yousef, I.M.1
Perwaiz, S.2
Lamireau, T.3
Tuchweber, B.4
-
40
-
-
0035130658
-
Plasma analysis of di- and trihydroxycholestanoic acid diastereoisomers in peroxisomal alphamethylacyl- CoA racemase deficiency
-
Ferdinandusse, S., H. Overmars, S. Denis, H. R. Waterham, R. J. A. Wanders, and P. Vreken. 2001. Plasma analysis of di- and trihydroxycholestanoic acid diastereoisomers in peroxisomal alphamethylacyl- CoA racemase deficiency. J. Lipid Res. 42: 137-141.
-
(2001)
J. Lipid Res
, vol.42
, pp. 137-141
-
-
Ferdinandusse, S.1
Overmars, H.2
Denis, S.3
Waterham, H.R.4
Wanders, R.J.A.5
Vreken, P.6
-
41
-
-
0037219301
-
Liver disease caused by failure to racemize trihydroxycholestanoic acid: Gene mutation and effect of bile acid therapy
-
Setchell, K. D., J. E. Heubi, K. E. Bove, N. C. O'Connell, T. Brewsaugh, S. J. Steinberg, A. Moser, and R. H. Squires, Jr. 2003. Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy. Gastroenterology. 124: 217-232.
-
(2003)
Gastroenterology
, vol.124
, pp. 217-232
-
-
Setchell, K.D.1
Heubi, J.E.2
Bove, K.E.3
O'Connell, N.C.4
Brewsaugh, T.5
Steinberg, S.J.6
Moser, A.7
Squires Jr., R.H.8
-
42
-
-
0026668028
-
Substrate specifi cities of rat liver peroxisomal acyl-CoA oxidases: Palmitoyl-CoA oxidase (inducible acyl-CoA oxidase), pristanoyl-CoA oxidase (non-inducible acyl-CoA oxidase), and trihydroxycoprostanoyl-CoA oxidase
-
van Veldhoven, P. P., G. Vanhove, S. Assselberghs, H. J. Eyssen, and G. P. Mannaerts. 1992. Substrate specifi cities of rat liver peroxisomal acyl-CoA oxidases: palmitoyl-CoA oxidase (inducible acyl-CoA oxidase), pristanoyl-CoA oxidase (non-inducible acyl-CoA oxidase), and trihydroxycoprostanoyl-CoA oxidase. J. Biol. Chem. 267: 20065-20074.
-
(1992)
J. Biol. Chem
, vol.267
, pp. 20065-20074
-
-
van Veldhoven, P.P.1
Vanhove, G.2
Assselberghs, S.3
Eyssen, H.J.4
Mannaerts, G.P.5
-
43
-
-
0027316040
-
Studies on the substrate specifi city of the inducible and non-inducible acyl-CoA oxidases from rat kidney peroxisomes
-
Wanders, R. J., S. W. Denis, and G. Dacremont. 1993. Studies on the substrate specifi city of the inducible and non-inducible acyl-CoA oxidases from rat kidney peroxisomes. J. Biochem. (Tokyo). 113: 577-582.
-
(1993)
J. Biochem. (Tokyo)
, vol.113
, pp. 577-582
-
-
Wanders, R.J.1
Denis, S.W.2
Dacremont, G.3
-
44
-
-
34848897852
-
Clinical, biochemical, and mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency
-
Ferdinandusse, S., S. Denis, E. M. Hogenhout, J. Koster, C. W. van Roermund, L. IJlst, A. B. Moser, R. J. Wanders, and H. R. Waterham. 2007. Clinical, biochemical, and mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency. Hum. Mutat. 28: 904-912.
-
(2007)
Hum. Mutat
, vol.28
, pp. 904-912
-
-
Ferdinandusse, S.1
Denis, S.2
Hogenhout, E.M.3
Koster, J.4
van Roermund, C.W.5
IJlst, L.6
Moser, A.B.7
Wanders, R.J.8
Waterham, H.R.9
-
45
-
-
0030860855
-
Bile acid profiles in a peroxisomal D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein defi ciency
-
Une, M., M. Konishi, Y. Suzuki, S. Akaboshi, M. Yoshii, T. Kuramoto, and K. Fujimura. 1997. Bile acid profiles in a peroxisomal D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein defi ciency. J. Biochem. (Tokyo). 122: 655-658.
-
(1997)
J. Biochem. (Tokyo)
, vol.122
, pp. 655-658
-
-
Une, M.1
Konishi, M.2
Suzuki, Y.3
Akaboshi, S.4
Yoshii, M.5
Kuramoto, T.6
Fujimura, K.7
-
46
-
-
0032444553
-
Sensitive analysis of serum 3alpha, 7alpha, 12alpha,24-tetrahydroxy- 5beta-cholestan-26-oic acid diastereomers using gas chromatography-mass spectrometry and its application in peroxisomal D-bifunctional protein deficiency
-
Vreken, P., A. van Rooij, S. Denis, E. G. van Grunsven, D. A. Cuebas, and R. J. Wanders. 1998. Sensitive analysis of serum 3alpha, 7alpha, 12alpha,24-tetrahydroxy- 5beta-cholestan-26-oic acid diastereomers using gas chromatography-mass spectrometry and its application in peroxisomal D-bifunctional protein deficiency. J. Lipid Res. 39: 2452-2458.
-
(1998)
J. Lipid Res
, vol.39
, pp. 2452-2458
-
-
Vreken, P.1
van Rooij, A.2
Denis, S.3
van Grunsven, E.G.4
Cuebas, D.A.5
Wanders, R.J.6
-
47
-
-
0036566440
-
The role of alpha-methylacyl-CoA racemase in bile acid synthesis
-
Cuebas, D. A., C. Phillips, W. Schmitz, E. Conzelmann, and D. K. Novikov. 2002. The role of alpha-methylacyl-CoA racemase in bile acid synthesis. Biochem. J. 363: 801-807.
-
(2002)
Biochem. J
, vol.363
, pp. 801-807
-
-
Cuebas, D.A.1
Phillips, C.2
Schmitz, W.3
Conzelmann, E.4
Novikov, D.K.5
-
48
-
-
29944445799
-
Clinical and biochemical spectrum of D-bifunctional protein deficiency
-
Ferdinandusse, S., S. Denis, P. A. Mooyer, C. Dekker, M. Duran, R. J. Soorani-Lunsing, E. Boltshauser, A. Macaya, J. Gartner, C. B. Majoie, et al. 2006. Clinical and biochemical spectrum of D-bifunctional protein deficiency. Ann. Neurol. 59: 92-104.
-
(2006)
Ann. Neurol
, vol.59
, pp. 92-104
-
-
Ferdinandusse, S.1
Denis, S.2
Mooyer, P.A.3
Dekker, C.4
Duran, M.5
Soorani-Lunsing, R.J.6
Boltshauser, E.7
Macaya, A.8
Gartner, J.9
Majoie, C.B.10
-
49
-
-
33646885229
-
Mutations in the gene encoding peroxisomal sterol carrier protein X (SCPx) cause leukencephalopathy with dystonia and motor neuropathy
-
Ferdinandusse, S., P. Kostopoulos, S. Denis, H. Rusch, H. Overmars, U. Dillmann, W. Reith, D. Haas, R. J. A. Wanders, M. Duran, et al. 2006. Mutations in the gene encoding peroxisomal sterol carrier protein X (SCPx) cause leukencephalopathy with dystonia and motor neuropathy. Am. J. Hum. Genet. 78: 1046-1052.
-
(2006)
Am. J. Hum. Genet
, vol.78
, pp. 1046-1052
-
-
Ferdinandusse, S.1
Kostopoulos, P.2
Denis, S.3
Rusch, H.4
Overmars, H.5
Dillmann, U.6
Reith, W.7
Haas, D.8
Wanders, R.J.A.9
Duran, M.10
-
50
-
-
0033039199
-
Rapid analysis of conjugated bile acids in plasma using electrospray tandem mass spectrometry: Application for selective screening of peroxisomal disorders
-
Bootsma, A. H., H. Overmars, A. van Rooij, A. E. van Lint, R. J. Wanders, A. H. van Gennip, and P. Vreken. 1999. Rapid analysis of conjugated bile acids in plasma using electrospray tandem mass spectrometry: application for selective screening of peroxisomal disorders. J. Inherit. Metab. Dis. 22: 307-310.
-
(1999)
J. Inherit. Metab. Dis
, vol.22
, pp. 307-310
-
-
Bootsma, A.H.1
Overmars, H.2
van Rooij, A.3
van Lint, A.E.4
Wanders, R.J.5
van Gennip, A.H.6
Vreken, P.7
-
51
-
-
21444446060
-
Developmental changes of bile acid composition and conjugation in L- and D-bifunctional protein single and double knockout mice
-
Ferdinandusse, S., S. Denis, H. Overmars, L. Van Eeckhoudt, P. P. Van Veldhoven, M. Duran, R. J. Wanders, and M. Baes. 2005. Developmental changes of bile acid composition and conjugation in L- and D-bifunctional protein single and double knockout mice. J. Biol. Chem. 280: 18658-18666.
-
(2005)
J. Biol. Chem
, vol.280
, pp. 18658-18666
-
-
Ferdinandusse, S.1
Denis, S.2
Overmars, H.3
Van Eeckhoudt, L.4
Van Veldhoven, P.P.5
Duran, M.6
Wanders, R.J.7
Baes, M.8
-
52
-
-
0019918861
-
The effect of bile acid structure on the activity of bile acid-CoA:glycine/taurine-N-acetyltransferase
-
Czuba, B., and D. A. Vessey. 1982. The effect of bile acid structure on the activity of bile acid-CoA:glycine/taurine-N-acetyltransferase. J. Biol. Chem. 257: 8761-8765.
-
(1982)
J. Biol. Chem
, vol.257
, pp. 8761-8765
-
-
Czuba, B.1
Vessey, D.A.2
-
53
-
-
0033985195
-
Bile acid synthetic defects and liver disease
-
Bove, K. E., C. C. Daugherty, W. Tyson, G. Mierau, J. E. Heubi, W. F. Balistreri, and K. D. Setchell. 2000. Bile acid synthetic defects and liver disease. Pediatr. Dev. Pathol. 3: 1-16.
-
(2000)
Pediatr. Dev. Pathol
, vol.3
, pp. 1-16
-
-
Bove, K.E.1
Daugherty, C.C.2
Tyson, W.3
Mierau, G.4
Heubi, J.E.5
Balistreri, W.F.6
Setchell, K.D.7
-
54
-
-
49249110174
-
Mechanisms of disease: Inborn errors of bile acid synthesis
-
Sundaram, S. S., K. E. Bove, M. A. Lovell, and R. J. Sokol. 2008. Mechanisms of disease: inborn errors of bile acid synthesis. Nat. Clin. Pract. Gastroenterol. Hepatol. 5: 456-468.
-
(2008)
Nat. Clin. Pract. Gastroenterol. Hepatol
, vol.5
, pp. 456-468
-
-
Sundaram, S.S.1
Bove, K.E.2
Lovell, M.A.3
Sokol, R.J.4
-
55
-
-
19444371845
-
Peroxisome biogenesis disorders: The role of peroxisomes and metabolic dysfunction in developing brain
-
Faust, P. L., D. Banka, R. Siriratsivawong, V. G. Ng, and T. M. Wikander. 2005. Peroxisome biogenesis disorders: the role of peroxisomes and metabolic dysfunction in developing brain. J. Inherit. Metab. Dis. 28: 369-383.
-
(2005)
J. Inherit. Metab. Dis
, vol.28
, pp. 369-383
-
-
Faust, P.L.1
Banka, D.2
Siriratsivawong, R.3
Ng, V.G.4
Wikander, T.M.5
-
56
-
-
60649100200
-
Toxicity of peroxisomal C27-bile acid intermediates
-
Ferdinandusse, S., S. Denis, G. Dacremont, and R. J. Wanders. 2009. Toxicity of peroxisomal C27-bile acid intermediates. Mol. Genet. Metab. 96: 121-128.
-
(2009)
Mol. Genet. Metab
, vol.96
, pp. 121-128
-
-
Ferdinandusse, S.1
Denis, S.2
Dacremont, G.3
Wanders, R.J.4
-
57
-
-
4444311570
-
Mitochondrially-mediated toxicity of bile acids
-
Palmeira, C. M., and A. P. Rolo. 2004. Mitochondrially-mediated toxicity of bile acids. Toxicology. 203: 1-15.
-
(2004)
Toxicology
, vol.203
, pp. 1-15
-
-
Palmeira, C.M.1
Rolo, A.P.2
-
58
-
-
0015848845
-
Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome
-
Goldfischer, S., C. L. Moore, A. B. Johnson, A. J. Spiro, M. P. Valsamis, H. K. Wisniewski, R. H. Ritch, W. T. Norton, I. Rapin, and L. M. Gartner. 1973. Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome. Science. 182: 62-64.
-
(1973)
Science
, vol.182
, pp. 62-64
-
-
Goldfischer, S.1
Moore, C.L.2
Johnson, A.B.3
Spiro, A.J.4
Valsamis, M.P.5
Wisniewski, H.K.6
Ritch, R.H.7
Norton, W.T.8
Rapin, I.9
Gartner, L.M.10
-
59
-
-
16244397057
-
Absence of peroxisomes in mouse hepatocytes causes mitochondrial and ER abnormalities
-
Dirkx, R., I. Vanhorebeek, K. Martens, A. Schad, M. Grabenbauer, D. Fahimi, P. Declercq, P. P. Van Veldhoven, and M. Baes. 2005. Absence of peroxisomes in mouse hepatocytes causes mitochondrial and ER abnormalities. Hepatology. 41: 868-878.
-
(2005)
Hepatology
, vol.41
, pp. 868-878
-
-
Dirkx, R.1
Vanhorebeek, I.2
Martens, K.3
Schad, A.4
Grabenbauer, M.5
Fahimi, D.6
Declercq, P.7
Van Veldhoven, P.P.8
Baes, M.9
-
60
-
-
0034799180
-
Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse)
-
Baumgart, E., I. Vanhorebeek, M. Grabenbauer, M. Borgers, P. E. Declercq, H. D. Fahimi, and M. Baes. 2001. Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse). Am. J. Pathol. 159: 1477-1494.
-
(2001)
Am. J. Pathol
, vol.159
, pp. 1477-1494
-
-
Baumgart, E.1
Vanhorebeek, I.2
Grabenbauer, M.3
Borgers, M.4
Declercq, P.E.5
Fahimi, H.D.6
Baes, M.7
-
61
-
-
0025089946
-
Pathology of hepatic peroxisomes and mitochondria in patients with peroxisomal disorders
-
Hughes, J. L., A. Poulos, E. Robertson, C. W. Chow, L. J. Sheffield, J. Christodoulou, and R. F. Carter. 1990. Pathology of hepatic peroxisomes and mitochondria in patients with peroxisomal disorders. Virchows Arch. A Pathol. Anat. Histopathol. 416: 255-264.
-
(1990)
Virchows Arch. A Pathol. Anat. Histopathol
, vol.416
, pp. 255-264
-
-
Hughes, J.L.1
Poulos, A.2
Robertson, E.3
Chow, C.W.4
Sheffield, L.J.5
Christodoulou, J.6
Carter, R.F.7
-
62
-
-
0019168913
-
Liver in the cerebro-hepato-renal syndrome: Defective bile acid synthesis and abnormal mitochondria
-
Mathis, R. K., J. B. Watkins, P. Szczepanik-Van Leeuwen, and I. T. Lott. 1980. Liver in the cerebro-hepato-renal syndrome: defective bile acid synthesis and abnormal mitochondria. Gastroenterology. 79: 1311-1317.
-
(1980)
Gastroenterology
, vol.79
, pp. 1311-1317
-
-
Mathis, R.K.1
Watkins, J.B.2
Szczepanik-Van Leeuwen, P.3
Lott, I.T.4
-
63
-
-
0036667787
-
Oral bile acid treatment in two Japanese patients with Zellweger syndrome
-
Maeda, K., A. Kimura, Y. Yamato, H. Nittono, H. Takei, T. Sato, H. Mitsubuchi, T. Murai, and T. Kurosawa. 2002. Oral bile acid treatment in two Japanese patients with Zellweger syndrome. J. Pediatr. Gastroenterol. Nutr. 35: 227-230.
-
(2002)
J. Pediatr. Gastroenterol. Nutr
, vol.35
, pp. 227-230
-
-
Maeda, K.1
Kimura, A.2
Yamato, Y.3
Nittono, H.4
Takei, H.5
Sato, T.6
Mitsubuchi, H.7
Murai, T.8
Kurosawa, T.9
-
64
-
-
0026571062
-
Oral bile acid treatment and the patient with Zellweger syndrome
-
Setchell, K. D., P. Bragetti, L. Zimmer-Nechemias, C. Daugherty, M. A. Pelli, R. Vaccaro, G. Gentili, E. Distrutti, G. Dozzini, A. Morelli, et al. 1992. Oral bile acid treatment and the patient with Zellweger syndrome. Hepatology. 15: 198-207.
-
(1992)
Hepatology
, vol.15
, pp. 198-207
-
-
Setchell, K.D.1
Bragetti, P.2
Zimmer-Nechemias, L.3
Daugherty, C.4
Pelli, M.A.5
Vaccaro, R.6
Gentili, G.7
Distrutti, E.8
Dozzini, G.9
Morelli, A.10
-
65
-
-
33745740400
-
Defects in bile acid biosynthesis-diagnosis and treatment
-
Setchell, K. D., and J. E. Heubi. 2006. Defects in bile acid biosynthesis-diagnosis and treatment. J. Pediatr. Gastroenterol. Nutr. 43 (Suppl 1): S17-S22.
-
(2006)
J. Pediatr. Gastroenterol. Nutr
, vol.43
, Issue.SUPPL. 1
-
-
Setchell, K.D.1
Heubi, J.E.2
-
66
-
-
34247354077
-
Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger mice
-
Keane, M. H., H. Overmars, T. M. Wikander, S. Ferdinandusse, M. Duran, R. J. Wanders, and P. L. Faust. 2007. Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger mice. Hepatology. 45: 982-997.
-
(2007)
Hepatology
, vol.45
, pp. 982-997
-
-
Keane, M.H.1
Overmars, H.2
Wikander, T.M.3
Ferdinandusse, S.4
Duran, M.5
Wanders, R.J.6
Faust, P.L.7
-
67
-
-
0346690402
-
Hepatoprotection by the farnesoid X receptor agonist GW4064 in rat models of intra- and extrahepatic cholestasis
-
Liu, Y., J. Binz, M. J. Numerick, S. Dennis, G. Luo, B. Desai, K. I. MacKenzie, T. A. Mansfi eld, S. A. Kliewer, B. Goodwin, et al. 2003. Hepatoprotection by the farnesoid X receptor agonist GW4064 in rat models of intra- and extrahepatic cholestasis. J. Clin. Invest. 112: 1678-1687.
-
(2003)
J. Clin. Invest
, vol.112
, pp. 1678-1687
-
-
Liu, Y.1
Binz, J.2
Numerick, M.J.3
Dennis, S.4
Luo, G.5
Desai, B.6
MacKenzie, K.I.7
Mansfi eld, T.A.8
Kliewer, S.A.9
Goodwin, B.10
|