-
1
-
-
0031907132
-
A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis
-
Bull LN, van Eijk MJ, Pawlikowska L, DeYoung JA, Juijn JA, et al. (1998) A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis. Nat Genet 18: 219-224.
-
(1998)
Nat Genet
, vol.18
, pp. 219-224
-
-
Bull, L.N.1
van Eijk, M.J.2
Pawlikowska, L.3
DeYoung, J.A.4
Juijn, J.A.5
-
2
-
-
0029992825
-
A subfamily of P-type ATPases with aminophospholipid transporting activity
-
Tang X, Halleck MS, Schlegel RA, Williamson P (1996) A subfamily of P-type ATPases with aminophospholipid transporting activity. Science 272: 1495-1497.
-
(1996)
Science
, vol.272
, pp. 1495-1497
-
-
Tang, X.1
Halleck, M.S.2
Schlegel, R.A.3
Williamson, P.4
-
5
-
-
0034812920
-
Familial intrahepatic cholestasis 1: Studies of localization and function
-
Ujhazy P, Ortiz D, Misra S, Li S, Moseley J, et al. (2001) Familial intrahepatic cholestasis 1: Studies of localization and function. Hepatology 34: 768-775.
-
(2001)
Hepatology
, vol.34
, pp. 768-775
-
-
Ujhazy, P.1
Ortiz, D.2
Misra, S.3
Li, S.4
Moseley, J.5
-
6
-
-
38649113883
-
ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity
-
Paulusma CC, Folmer DE, Ho-Mok KS, de Waart DR, Hilarius PM, et al. (2008) ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity. Hepatology 47: 268-278.
-
(2008)
Hepatology
, vol.47
, pp. 268-278
-
-
Paulusma, C.C.1
Folmer, D.E.2
Ho-Mok, K.S.3
de Waart, D.R.4
Hilarius, P.M.5
-
7
-
-
60449097716
-
ATP8B1 Deficiency Disrupts the Bile Canalicular Membrane Bilayer Structure in Hepatocytes, But FXR Expression and Activity Are Maintained
-
Cai SY, Gautam S, Nguyen T, Soroka CJ, Rahner C, et al. (2008) ATP8B1 Deficiency Disrupts the Bile Canalicular Membrane Bilayer Structure in Hepatocytes, But FXR Expression and Activity Are Maintained. Gastroenterology 136: 1060-9.
-
(2008)
Gastroenterology
, vol.136
, pp. 1060-1069
-
-
Cai, S.Y.1
Gautam, S.2
Nguyen, T.3
Soroka, C.J.4
Rahner, C.5
-
8
-
-
0033652101
-
A missense mutation in FIC1 is associated with greenland familial cholestasis
-
Klomp LW, Bull LN, Knisely AS, van Der Doelen MA, Juijn JA, et al. (2000) A missense mutation in FIC1 is associated with greenland familial cholestasis. Hepatology 32: 1337-1341.
-
(2000)
Hepatology
, vol.32
, pp. 1337-1341
-
-
Klomp, L.W.1
Bull, L.N.2
Knisely, A.S.3
van Der Doelen, M.A.4
Juijn, J.A.5
-
9
-
-
3042782537
-
Characterization of mutations in ATP8B1 associated with hereditary cholestasis
-
Klomp LW, Vargas JC, van Mil SW, Pawlikowska L, Strautnieks SS, et al. (2004) Characterization of mutations in ATP8B1 associated with hereditary cholestasis. Hepatology 40: 27-38.
-
(2004)
Hepatology
, vol.40
, pp. 27-38
-
-
Klomp, L.W.1
Vargas, J.C.2
van Mil, S.W.3
Pawlikowska, L.4
Strautnieks, S.S.5
-
10
-
-
0014442684
-
Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred
-
Clayton RJ, Iber FL, Ruebner BH, McKusick VA (1969) Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 117: 112-124.
-
(1969)
Am J Dis Child
, vol.117
, pp. 112-124
-
-
Clayton, R.J.1
Iber, F.L.2
Ruebner, B.H.3
McKusick, V.A.4
-
12
-
-
11144355538
-
A mouse genetic model for familial cholestasis caused by ATP8B1 mutatons reveals perturbed bile salt homeostasis but no impairment in bile secretion
-
Pawlikowska L, Groen AK, Eppens EF, Kunne C, Ottenhoff R, et al. (2004) A mouse genetic model for familial cholestasis caused by ATP8B1 mutatons reveals perturbed bile salt homeostasis but no impairment in bile secretion. Hum Mol Genet 15: 881-892.
-
(2004)
Hum Mol Genet
, vol.15
, pp. 881-892
-
-
Pawlikowska, L.1
Groen, A.K.2
Eppens, E.F.3
Kunne, C.4
Ottenhoff, R.5
-
13
-
-
33745906532
-
Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport
-
Paulusma CC, Groen A, Kunne C, Ho-Mok KS, Spijkerboer AL, et al. (2006) Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport. Hepatology 44: 195-204.
-
(2006)
Hepatology
, vol.44
, pp. 195-204
-
-
Paulusma, C.C.1
Groen, A.2
Kunne, C.3
Ho-Mok, K.S.4
Spijkerboer, A.L.5
-
14
-
-
44649139336
-
Abcg5/8 independent biliary cholesterol excretion in Atp8b1-deficient mice
-
Groen A, Kunne C, Jongsma G, van den Oever K, Mok KS, et al. (2008) Abcg5/8 independent biliary cholesterol excretion in Atp8b1-deficient mice. Gastroenterology 134: 2091-2100.
-
(2008)
Gastroenterology
, vol.134
, pp. 2091-2100
-
-
Groen, A.1
Kunne, C.2
Jongsma, G.3
van den Oever, K.4
Mok, K.S.5
-
15
-
-
33846128172
-
Increased serum concentrations of secondary bile salts during cholate feeding are due to coprophagy. A study with wild-type and Atp8b1-deficient mice
-
Groen A, Kunne C, Oude Elferink RP (2006) Increased serum concentrations of secondary bile salts during cholate feeding are due to coprophagy. A study with wild-type and Atp8b1-deficient mice. Mol Pharm 3: 756-761.
-
(2006)
Mol Pharm
, vol.3
, pp. 756-761
-
-
Groen, A.1
Kunne, C.2
Oude Elferink, R.P.3
-
16
-
-
34249312304
-
Intestinal bile salt absorption in Atp8b1 deficient mice
-
Groen A, Kunne C, Paulusma CC, Kramer W, Agellon LB, et al. (2007) Intestinal bile salt absorption in Atp8b1 deficient mice. J Hepatol 47: 114-122.
-
(2007)
J Hepatol
, vol.47
, pp. 114-122
-
-
Groen, A.1
Kunne, C.2
Paulusma, C.C.3
Kramer, W.4
Agellon, L.B.5
-
18
-
-
34347358580
-
Depletion of high-density lipoprotein and appearance of triglyceride-rich low-density lipoprotein in a Japanese patient with FIC1 deficiency manifesting benign recurrent intrahepatic cholestasis
-
Nagasaka H, Chiba H, Hui SP, Takikawa H, Miida T, et al. (2007) Depletion of high-density lipoprotein and appearance of triglyceride-rich low-density lipoprotein in a Japanese patient with FIC1 deficiency manifesting benign recurrent intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 45: 96-105.
-
(2007)
J Pediatr Gastroenterol Nutr
, vol.45
, pp. 96-105
-
-
Nagasaka, H.1
Chiba, H.2
Hui, S.P.3
Takikawa, H.4
Miida, T.5
-
19
-
-
25144441493
-
Resistance to parathyroid hormone in two patients with familial intrahepatic cholestasis: Possible involvement of the ATP8B1 gene in calcium regulation via parathyroid hormone
-
Nagasaka H, Yorifuji T, Kosugiyama K, Egawa H, Kawai M, et al. (2004) Resistance to parathyroid hormone in two patients with familial intrahepatic cholestasis: possible involvement of the ATP8B1 gene in calcium regulation via parathyroid hormone. J Pediatr Gastroenterol Nutr 39: 404-409.
-
(2004)
J Pediatr Gastroenterol Nutr
, vol.39
, pp. 404-409
-
-
Nagasaka, H.1
Yorifuji, T.2
Kosugiyama, K.3
Egawa, H.4
Kawai, M.5
-
20
-
-
77749240873
-
Differences in presentation and progression between severe FIC1 and BSEP deficiencies
-
in press
-
Pawlikowska L, Strautnieks S, Jankowska I, Czubkowski P, Emerick K, et al. Differences in presentation and progression between severe FIC1 and BSEP deficiencies. J Hepat in press.
-
J Hepat
-
-
Pawlikowska, L.1
Strautnieks, S.2
Jankowska, I.3
Czubkowski, P.4
Emerick, K.5
-
21
-
-
0036218724
-
Bile acid-activated nuclear receptor FXR suppresses apolipoprotein A-I transcription via a negative FXR response element
-
Claudel T, Sturm E, Duez H, Torra IP, Sirvent A, et al. (2002) Bile acid-activated nuclear receptor FXR suppresses apolipoprotein A-I transcription via a negative FXR response element. J Clin Invest 109: 961-971.
-
(2002)
J Clin Invest
, vol.109
, pp. 961-971
-
-
Claudel, T.1
Sturm, E.2
Duez, H.3
Torra, I.P.4
Sirvent, A.5
-
22
-
-
0035102561
-
The abnormal lipid spectrum in malignant obstructive jaundice in relation to endotoxin sensitivity and the result of preoperative biliary drainage
-
Kimmings N, Sewnath ME, Mairuhu WM, Van Zanten AP, Rauws EA, et al. (2001) The abnormal lipid spectrum in malignant obstructive jaundice in relation to endotoxin sensitivity and the result of preoperative biliary drainage. Surgery 129: 282-291.
-
(2001)
Surgery
, vol.129
, pp. 282-291
-
-
Kimmings, N.1
Sewnath, M.E.2
Mairuhu, W.M.3
Van Zanten, A.P.4
Rauws, E.A.5
-
23
-
-
0015245202
-
Cholestasis: Lamellar structure of the abnormal human serum lipoprotein
-
Hamilton RL, Havel RJ, Kane JP, Blaurock AE, Sata T (1971) Cholestasis: lamellar structure of the abnormal human serum lipoprotein. Science 172: 475-478.
-
(1971)
Science
, vol.172
, pp. 475-478
-
-
Hamilton, R.L.1
Havel, R.J.2
Kane, J.P.3
Blaurock, A.E.4
Sata, T.5
-
24
-
-
0032212897
-
Class III P-glycoproteins mediate the formation of lipoprotein X in the mouse
-
Elferink RP, Ottenhoff R, van Marle J, Frijters CM, Smith AJ, et al. (1998) Class III P-glycoproteins mediate the formation of lipoprotein X in the mouse. J Clin Invest 102: 1749-1757.
-
(1998)
J Clin Invest
, vol.102
, pp. 1749-1757
-
-
Elferink, R.P.1
Ottenhoff, R.2
van Marle, J.3
Frijters, C.M.4
Smith, A.J.5
-
25
-
-
20044388117
-
Evaluation of risk for atherosclerosis in Alagille syndrome and progressive familial intrahepatic cholestasis: Two congenital cholestatic diseases with different lipoprotein metabolisms
-
Nagasaka H, Yorifuji T, Egawa H, Yanai H, Fujisawa T, et al. (2005) Evaluation of risk for atherosclerosis in Alagille syndrome and progressive familial intrahepatic cholestasis: two congenital cholestatic diseases with different lipoprotein metabolisms. J Pediatr 146: 329-335.
-
(2005)
J Pediatr
, vol.146
, pp. 329-335
-
-
Nagasaka, H.1
Yorifuji, T.2
Egawa, H.3
Yanai, H.4
Fujisawa, T.5
-
26
-
-
65649087129
-
Activity of the bile salt export pump (ABCB11) is critically dependent on canalicular membrane cholesterol content
-
Paulusma CC, de Waart DR, Kunne C, Mok KS, Elferink RP (2009) Activity of the bile salt export pump (ABCB11) is critically dependent on canalicular membrane cholesterol content. J Biol Chem 284: 9947-9954.
-
(2009)
J Biol Chem
, vol.284
, pp. 9947-9954
-
-
Paulusma, C.C.1
de Waart, D.R.2
Kunne, C.3
Mok, K.S.4
Elferink, R.P.5
-
27
-
-
0028790118
-
Regulation of protein secretion into bile: Studies in mice with a disrupted mdr2 p-glycoprotein gene
-
Groen AK, Van Wijland MJ, Frederiks WM, Smit JJ, Schinkel AH, et al. (1995) Regulation of protein secretion into bile: studies in mice with a disrupted mdr2 p-glycoprotein gene. Gastroenterology 109: 1997-2006.
-
(1995)
Gastroenterology
, vol.109
, pp. 1997-2006
-
-
Groen, A.K.1
Van Wijland, M.J.2
Frederiks, W.M.3
Smit, J.J.4
Schinkel, A.H.5
-
28
-
-
0000120766
-
Estimating the Dimension of a Model
-
Schwarz G (1978) Estimating the Dimension of a Model. Ann Stat 6: 461-464.
-
(1978)
Ann Stat
, vol.6
, pp. 461-464
-
-
Schwarz, G.1
-
29
-
-
28444445926
-
-
R Foundation for Statistical Computing. Vienna: Austria. ISBN 3-900051-07-0, URL
-
R Development Core Team. (2005) R: A language and environment for statistical computing. R Foundation for Statistical Computing. Vienna: Austria. ISBN 3-900051-07-0, URL http://www.R-project.org.
-
(2005)
R: A language and environment for statistical computing
-
-
|