Wagner M, Trauner M

Idioma: Ingles.
Referencias bibliográficas: 324
Paginas: 77-99
Archivo PDF: 218.90 Kb.

FRAGMENTO

Sin resumen

REFERENCIAS (EN ESTE ARTÍCULO)

1. Hagenbuch B, Stieger B, Foguet M, Lubbert H, Meier PJ. Functional expression cloning and characterization of the hepatocyte Na+/bile acid cotransport system. Proc Natl Acad Sci U S A 1991; 88(23): 10629-33.

2. Trauner M, Boyer JL. Bile salt transporters: molecular characterization, function, and regulation. Physiol Rev 2003; 83(2): 633-71.

3. Kullak-Ublick GA, Stieger B, Meier PJ. Enterohepatic bile salt transporters in normal physiology and liver disease. Gastroenterology 2004; 126(1): 322-42.

4. Meier PJ, Stieger B. Bile salt transporters. Annu Rev Physiol 2002; 64: 635-61.

5. Jansen PL, Sturm E. Genetic cholestasis, causes and consequences for hepatobiliary transport. Liver Int 2003; 23(5): 315-22.

6. Trauner M, Meier PJ, Boyer JL. Molecular pathogenesis of cholestasis. N Engl J Med 1998; 339(17): 1217-27.

7. Anwer MS. Cellular regulation of hepatic bile acid transport in health and cholestasis. Hepatology 2004; 39(3): 581-90.

8. Eloranta JJ, Kullak-Ublick GA. Coordinate transcriptional regulation of bile acid homeostasis and drug metabolism. Arch Biochem Biophys 2005; 433(2): 397-412.

9. Karpen SJ. Nuclear receptor regulation of hepatic function. J Hepatol 2002; 36(6): 832-50.

10. Wolkoff AW, Cohen DE. Bile acid regulation of hepatic physiology: I. Hepatocyte transport of bile acids. Am J Physiol Gastrointest Liver Physiol 2003; 284(2): G175-G179.

11. Chiang JY. Bile acid regulation of hepatic physiology: III. Bile acids and nuclear receptors. Am J Physiol Gastrointest Liver Physiol 2003; 284(3): G349-G356.

12. Hofmann AF. The continuing importance of bile acids in liver and intestinal disease. Arch Intern Med 1999; 159(22): 2647-58.

13. Kullak-Ublick GA, Stieger B, Hagenbuch B, Meier PJ. Hepatic transport of bile salts. Semin Liver Dis 2000; 20(3): 273-92.

14. Hofmann AF. Bile Acids: The Good, the Bad, and the Ugly. News Physiol Sci 1999; 14: 24-9.

15. Hofmann AF. Cholestatic liver disease: pathophysiology and therapeutic options. Liver 2002; 22 Suppl 2: 14-9.

16. Chiang JY. Bile acid regulation of gene expression: roles of nuclear hormone receptors. Endocr Rev 2002; 23(4): 443-63.

17. De FE, Mitro N, Godio C, Gilardi F, Caruso D, Crestani M. Bile acid signaling to the nucleus: finding new connections in the transcriptional regulation of metabolic pathways. Biochimie 2004; 86(11): 771-8.

18. Chiang JY. Regulation of bile acid synthesis: pathways, nuclear receptors, and mechanisms. J Hepatol 2004; 40(3): 539-51.

19. Russell DW. The enzymes, regulation, and genetics of bile acid synthesis. Annu Rev Biochem 2003; 72: 137-74.

20. Duane WC, Javitt NB. 27-hydroxycholesterol: production rates in normal human subjects. J Lipid Res 1999; 40(7): 1194-9.

21. Axelson M, Sjovall J. Potential bile acid precursors in plasma—possible indicators of biosynthetic pathways to cholic and chenodeoxycholic acids in man. J Steroid Biochem 1990; 36(6): 631-40.

22. Radominska-Pyrek A, Zimniak P, Irshaid YM, Lester R, Tephly TR, St PJ. Glucuronidation of 6 alpha-hydroxy bile acids by human liver microsomes. J Clin Invest 1987; 80(1): 234-41.

23. Takikawa H, Otsuka H, Beppu T, Seyama Y, Yamakawa T. Serum concentrations of bile acid glucuronides in hepatobiliary diseases. Digestion 1983; 27(4): 189-95.

24. Marschall HU, Matern H, Egestad B, Matern S, Sjovall S. 6 alpha-glucuronidation of hyodeoxycholic acid by human liver, kidney and small bowel microsomes. Biochim Biophys Acta 1987; 921(2): 392-7.

25. van Berge Henegouwen GP, Brandt KH, Eyssen H, Parmentier G. Sulphated and unsulphated bile acids in serum, bile, and urine of patients with cholestasis. Gut 1976; 17(11): 861-9.

26. Radominska A, Comer KA, Zimniak P, Falany J, Iscan M, Falany CN. Human liver steroid sulphotransferase sulphates bile acids. Biochem J 1990; 272(3): 597-604.

27. Palmer RH. The formation of bile acid sulfates: a new pathway of bile acid metabolism in humans. Proc Natl Acad Sci USA 1967; 58(3): 1047-50.

28. Meier PJ. Molecular mechanisms of hepatic bile salt transport from sinusoidal blood into bile. Am J Physiol 1995; 269(6 Pt 1): G801-G812.

29. Arias IM. The biology of hepatic endothelial cell fenestrae. Prog Liver Dis 1990; 9: 11-26.

30. Berk PD, Potter BJ, Stremmel W. Role of plasma membrane ligand-binding proteins in the hepatocellular uptake of albumin-bound organic anions. Hepatology 1987; 7(1): 165-76.

31. Reichen J. The Role of the Sinusoidal Endothelium in Liver Function. News Physiol Sci 1999; 14: 117-21.

32. Layden TJ, Boyer JL. Influence of bile acids on bile canalicular membrane morphology and the lobular gradient in canalicular size. Lab Invest 1978; 39(2): 110-9.

33. Meier PJ, Eckhardt U, Schroeder A, Hagenbuch B, Stieger B. Substrate specificity of sinusoidal bile acid and organic anion uptake systems in rat and human liver. Hepatology 1997; 26(6): 1667-77.

34. Hagenbuch B, Meier PJ. The superfamily of organic anion transporting polypeptides. Biochim Biophys Acta 2003; 1609(1): 1-18.

35. St Pierre MV, Kullak-Ublick GA, Hagenbuch B, Meier PJ. Transport of bile acids in hepatic and non-hepatic tissues. J Exp Biol 2001; 204(Pt 10): 1673-86.

36. Hirohashi T, Suzuki H, Takikawa H, Sugiyama Y. ATP-dependent transport of bile salts by rat multidrug resistance-associated protein 3 (Mrp3). J Biol Chem 2000; 275(4): 2905-10.

37. Zelcer N, Reid G, Wielinga P, Kuil A, van dH, I, Schuetz JD, Borst P. Steroid and bile acid conjugates are substrates of human multidrug-resistance protein (MRP) 4 (ATP-binding cassette C4). Biochem J 2003; 371(Pt 2): 361-7.

38. Rius M, Nies AT, Hummel-Eisenbeiss J, Jedlitschky G, Keppler D. Cotransport of reduced glutathione with bile salts by MRP4 (ABCC4) localized to the basolateral hepatocyte membrane. Hepatology 2003; 38(2): 374-84.

39. Li L, Meier PJ, Ballatori N. Oatp2 mediates bidirectional organic solute transport: a role for intracellular glutathione. Mol Pharmacol 2000; 58(2): 335-40.

40. Seward DJ, Koh AS, Boyer JL, Ballatori N. Functional complementation between a novel mammalian polygenic transport complex and an evolutionarily ancient organic solute transporter, OSTalpha-OSTbeta. J Biol Chem 2003; 278(30): 27473-82.

41. Dawson PA, Hubbert M, Haywood J, Craddock AL, Zerangue N, Christian WV, Ballatori N. The Heteromeric Organic Solute Transporter {alpha}-{beta}, Ost{alpha}-Ost{beta}, Is an Ileal Basolateral Bile Acid Transporter. J Biol Chem 2005; 280(8): 6960-8.

42. Agellon LB, Torchia EC. Intracellular transport of bile acids. Biochim Biophys Acta 2000; 1486(1): 198-209.

43. Jansen PL, Strautnieks SS, Jacquemin E, Hadchouel M, Sokal EM, Hooiveld GJ, Koning JH, et al. Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis. Gastroenterology 1999; 117(6): 1370-9.

44. Wang R, Salem M, Yousef IM, Tuchweber B, Lam P, Childs SJ, Helgason CD, et al. Targeted inactivation of sister of P-glycoprotein gene (spgp) in mice results in nonprogressive but persistent intrahepatic cholestasis. Proc Natl Acad Sci USA 2001; 98(4): 2011-6.

45. Akita H, Suzuki H, Ito K, Kinoshita S, Sato N, Takikawa H, Sugiyama Y. Characterization of bile acid transport mediated by multidrug resistance associated protein 2 and bile salt export pump. Biochim Biophys Acta 2001; 1511(1): 7-16.

46. Keppler D, Konig J. Hepatic secretion of conjugated drugs and endogenous substances. Semin Liver Dis 2000; 20(3): 265-72.

47. Suzuki M, Suzuki H, Sugimoto Y, Sugiyama Y. ABCG2 transports sulfated conjugates of steroids and xenobiotics. J Biol Chem 2003; 278(25): 22644-9.

48. Smit JJ, Schinkel AH, Oude Elferink RP, Groen AK, Wagenaar E, van DL, Mol CA, et al. Homozygous disruption of the murine mdr2 P-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease. Cell 1993; 75(3): 451-62.

49. Yu L, Hammer RE, Li-Hawkins J, von BK, Lutjohann D, Cohen JC, Hobbs HH. Disruption of Abcg5 and Abcg8 in mice reveals their crucial role in biliary cholesterol secretion. Proc Natl Acad Sci USA 2002; 99(25): 16237-42.

50. Meier PJ, Knickelbein R, Moseley RH, Dobbins JW, Boyer JL. Evidence for carrier-mediated chloride/bicarbonate exchange in canalicular rat liver plasma membrane vesicles. J Clin Invest 1985; 75(4): 1256-63.

51. Kanno N, Lesage G, Glaser S, Alvaro D, Alpini G. Functional heterogeneity of the intrahepatic biliary epithelium. Hepatology 2000; 31(3): 555-61.

52. Hofmann AF. The cholehepatic circulation of unconjugated bile acids: an update. In: Paumgartner G, Stiehl A, Gerok W, editors. Bile acids and the hepatobiliary system. Boston: Kluwer; 1993.p.143-60.

53. Lazaridis KN, Pham L, Tietz P, Marinelli RA, deGroen PC, Levine S, Dawson PA, et al. Rat cholangiocytes absorb bile acids at their apical domain via the ileal sodium-dependent bile acid transporter. J Clin Invest 1997; 100(11): 2714-21.

54. Benedetti A, Di SA, Marucci L, Svegliati-Baroni G, Schteingart CD, Ton-Nu HT, Hofmann AF. Carrier-mediated transport of conjugated bile acids across the basolateral membrane of biliary epithelial cells. Am J Physiol 1997; 272(6 Pt 1): G1416-G1424.

55. Chignard N, Mergey M, Veissiere D, Parc R, Capeau J, Poupon R, Paul A, et al. Bile acid transport and regulating functions in the human biliary epithelium. Hepatology 2001; 33(3): 496-503.

56. Soroka CJ, Lee JM, Azzaroli F, Boyer JL. Cellular localization and up-regulation of multidrug resistance-associated protein 3 in hepatocytes and cholangiocytes during obstructive cholestasis in rat liver. Hepatology 2001; 33(4): 783-91.

57. Kool M, van der LM, de HM, Scheffer GL, de Vree JM, Smith AJ, Jansen G, et al. MRP3, an organic anion transporter able to transport anti-cancer drugs. Proc Natl Acad Sci USA 1999; 96(12): 6914-9.

58. Lazaridis KN, Tietz P, Wu T, Kip S, Dawson PA, LaRusso NF. Alternative splicing of the rat sodium/bile acid transporter changes its cellular localization and transport properties. Proc Natl Acad Sci USA 2000; 97(20): 11092-7.

59. Craddock AL, Love MW, Daniel RW, Kirby LC, Walters HC, Wong MH, Dawson PA. Expression and transport properties of the human ileal and renal sodium-dependent bile acid transporter. Am J Physiol 1998; 274(1 Pt 1): G157-G169.

60. Walters HC, Craddock AL, Fusegawa H, Willingham MC, Dawson PA. Expression, transport properties, and chromosomal location of organic anion transporter subtype 3. Am J Physiol Gastrointest Liver Physiol 2000; 279(6): G1188-G1200.

61. Amelsberg A, Jochims C, Richter CP, Nitsche R, Folsch UR. Evidence for an anion exchange mechanism for uptake of conjugated bile acid from the rat jejunum. Am J Physiol 1999; 276(3 Pt 1): G737-G742.

62. Gong YZ, Everett ET, Schwartz DA, Norris JS, Wilson FA. Molecular cloning, tissue distribution, and expression of a 14-kDa bile acid-binding protein from rat ileal cytosol. Proc Natl Acad Sci USA 1994; 91(11): 4741-5.

63. Hirohashi T, Suzuki H, Ito K, Ogawa K, Kume K, Shimizu T, Sugiyama Y. Hepatic expression of multidrug resistance-associated protein-like proteins maintained in eisai hyperbilirubinemic rats. Mol Pharmacol 1998; 53(6): 1068-75.

64. Zeng H, Liu G, Rea PA, Kruh GD. Transport of amphipathic anions by human multidrug resistance protein 3. Cancer Res 2000; 60(17): 4779-84.

65. Cherrington NJ, Hartley DP, Li N, Johnson DR, Klaassen CD. Organ distribution of multidrug resistance proteins 1, 2, and 3 (Mrp1, 2, and 3) mRNA and hepatic induction of Mrp3 by constitutive androstane receptor activators in rats. J Pharmacol Exp Ther 2002; 300(1): 97-104.

66. Rost D, Mahner S, Sugiyama Y, Stremmel W. Expression and localization of the multidrug resistance-associated protein 3 in rat small and large intestine. Am J Physiol Gastrointest Liver Physiol 2002; 282(4): G720-G726.

67. Zollner G, Fickert P, Fuchsbichler A, Silbert D, Wagner M, Arbeiter S, Gonzalez FJ, et al. Role of nuclear bile acid receptor, FXR, in adaptive ABC transporter regulation by cholic and ursodeoxycholic acid in mouse liver, kidney and intestine. J Hepatol 2003; 39(4): 480-8.

68. Wilson FA, Burckhardt G, Murer H, Rumrich G, Ullrich KJ. Sodium-coupled taurocholate transport in the proximal convolution of the rat kidney in vivo and in vitro. J Clin Invest 1981; 67(4): 1141-50.

69. Raedsch R, Lauterburg BH, Hofmann AF. Altered bile acid metabolism in primary biliary cirrhosis. Dig Dis Sci 1981; 26(5): 394-401.

70. Bergwerk AJ, Shi X, Ford AC, Kanai N, Jacquemin E, Burk RD, Bai S, et al. Immunologic distribution of an organic anion transport protein in rat liver and kidney. Am J Physiol 1996; 271(2 Pt 1): G231-G238.

71. Lee J, Azzaroli F, Wang L, Soroka CJ, Gigliozzi A, Setchell KD, Kramer W, et al. Adaptive regulation of bile salt transporters in kidney and liver in obstructive cholestasis in the rat. Gastroenterology 2001; 121(6): 1473-84.

72. Wagner M, Fickert P, Marschall HU, Zollner G, Silbert D, Fuchsbichler A, Gumhold J, et al. Alternative Exretory Routes for Accumulating Bile Acids via Adaptive Renal ABC Transporter Expression in Common Bile Duct-ligated (CBDL) Mice. Gastroenterology 2004: 126[4, Suppl. 2], A-129.

73. Kullak-Ublick GA, Hagenbuch B, Stieger B, Schteingart CD, Hofmann AF, Wolkoff AW, Meier PJ. Molecular and functional characterization of an organic anion transporting polypeptide cloned from human liver. Gastroenterology 1995; 109(4): 1274-82.

74. Schaub TP, Kartenbeck J, Konig J, Spring H, Dorsam J, Staehler G, Storkel S, et al. Expression of the MRP2 gene-encoded conjugate export pump in human kidney proximal tubules and in renal cell carcinoma. J Am Soc Nephrol 1999; 10(6): 1159-69.

75. van Aubel RA, Smeets PH, Peters JG, Bindels RJ, Russel FG. The MRP4/ABCC4 gene encodes a novel apical organic anion transporter in human kidney proximal tubules: putative efflux pump for urinary cAMP and cGMP. J Am Soc Nephrol 2002; 13(3): 595-603.

76. Soroka C, Chi SY, Boyer J L. Effects of cholestasis on the regulation of membrane transporter expression in intestine and kidney. Hepatology 2002: 36[4, Suppl 2], 1195.

77. Handschin C, Meyer UA. Induction of drug metabolism: the role of nuclear receptors. Pharmacol Rev 2003; 55(4): 649-73.

78. Xu L, Glass CK, Rosenfeld MG. Coactivator and corepressor complexes in nuclear receptor function. Curr Opin Genet Dev 1999; 9(2): 140-7.

79. Parks DJ, Blanchard SG, Bledsoe RK, Chandra G, Consler TG, Kliewer SA, Stimmel JB, et al. Bile acids: natural ligands for an orphan nuclear receptor. Science 1999; 284(5418): 1365-8.

80. Makishima M, Okamoto AY, Repa JJ, Tu H, Learned RM, Luk A, Hull MV, et al. Identification of a nuclear receptor for bile acids. Science 1999; 284(5418): 1362-5.

81. Wang H, Chen J, Hollister K, Sowers LC, Forman BM. Endogenous bile acids are ligands for the nuclear receptor FXR/BAR. Mol Cell 1999; 3(5): 543-53.

82. Xie W, Radominska-Pandya A, Shi Y, Simon CM, Nelson MC, Ong ES, Waxman DJ, et al. An essential role for nuclear receptors SXR/PXR in detoxification of cholestatic bile acids. Proc Natl Acad Sci USA 2001; 98(6): 3375-80.

83. Staudinger JL, Goodwin B, Jones SA, Hawkins-Brown D, MacKenzie KI, LaTour A, Liu Y, et al. The nuclear receptor PXR is a lithocholic acid sensor that protects against liver toxicity. Proc Natl Acad Sci U SA 2001; 98(6): 3369-74.

84. Makishima M, Lu TT, Xie W, Whitfield GK, Domoto H, Evans RM, Haussler MR, et al. Vitamin D receptor as an intestinal bile acid sensor. Science 2002; 296(5571): 1313-6.

85. Song C, Hiipakka RA, Liao S. Selective activation of liver X receptor alpha by 6alpha-hydroxy bile acids and analogs. Steroids 2000; 65(8): 423-7.

86. Huang W, Zhang J, Chua SS, Qatanani M, Han Y, Granata R, Moore DD. Induction of bilirubin clearance by the constitutive androstane receptor (CAR). Proc Natl Acad Sci USA 2003; 100(7): 4156-61.

87. Assem M, Schuetz EG, Leggas M, Sun D, Yasuda K, Reid G, Zelcer N, et al. Interactions between hepatic Mrp4 and Sult2a as revealed by the constitutive androstane receptor and Mrp4 knockout mice. J Biol Chem 2004; 279(21): 22250-7.

88. Mangelsdorf DJ, Evans RM. The RXR heterodimers and orphan receptors. Cell 1995; 83(6): 841-50.

89. Tzameli I, Chua SS, Cheskis B, Moore DD. Complex effects of rexinoids on ligand dependent activation or inhibition of the xenobiotic receptor, CAR. Nucl Recept 2003; 1(1): 2.

90. Davis RA, Miyake JH, Hui TY, Spann NJ. Regulation of cholesterol-7alpha-hydroxylase: BAREly missing a SHP. J Lipid Res 2002; 43(4): 533-43.

91. Jung D, Kullak-Ublick GA. Hepatocyte nuclear factor 1 alpha: a key mediator of the effect of bile acids on gene expression. Hepatology 2003; 37(3): 622-31.

92. Jung D, Hagenbuch B, Fried M, Meier PJ, Kullak-Ublick GA. Role of liver-enriched transcription factors and nuclear receptors in regulating the human, mouse, and rat NTCP gene. Am J Physiol Gastrointest Liver Physiol 2004; 286(5): G752-G761.

93. Lu TT, Repa JJ, Mangelsdorf DJ. Orphan nuclear receptors as eLiXiRs and FiXeRs of sterol metabolism. J Biol Chem 2001; 276(41): 37735-8.

94. Otte K, Kranz H, Kober I, Thompson P, Hoefer M, Haubold B, Remmel B, et al. Identification of farnesoid X receptor beta as a novel mammalian nuclear receptor sensing lanosterol. Mol Cell Biol 2003; 23(3): 864-72.

95. Huber RM, Murphy K, Miao B, Link JR, Cunningham MR, Rupar MJ, Gunyuzlu PL, et al. Generation of multiple farnesoid-X-receptor isoforms through the use of alternative promoters. Gene 2002; 290(1-2): 35-43.

96. Bishop-Bailey D, Walsh DT, Warner TD. Expression and activation of the farnesoid X receptor in the vasculature. Proc Natl Acad Sci USA 2004; 101(10): 3668-73.

97. Yu J, Lo JL, Huang L, Zhao A, Metzger E, Adams A, Meinke PT, et al. Lithocholic acid decreases expression of bile salt export pump through farnesoid X receptor antagonist activity. J Biol Chem 2002; 277(35): 31441-7.

98. Lew JL, Zhao A, Yu J, Huang L, de PN, Pelaez F, Wright SD, et al. The farnesoid X receptor controls gene expression in a ligand- and promoter-selective fashion. J Biol Chem 2004; 279(10): 8856-61.

99. Karpen SJ. Bile acids go nuclear! Hepatology 1999; 30(4): 1107-9.

100. Laffitte BA, Kast HR, Nguyen CM, Zavacki AM, Moore DD, Edwards PA. Identification of the DNA binding specificity and potential target genes for the farnesoid X-activated receptor. J Biol Chem 2000; 275(14): 10638-47.

101. Urizar NL, Dowhan DH, Moore DD. The farnesoid X-activated receptor mediates bile acid activation of phospholipid transfer protein gene expression. J Biol Chem 2000; 275(50): 39313-7.

102. Kast HR, Goodwin B, Tarr PT, Jones SA, Anisfeld AM, Stoltz CM, Tontonoz P, et al. Regulation of multidrug resistance-associated protein 2 (ABCC2) by the nuclear receptors pregnane X receptor, farnesoid X-activated receptor, and constitutive androstane receptor. J Biol Chem 2002; 277(4): 2908-15.

103. Song CS, Echchgadda I, Baek BS, Ahn SC, Oh T, Roy AK, Chatterjee B. Dehydroepiandrosterone sulfotransferase gene induction by bile acid activated farnesoid X receptor. J Biol Chem 2001; 276(45): 42549-56.

104. Barbier O, Torra IP, Sirvent A, Claudel T, Blanquart C, Duran-Sandoval D, Kuipers F, et al. FXR induces the UGT2B4 enzyme in hepatocytes: a potential mechanism of negative feedback control of FXR activity. Gastroenterology 2003; 124(7): 1926-40.

105. Ananthanarayanan M, Balasubramanian N, Makishima M, Mangelsdorf DJ, Suchy FJ. Human bile salt export pump promoter is transactivated by the farnesoid X receptor/bile acid receptor. J Biol Chem 2001; 276(31): 28857-65.

106. Gerloff T, Geier A, Roots I, Meier PJ, Gartung C. Functional analysis of the rat bile salt export pump gene promoter. Eur J Biochem 2002; 269(14): 3495-503.

107. Plass JR, Mol O, Heegsma J, Geuken M, Faber KN, Jansen PL, Muller M. Farnesoid X receptor and bile salts are involved in transcriptional regulation of the gene encoding the human bile salt export pump. Hepatology 2002; 35(3): 589-96.

108. Kitada H, Miyata M, Nakamura T, Tozawa A, Honma W, Shimada M, Nagata K, et al. Protective role of hydroxysteroid sulfotransferase in lithocholic acid-induced liver toxicity. J Biol Chem 2003; 278(20): 17838-44.

109. Jung D, Podvinec M, Meyer UA, Mangelsdorf DJ, Fried M, Meier PJ, Kullak-Ublick GA. Human organic anion transporting polypeptide 8 promoter is transactivated by the farnesoid X receptor/bile acid receptor. Gastroenterology 2002; 122(7): 1954-66.

110. Huang L, Zhao A, Lew JL, Zhang T, Hrywna Y, Thompson JR, de PN, et al. Farnesoid X receptor activates transcription of the phospholipid pump MDR3. J Biol Chem 2003; 278(51): 51085-90.

111. Grober J, Zaghini I, Fujii H, Jones SA, Kliewer SA, Willson TM, Ono T, et al. Identification of a bile acid-responsive element in the human ileal bile acid-binding protein gene. Involvement of the farnesoid X receptor/9-cis-retinoic acid receptor heterodimer. J Biol Chem 1999; 274(42): 29749-54.

112. Sinal CJ, Tohkin M, Miyata M, Ward JM, Lambert G, Gonzalez FJ. Targeted disruption of the nuclear receptor FXR/BAR impairs bile acid and lipid homeostasis. Cell 2000; 102(6): 731-44.

113. Pircher PC, Kitto JL, Petrowski ML, Tangirala RK, Bischoff ED, Schulman IG, Westin SK. Farnesoid X receptor regulates bile acid-amino acid conjugation. J Biol Chem 2003; 278(30): 27703-11.

114. Echchgadda I, Song CS, Roy AK, Chatterjee B. Dehydroepiandrosterone sulfotransferase is a target for transcriptional induction by the vitamin D receptor. Mol Pharmacol 2004; 65(3): 720-9.

115. Denson LA, Bohan A, Bajwa HJ, Held MA, Boyer JL. Alterations in nuclear hormone receptors (NHR) are associated with changes in the expression of multidrug resistance proteins Mrp2 and Mrp3 in liver and kidney in cholestasis. Hepatology 2001: 34[4, Suppl 2], 367.

116. Guo GL, Lambert G, Negishi M, Ward JM, Brewer HB, Jr., Kliewer SA, Gonzalez FJ, et al. Complementary roles of farnesoid X receptor, pregnane X receptor, and constitutive androstane receptor in protection against bile acid toxicity. J Biol Chem 2003; 278(46): 45062-71.

117. Chen F, Ananthanarayanan M, Emre S, Neimark E, Bull LN, Knisely AS, Strautnieks SS, et al. Progressive familial intrahepatic cholestasis, type 1, is associated with decreased farnesoid X receptor activity. Gastroenterology 2004; 126(3): 756-64.

118. Goodwin B, Jones SA, Price RR, Watson MA, McKee DD, Moore LB, Galardi C, et al. A regulatory cascade of the nuclear receptors FXR, SHP-1, and LRH-1 represses bile acid biosynthesis. Mol Cell 2000; 6(3): 517-26.

119. Kanaya E, Shiraki T, Jingami H. The nuclear bile acid receptor FXR is activated by PGC-1alpha in a ligand-dependent manner. Biochem J 2004; 382(Pt 3): 913-21.

120. Lee YK, Dell H, Dowhan DH, Hadzopoulou-Cladaras M, Moore DD. The orphan nuclear receptor SHP inhibits hepatocyte nuclear factor 4 and retinoid X receptor transactivation: two mechanisms for repression. Mol Cell Biol 2000; 20(1): 187-95.

121. Li D, Zimmerman TL, Thevananther S, Lee HY, Kurie JM, Karpen SJ. Interleukin-1 beta-mediated suppression of RXR:RAR transactivation of the Ntcp promoter is JNK-dependent. J Biol Chem 2002; 277(35): 31416-22.

122. Denson LA, Sturm E, Echevarria W, Zimmerman TL, Makishima M, Mangelsdorf DJ, Karpen SJ. The orphan nuclear receptor, shp, mediates bile acid-induced inhibition of the rat bile acid transporter, ntcp. Gastroenterology 2001; 121(1): 140-7.

123. Zollner G, Fickert P, Silbert D, Fuchsbichler A, Stumptner C, Zatloukal K, Denk H, et al. Induction of short heterodimer partner 1 precedes downregulation of Ntcp in bile duct-ligated mice. Am J Physiol Gastrointest Liver Physiol 2002; 282(1): G184-G191.

124. Wang L, Lee YK, Bundman D, Han Y, Thevananther S, Kim CS, Chua SS, et al. Redundant pathways for negative feedback regulation of bile acid production. Dev Cell 2002; 2(6): 721-31.

125. De FE, Mitro N, Gilardi F, Caruso D, Galli G, Crestani M. Coordinated control of cholesterol catabolism to bile acids and of gluconeogenesis via a novel mechanism of transcription regulation linked to the fasted-to-fed cycle. J Biol Chem 2003; 278(40): 39124-32.

126. Gupta S, Stravitz RT, Dent P, Hylemon PB. Down-regulation of cholesterol 7alpha-hydroxylase (CYP7A1) gene expression by bile acids in primary rat hepatocytes is mediated by the c-Jun N-terminal kinase pathway. J Biol Chem 2001; 276(19): 15816-22.

127. Trauner M, Arrese M, Lee H, Boyer JL, Karpen SJ. Endotoxin downregulates rat hepatic ntcp gene expression via decreased activity of critical transcription factors. J Clin Invest 1998; 101(10): 2092-100.

128. Denson LA, Auld KL, Schiek DS, McClure MH, Mangelsdorf DJ, Karpen SJ. Interleukin-1beta suppresses retinoid transactivation of two hepatic transporter genes involved in bile formation. J Biol Chem 2000; 275(12): 8835-43.

129. Wang B, Cai SR, Gao C, Sladek FM, Ponder KP. Lipopolysaccharide results in a marked decrease in hepatocyte nuclear factor 4 alpha in rat liver. Hepatology 2001; 34(5): 979-89.

130. Zhang M, Chiang JY. Transcriptional regulation of the human sterol 12alpha-hydroxylase gene (CYP8B1): roles of heaptocyte nuclear factor 4alpha in mediating bile acid repression. J Biol Chem 2001; 276(45): 41690-9.

131. Ktistaki E, Talianidis I. Modulation of hepatic gene expression by hepatocyte nuclear factor 1. Science 1997; 277(5322): 109-12.

132. Shih DQ, Bussen M, Sehayek E, Ananthanarayanan M, Shneider BL, Suchy FJ, Shefer S, et al. Hepatocyte nuclear factor-1alpha is an essential regulator of bile acid and plasma cholesterol metabolism. Nat Genet 2001; 27(4): 375-82.

133. Hayhurst GP, Lee YH, Lambert G, Ward JM, Gonzalez FJ. Hepatocyte nuclear factor 4alpha (nuclear receptor 2A1) is essential for maintenance of hepatic gene expression and lipid homeostasis. Mol Cell Biol 2001; 21(4): 1393-403.

134. Jung D, Hagenbuch B, Gresh L, Pontoglio M, Meier PJ, Kullak-Ublick GA. Characterization of the human OATP-C (SLC21A6) gene promoter and regulation of liver-specific OATP genes by hepatocyte nuclear factor 1 alpha. J Biol Chem 2001; 276(40): 37206-14.

135. Li N, Klaassen CD. Role of liver-enriched transcription factors in the down-regulation of organic anion transporting polypeptide 4 (oatp4; oatplb2; slc21a10) by lipopolysaccharide. Mol Pharmacol 2004; 66(3): 694-701.

136. Chen F, Ma L, Dawson PA, Sinal CJ, Sehayek E, Gonzalez FJ, Breslow J, et al. Liver receptor homologue-1 mediates species- and cell line-specific bile acid-dependent negative feedback regulation of the apical sodium-dependent bile acid transporter. J Biol Chem 2003; 278(22): 19909-16.

137. Neimark E, Chen F, Li X, Shneider BL. Bile acid-induced negative feedback regulation of the human ileal bile acid transporter. Hepatology 2004; 40(1): 149-56.

138. Schuetz EG, Strom S, Yasuda K, Lecureur V, Assem M, Brimer C, Lamba J, et al. Disrupted bile acid homeostasis reveals an unexpected interaction among nuclear hormone receptors, transporters, and cytochrome P450. J Biol Chem 2001; 276(42): 39411-8.

139. Jones SA, Moore LB, Shenk JL, Wisely GB, Hamilton GA, McKee DD, Tomkinson NC, et al. The pregnane X receptor: a promiscuous xenobiotic receptor that has diverged during evolution. Mol Endocrinol 2000; 14(1): 27-39.

140. Watkins RE, Wisely GB, Moore LB, Collins JL, Lambert MH, Williams SP, Willson TM, et al. The human nuclear xenobiotic receptor PXR: structural determinants of directed promiscuity. Science 2001; 292(5525): 2329-33.

141. Lehmann JM, McKee DD, Watson MA, Willson TM, Moore JT, Kliewer SA. The human orphan nuclear receptor PXR is activated by compounds that regulate CYP3A4 gene expression and cause drug interactions. J Clin Invest 1998; 102(5): 1016-23.

142. Moore LB, Parks DJ, Jones SA, Bledsoe RK, Consler TG, Stimmel JB, Goodwin B, et al. Orphan nuclear receptors constitutive androstane receptor and pregnane X receptor share xenobiotic and steroid ligands. J Biol Chem 2000; 275(20): 15122-7.

143. Landes N, Pfluger P, Kluth D, Birringer M, Ruhl R, Bol GF, Glatt H, et al. Vitamin E activates gene expression via the pregnane X receptor. Biochem Pharmacol 2003; 65(2): 269-73.

144. Moore LB, Goodwin B, Jones SA, Wisely GB, Serabjit-Singh CJ, Willson TM, Collins JL, et al. St. John’s wort induces hepatic drug metabolism through activation of the pregnane X receptor. Proc Natl Acad Sci USA 2000; 97(13): 7500-2.

145. Zhang H, LeCulyse E, Liu L, Hu M, Matoney L, Zhu W, Yan B. Rat pregnane X receptor: molecular cloning, tissue distribution, and xenobiotic regulation. Arch Biochem Biophys 1999; 368(1): 14-22.

146. Geick A, Eichelbaum M, Burk O. Nuclear receptor response elements mediate induction of intestinal MDR1 by rifampin. J Biol Chem 2001; 276(18): 14581-7.

147. Synold TW, Dussault I, Forman BM. The orphan nuclear receptor SXR coordinately regulates drug metabolism and efflux. Nat Med 2001; 7(5): 584-90.

148. Rosenfeld JM, Vargas R, Jr., Xie W, Evans RM. Genetic profiling defines the xenobiotic gene network controlled by the nuclear receptor pregnane X receptor. Mol Endocrinol 2003; 17(7): 1268-82.

149. Teng S, Jekerle V, Piquette-Miller M. Induction of ABCC3 (MRP3) by pregnane X receptor activators. Drug Metab Dispos 2003; 31(11): 1296-9.

150. Wagner M, Halilbasic E, Zollner G, Fickert P, Gumhold J, Silbert D, Fuchsbichler A, et al. Xenobiotic nuclear receptor (PXR, CAR) agonists coordinately stimulate a spectrum of detoxification enzymes and transport systems involved in the adaptive response to cholestasis in vivo. Hepatology 2004: 40, Suppl. 1.

151. Li T, Chiang JY. Mechanism of rifampicin and pregnane X receptor inhibition of human cholesterol 7 alpha-hydroxylase gene transcription. Am J Physiol Gastrointest Liver Physiol 2005; 288(1): G74-G84.

152. Bhalla S, Ozalp C, Fang S, Xiang L, Kemper JK. Ligand-activated pregnane X receptor interferes with HNF-4 signaling by targeting a common coactivator PGC-1alpha. Functional implications in hepatic cholesterol and glucose metabolism. J Biol Chem 2004; 279(43): 45139-47.

153. Sonoda J, Xie W, Rosenfeld JM, Barwick JL, Guzelian PS, Evans RM. Regulation of a xenobiotic sulfonation cascade by nuclear pregnane X receptor (PXR). Proc Natl Acad Sci USA 2002; 99(21): 13801-6.

154. Chen C, Staudinger JL, Klaassen CD. Nuclear receptor, pregname X receptor, is required for induction of UDP-glucuronosyltranferases in mouse liver by pregnenolone-16 alpha-carbonitrile. Drug Metab Dispos 2003; 31(7): 908-15.

155. Maglich JM, Stoltz CM, Goodwin B, Hawkins-Brown D, Moore JT, Kliewer SA. Nuclear pregnane x receptor and constitutive androstane receptor regulate overlapping but distinct sets of genes involved in xenobiotic detoxification. Mol Pharmacol 2002; 62(3): 638-46.

156. Frank C, Makkonen H, Dunlop TW, Matilainen M, Vaisanen S, Carlberg C. Identification of pregnane X receptor binding sites in the regulatory regions of genes involved in bile acid homeostasis. J Mol Biol 2005; 346(2): 505-19.

157. Ourlin JC, Lasserre F, Pineau T, Fabre JM, Sa-Cunha A, Maurel P, Vilarem MJ, et al. The small heterodimer partner interacts with the pregnane X receptor and represses its transcriptional activity. Mol Endocrinol 2003; 17(9): 1693-703.

158. Goodwin B, Moore JT. CAR: detailing new models. Trends Pharmacol Sci 2004; 25(8): 437-41.

159. Frank C, Gonzalez MM, Oinonen C, Dunlop TW, Carlberg C. Characterization of DNA complexes formed by the nuclear receptor constitutive androstane receptor. J Biol Chem 2003; 278(44): 43299-310.

160. Baes M, Gulick T, Choi HS, Martinoli MG, Simha D, Moore DD. A new orphan member of the nuclear hormone receptor superfamily that interacts with a subset of retinoic acid response elements. Mol Cell Biol 1994; 14(3): 1544-52.

161. Choi HS, Chung M, Tzameli I, Simha D, Lee YK, Seol W, Moore DD. Differential transactivation by two isoforms of the orphan nuclear hormone receptor CAR. J Biol Chem 1997; 272(38): 23565-71.

162. Xie W, Barwick JL, Simon CM, Pierce AM, Safe S, Blumberg B, Guzelian PS, et al. Reciprocal activation of xenobiotic response genes by nuclear receptors SXR/PXR and CAR. Genes Dev 2000; 14(23): 3014-23.

163. Zhang J, Huang W, Qatanani M, Evans RM, Moore DD. The constitutive androstane receptor and pregnane X receptor function coordinately to prevent bile acid-induced hepatotoxicity. J Biol Chem 2004; in press.

164. Saini SP, Sonoda J, Xu L, Toma D, Uppal H, Mu Y, Ren S, et al. A novel constitutive androstane receptor-mediated and CYP3A-independent pathway of bile acid detoxification. Mol Pharmacol 2004; 65(2): 292-300.

165. Huang W, Zhang J, Moore DD. A traditional herbal medicine enhances bilirubin clearance by activating the nuclear receptor CAR. J Clin Invest 2004; 113(1): 137-43.

166. Bae Y, Kemper JK, Kemper B. Repression of CAR-mediated transactivation of CYP2B genes by the orphan nuclear receptor, short heterodimer partner (SHP). DNA Cell Biol 2004; 23(2): 81-91.

167. Gascon-Barre M, Demers C, Mirshahi A, Neron S, Zalzal S, Nanci A. The normal liver harbors the vitamin D nuclear receptor in nonparenchymal and biliary epithelial cells. Hepatology 2003; 37(5): 1034-42.

168. Chen X, Pang SK, Chen F, Shneider BL. 1 alpha,25-dihydroxyvitamin D-3 up-regulates the rat apical sodium-dependent bile acid transporter (ASBT) via a specific vitamin D response element (VDRE) in the promoter region of ASBT gene. Hepatology 2004: 40[4, Suppl 2], 519A.

169. Honjo Y, Kobayashi Y, Sasaki S, Misawa H, Nakamura H. Inhibition of farnesoid X receptor-induced BSEP gene expression by ligand-activated vitamin D receptor. Hepatology 2004: 40[4, Suppl. 1].

170. Chen WS, Denk GU, Denson LA, Wang L, Soroka C, Boyer JL. Release of transcriptional repression from RXRa and RARa results in up-regulation of Mrp3 (the multidrug resistance-associated protein 3) in obstructive cholestasis. Hepatology 2004: 40[4; Suppl 1].

171. Inokuchi A, Hinoshita E, Iwamoto Y, Kohno K, Kuwano M, Uchiumi T. Enhanced expression of the human multidrug resistance protein 3 by bile salt in human enterocytes. A transcriptional control of a plausible bile acid transporter. J Biol Chem 2001; 276(50): 46822-9.

172. del Castillo-Olivares A, Gil G. Alpha 1-fetoprotein transcription factor is required for the expression of sterol 12alpha -hydroxylase, the specific enzyme for cholic acid synthesis. Potential role in the bile acid-mediated regulation of gene transcription. J Biol Chem 2000; 275(23): 17793-9.

173. Bohan A, Chen WS, Denson LA, Held MA, Boyer JL. Tumor necrosis factor alpha-dependent up-regulation of Lrh-1 and Mrp3(Abcc3) reduces liver injury in obstructive cholestasis. J Biol Chem 2003; 278(38): 36688-98.

174. Yang Y, Zhang M, Eggertsen G, Chiang JY. On the mechanism of bile acid inhibition of rat sterol 12alpha-hydroxylase gene (CYP8B1) transcription: roles of alpha-fetoprotein transcription factor and hepatocyte nuclear factor 4alpha. Biochim Biophys Acta 2002; 1583(1): 63-73.

175. Chianale J, Vollrath V, Wielandt AM, Amigo L, Rigotti A, Nervi F, Gonzalez S, et al. Fibrates induce mdr2 gene expression and biliary phospholipid secretion in the mouse. Biochem J 1996; 314 (Pt 3): 781-6.

176. Jung D, Fried M, Kullak-Ublick GA. Human apical sodium-dependent bile salt transporter gene (SLC10A2) is regulated by the peroxisome proliferator-activated receptor alpha. J Biol Chem 2002; 277(34): 30559-66.

177. Hunt MC, Yang YZ, Eggertsen G, Carneheim CM, Gafvels M, Einarsson C, Alexson SE. The peroxisome proliferator-activated receptor alpha (PPARalpha) regulates bile acid biosynthesis. J Biol Chem 2000; 275(37): 28947-53.

178. Patel DD, Knight BL, Soutar AK, Gibbons GF, Wade DP. The effect of peroxisome-proliferator-activated receptor-alpha on the activity of the cholesterol 7 alpha-hydroxylase gene. Biochem J 2000; 351 Pt 3: 747-53.

179. Barbier O, Villeneuve L, Bocher V, Fontaine C, Torra IP, Duhem C, Kosykh V, et al. The UDP-glucuronosyltransferase 1A9 enzyme is a peroxisome proliferator-activated receptor alpha and gamma target gene. J Biol Chem 2003; 278(16): 13975-83.

180. Fang HL, Strom SC, Cai H, Falany CN, Kocarek TA, Runge-Morris M. Regulation of Human Hepatic Hydroxysteroid Sulfotransferase Gene Expression by the Peroxisome Proliferator Activated Receptor Alpha Transcription Factor. Mol Pharmacol 2005.

181. Eloranta JJ, Jung D, Kullak-Ublick G. The human Na plus taurocholate cotransporting polypeptide gene (NTCP, SLC10A1) is activated by the glucocorticoid receptor and its coactivator PGC-1 and suppressed by the small heterodimer partner SHP. Hepatology 2004: 40[4, Suppl 1], 519A.

182. Alvaro D, Gigliozzi A, Marucci L, Alpini G, Barbaro B, Monterubbianesi R, Minetola L, et al. Corticosteroids modulate the secretory processes of the rat intrahepatic biliary epithelium. Gastroenterology 2002; 122(4): 1058-69.

183. Pascussi JM, Busson-Le CM, Maurel P, Vilarem MJ. Transcriptional analysis of the orphan nuclear receptor constitutive androstane receptor (NR1I3) gene promoter: identification of a distal glucocorticoid response element. Mol Endocrinol 2003; 17(1): 42-55.

184. Miura T, Ouchida R, Yoshikawa N, Okamoto K, Makino Y, Nakamura T, Morimoto C, et al. Functional modulation of the glucocorticoid receptor and suppression of NF-kappaB-dependent transcription by ursodeoxycholic acid. J Biol Chem 2001; 276(50): 47371-8.

185. Trauner M, Meier PJ, Boyer JL. Molecular regulation of hepatocellular transport systems in cholestasis. J Hepatol 1999; 31(1): 165-78.

186. Trauner M, Boyer JL. Cholestatic syndromes. Curr Opin Gastroenterol 2004; 20(3): 220-30.

187. Bull LN, Carlton VE, Stricker NL, Baharloo S, DeYoung JA, Freimer NB, Magid MS, et al. Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): evidence for heterogeneity. Hepatology 1997; 26(1): 155-64.

188. Clayton RJ, Iber FL, Ruebner BH, McKusick VA. Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969; 117(1): 112-24.

189. Eppens EF, van Mil SW, de Vree JM, Mok KS, Juijn JA, Oude Elferink RP, Berger R, et al. FIC1, the protein affected in two forms of hereditary cholestasis, is localized in the cholangiocyte and the canalicular membrane of the hepatocyte. J Hepatol 2001; 35(4): 436-43.

190. Ujhazy P, Ortiz D, Misra S, Li S, Moseley J, Jones H, Arias IM. Familial intrahepatic cholestasis 1: studies of localization and function. Hepatology 2001; 34(4 Pt 1): 768-75.

191. Bull LN, van Eijk MJ, Pawlikowska L, DeYoung JA, Juijn JA, Liao M, Klomp LW, et al. A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis. Nat Genet 1998; 18(3): 219-24.

192. Stieger B. FIC1: another bile salt carrier within the enterohepatic circulation? J Hepatol 2001; 35(4): 522-4.

193. Harris MJ, Kagawa T, Dawson PA, Arias IM. The effect of FICI expression on taurocholate transport by BSEP and IBAT in transfected MDCKII cells. Hepatology 2002: 36[4; Suppl 2], 309A.

194. Pawlikowska L, Groen A, Eppens EF, Kunne C, Ottenhoff R, Looije N, Knisely AS, et al. A mouse genetic model for familial cholestasis caused by ATP8B1 mutations reveals perturbed bile salt homeostasis but no impairment in bile secretion. Hum Mol Genet 2004; 13(8): 881-92.

195. Kok T, Hulzebos CV, Wolters H, Havinga R, Agellon LB, Stellaard F, Shan B, et al. Enterohepatic circulation of bile salts in farnesoid X receptor-deficient mice: efficient intestinal bile salt absorption in the absence of ileal bile acid-binding protein. J Biol Chem 2003; 278(43): 41930-7.

196. van Mil SW, van der Woerd WL, van der BG, Sturm E, Jansen PL, Bull LN, van dB I, et al. Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11. Gastroenterology 2004; 127(2): 379-84.

197. Strautnieks SS, Bull LN, Knisely AS, Kocoshis SA, Dahl N, Arnell H, Sokal E, et al. A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis. Nat Genet 1998; 20(3): 233-8.

198. Setchell KD, Rodrigues CM, Clerici C, Solinas A, Morelli A, Gartung C, Boyer J. Bile acid concentrations in human and rat liver tissue and in hepatocyte nuclei. Gastroenterology 1997; 112(1): 226-35.

199. de Vree JM, Jacquemin E, Sturm E, Cresteil D, Bosma PJ, Aten J, Deleuze JF, et al. Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis. Proc Natl Acad Sci USA 1998; 95(1): 282-7.

200. Jansen PL, Muller M. The molecular genetics of familial intrahepatic cholestasis. Gut 2000; 47(1): 1-5.

201. Jacquemin E, de Vree JM, Cresteil D, Sokal EM, Sturm E, Dumont M, Scheffer GL, et al. The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood. Gastroenterology 2001; 120(6): 1448-58.

202. Rosmorduc O, Hermelin B, Poupon R. MDR3 gene defect in adults with symptomatic intrahepatic and gallbladder cholesterol cholelithiasis. Gastroenterology 2001; 120(6): 1459-67.

203. Arrese M, Accatino L. Is intrahepatic cholestasis of pregnancy an MDR3-related disease? Gastroenterology 2003; 125(6): 1922-3.

204. Fickert P, Zollner G, Fuchsbichler A, Stumptner C, Weiglein AH, Lammert F, Marschall HU, et al. Ursodeoxycholic acid aggravates bile infarcts in bile duct-ligated and Mdr2 knockout mice via disruption of cholangioles. Gastroenterology 2002; 123(4): 1238-51.

205. Lammert F, Wang DQ, Hillebrandt S, Geier A, Fickert P, Trauner M, Matern S, et al. Spontaneous cholecysto- and hepatolithiasis in Mdr2-/- mice: a model for low phospholipid-associated cholelithiasis. Hepatology 2004; 39(1): 117-28.

206. Dumoulin FL, Reichel C, Sauerbruch T, Spengler U. Semiquantitation of intrahepatic MDR3 mRNA levels by reverse transcription/competitive polymerase chain reaction. J Hepatol 1997; 26(4): 852-6.

207. Zollner G, Fickert P, Silbert D, Fuchsbichler A, Marschall HU, Zatloukal K, Denk H, et al. Adaptive changes in hepatobiliary transporter expression in primary biliary cirrhosis. J Hepatol 2003; 38(6): 717-27.

208. Pauli-Magnus C, Kerb R, Fattinger K, Lang T, Anwald B, Kullak-Ublick GA, Beuers U, et al. BSEP and MDR3 haplotype structure in healthy Caucasians, primary biliary cirrhosis and primary sclerosing cholangitis. Hepatology 2004; 39(3): 779-91.

209. Paulusma CC, Kool M, Bosma PJ, Scheffer GL, ter Borg F, Scheper RJ, Tytgat GN, et al. A mutation in the human canalicular multispecific organic anion transporter gene causes the Dubin-Johnson syndrome. Hepatology 1997; 25(6): 1539-42.

210. Kartenbeck J, Leuschner U, Mayer R, Keppler D. Absence of the canalicular isoform of the MRP gene-encoded conjugate export pump from the hepatocytes in Dubin-Johnson syndrome. Hepatology 1996; 23(5): 1061-6.

211. Wada M, Toh S, Taniguchi K, Nakamura T, Uchiumi T, Kohno K, Yoshida I, et al. Mutations in the canilicular multispecific organic anion transporter (cMOAT) gene, a novel ABC transporter, in patients with hyperbilirubinemia II/Dubin-Johnson syndrome. Hum Mol Genet 1998; 7(2): 203-7.

212. Konig J, Rost D, Cui Y, Keppler D. Characterization of the human multidrug resistance protein isoform MRP3 localized to the basolateral hepatocyte membrane. Hepatology 1999; 29(4): 1156-63.

213. Jansen PL, Peters WH, Lamers WH. Hereditary chronic conjugated hyperbilirubinemia in mutant rats caused by defective hepatic anion transport. Hepatology 1985; 5(4): 573-9.

214. Kuipers F, Enserink M, Havinga R, van der Steen AB, Hardonk MJ, Fevery J, Vonk RJ. Separate transport systems for biliary secretion of sulfated and unsulfated bile acids in the rat. J Clin Invest 1988; 81(5): 1593-9.

215. Ito K, Suzuki H, Hirohashi T, Kume K, Shimizu T, Sugiyama Y. Molecular cloning of canalicular multispecific organic anion transporter defective in EHBR. Am J Physiol 1997; 272(1 Pt 1): G16-G22.

216. Tirona RG, Kim RB. Pharmacogenomics of organic anion-transporting polypeptides (OATP). Adv Drug Deliv Rev 2002; 54(10): 1343-52.

217. Zelcer N, van de Wetering K, de Waart DR, Kunne C, Elferink RO, Borst P. MRP3 knockout mice have normal bile salt homeostasis but altered bilirubin disposition. Hepatology 2004: 40[4, Suppl 1], 489A.

218. Levy C, Lindor KD. Drug-induced cholestasis. Clin Liver Dis 2003; 7(2): 311-30.

219. Riely CA, Bacq Y. Intrahepatic cholestasis of pregnancy. Clin Liver Dis 2004; 8(1): 167-76.

220. Li MK, Crawford JM. The pathology of cholestasis. Semin Liver Dis 2004; 24(1): 21-42.

221. Glantz A, Marschall HU, Mattsson LA. Intrahepatic cholestasis of pregnancy: Relationships between bile acid levels and fetal complication rates. Hepatology 2004; 40(2): 467-74.

222. Roman ID, Fernandez-Moreno MD, Fueyo JA, Roma MG, Coleman R. Cyclosporin A induced internalization of the bile salt export pump in isolated rat hepatocyte couplets. Toxicol Sci 2003; 71(2): 276-81.

223. Crocenzi FA, Mottino AD, Cao J, Veggi LM, Pozzi EJ, Vore M, Coleman R, et al. Estradiol-17beta-D-glucuronide induces endocytic internalization of Bsep in rats. Am J Physiol Gastrointest Liver Physiol 2003; 285(2): G449-G459.

224. Mottino AD, Cao J, Veggi LM, Crocenzi F, Roma MG, Vore M. Altered localization and activity of canalicular Mrp2 in estradiol-17beta-D-glucuronide-induced cholestasis. Hepatology 2002; 35(6): 1409-19.

225. Fattinger K, Funk C, Pantze M, Weber C, Reichen J, Stieger B, Meier PJ. The endothelin antagonist bosentan inhibits the canalicular bile salt export pump: a potential mechanism for hepatic adverse reactions. Clin Pharmacol Ther 2001; 69(4): 223-31.

226. Fouassier L, Kinnman N, Lefevre G, Lasnier E, Rey C, Poupon R, Elferink RP, et al. Contribution of mrp2 in alterations of canalicular bile formation by the endothelin antagonist bosentan. J Hepatol 2002; 37(2): 184-91.

227. Pauli-Magnus C, Lang T, Meier Y, Zodan-Marin T, Jung D, Breymann C, Zimmermann R, et al. Sequence analysis of bile salt export pump (ABCB11) and multidrug resistance p-glycoprotein 3 (ABCB4, MDR3) in patients with intrahepatic cholestasis of pregnancy. Pharmacogenetics 2004; 14(2): 91-102.

228. Vore M. Estrogen cholestasis. Membranes, metabolites, or receptors? Gastroenterology 1987; 93(3): 643-9.

229. Reyes H, Simon FR. Intrahepatic cholestasis of pregnancy: an estrogen-related disease. Semin Liver Dis 1993; 13(3): 289-301.

230. Lee JM, Trauner M, Soroka CJ, Stieger B, Meier PJ, Boyer JL. Expression of the bile salt export pump is maintained after chronic cholestasis in the rat. Gastroenterology 2000; 118(1): 163-72.

231. Bossard R, Stieger B, O’Neill B, Fricker G, Meier PJ. Ethinylestradiol treatment induces multiple canalicular membrane transport alterations in rat liver. J Clin Invest 1993; 91(6): 2714-20.

232. Simon FR, Fortune J, Iwahashi M, Gartung C, Wolkoff A, Sutherland E. Ethinyl estradiol cholestasis involves alterations in expression of liver sinusoidal transporters. Am J Physiol 1996; 271(6 Pt 1): G1043-G1052.

233. Lai K, Harnish DC, Evans MJ. Estrogen receptor alpha regulates expression of the orphan receptor small heterodimer partner. J Biol Chem 2003; 278(38): 36418-29.

234. Arrese M, Trauner M, Ananthanarayanan M, Pizarro M, Solis N, Accatino L, Soroka C, et al. Down-regulation of the Na+/taurocholate cotransporting polypeptide during pregnancy in the rat. J Hepatol 2003; 38(2): 148-55.

235. Wagner M, Fickert P, Zollner G, Silbert D, Gumhold J, Fuchsbichler A, Zatloukal K, et al. Hepatic and renal ABC transporter expression in mice depends on gender and changes in pregnancy. Gastroenterology 2004: 26[4, Suppl 2], A130.

236. Cao J, Huang L, Liu Y, Hoffman T, Stieger B, Meier PJ, Vore M. Differential regulation of hepatic bile salt and organic anion transporters in pregnant and postpartum rats and the role of prolactin. Hepatology 2001; 33(1): 140-7.

237. Cao J, Stieger B, Meier PJ, Vore M. Expression of rat hepatic multidrug resistance-associated proteins and organic anion transporters in pregnancy. Am J Physiol Gastrointest Liver Physiol 2002; 283(3): G757-G766.

238. Masuyama H, Hiramatsu Y, Mizutani Y, Inoshita H, Kudo T. The expression of pregnane X receptor and its target gene, cytochrome P450 3A1, in perinatal mouse. Mol Cell Endocrinol 2001; 172(1-2): 47-56.

239. Lamba V, Lamba J, Yasuda K, Strom S, Davila J, Hancock ML, Fackenthal JD, et al. Hepatic CYP2B6 expression: gender and ethnic differences and relationship to CYP2B6 genotype and CAR (constitutive androstane receptor) expression. J Pharmacol Exp Ther 2003; 307(3): 906-22.

240. Wang R, Lam P, Liu L, Forrest D, Yousef IM, Mignault D, Phillips MJ, et al. Severe cholestasis induced by cholic acid feeding in knockout mice of sister of P-glycoprotein. Hepatology 2003; 38(6): 1489-99.

241. Trauner M, Fickert P, Stauber RE. Inflammation-induced cholestasis. J Gastroenterol Hepatol 1999; 14(10): 946-59.

242. Zollner G, Fickert P, Zenz R, Fuchsbichler A, Stumptner C, Kenner L, Ferenci P, et al. Hepatobiliary transporter expression in percutaneous liver biopsies of patients with cholestatic liver diseases. Hepatology 2001; 33(3): 633-46.

243. Bolder U, Ton-Nu HT, Schteingart CD, Frick E, Hofmann AF. Hepatocyte transport of bile acids and organic anions in endotoxemic rats: impaired uptake and secretion. Gastroenterology 1997; 112(1): 214-25.

244. Roelofsen H, Schoemaker B, Bakker C, Ottenhoff R, Jansen PL, Elferink RP. Impaired hepatocanalicular organic anion transport in endotoxemic rats. Am J Physiol 1995; 269(3 Pt 1): G427-G434.

245. Whiting JF, Green RM, Rosenbluth AB, Gollan JL. Tumor necrosis factor-alpha decreases hepatocyte bile salt uptake and mediates endotoxin-induced cholestasis. Hepatology 1995; 22(4 Pt 1): 1273-8.

246. Moseley RH, Wang W, Takeda H, Lown K, Shick L, Ananthanarayanan M, Suchy FJ. Effect of endotoxin on bile acid transport in rat liver: a potential model for sepsis-associated cholestasis. Am J Physiol 1996; 271(1 Pt 1): G137-G146.

247. Geier A, Dietrich CG, Voigt S, Kim SK, Gerloff T, Kullak-Ublick GA, Lorenzen J, et al. Effects of proinflammatory cytokines on rat organic anion transporters during toxic liver injury and cholestasis. Hepatology 2003; 38(2): 345-54.

248. Green RM, Beier D, Gollan JL. Regulation of hepatocyte bile salt transporters by endotoxin and inflammatory cytokines in rodents. Gastroenterology 1996; 111(1): 193-8.

249. Karpen SJ, Sun AQ, Kudish B, Hagenbuch B, Meier PJ, Ananthanarayanan M, Suchy FJ. Multiple factors regulate the rat liver basolateral sodium-dependent bile acid cotransporter gene promoter. J Biol Chem 1996; 271(25): 15211-21.

250. Beigneux AP, Moser AH, Shigenaga JK, Grunfeld C, Feingold KR. The acute phase response is associated with retinoid X receptor repression in rodent liver. J Biol Chem 2000; 275(21): 16390-9.

251. Sturm E, Havinga R, Baller JF, Wolters H, van RN, Kamps JA, Verkade HJ, et al. Kupffer cell depletion with liposomal clodronate prevents suppression of Ntcp expression in endotoxin-treated rats. J Hepatol 2005; 42(1): 102-9.

252. Geier A, Zollner G, Dietrich CG, Wagner M, Fickert P, Denk H, van RN, et al. Cytokine-independent repression of rodent Ntcp in obstructive cholestasis. Hepatology 2005; 41(3): 470-7.

253. Memon RA, Moser AH, Shigenaga JK, Grunfeld C, Feingold KR. In vivo and in vitro regulation of sterol 27-hydroxylase in the liver during the acute phase response. potential role of hepatocyte nuclear factor-1. J Biol Chem 2001; 276(32): 30118-26.

254. Ros JE, Schuetz JD, Geuken M, Streetz K, Moshage H, Kuipers F, Manns MP, et al. Induction of Mdr1b expression by tumor necrosis factor-alpha in rat liver cells is independent of p53 but requires NF-kappaB signaling. Hepatology 2001; 33(6): 1425-31.

255. Assenat E, Gerbal-Chaloin S, Larrey D, Saric J, Fabre JM, Maurel P, Vilarem MJ, et al. Interleukin 1beta inhibits CAR-induced expression of hepatic genes involved in drug and bilirubin clearance. Hepatology 2004; 40(4): 951-60.

256. Trauner M, Arrese M, Soroka CJ, Ananthanarayanan M, Koeppel TA, Schlosser SF, Suchy FJ, et al. The rat canalicular conjugate export pump (Mrp2) is down-regulated in intrahepatic and obstructive cholestasis. Gastroenterology 1997; 113(1): 255-64.

257. Kubitz R, Wettstein M, Warskulat U, Haussinger D. Regulation of the multidrug resistance protein 2 in the rat liver by lipopolysaccharide and dexamethasone. Gastroenterology 1999; 116(2): 401-10.

258. Kim MS, Shigenaga J, Moser A, Feingold K, Grunfeld C. Repression of farnesoid X receptor during the acute phase response. J Biol Chem 2003; 278(11): 8988-95.

259. Beigneux AP, Moser AH, Shigenaga JK, Grunfeld C, Feingold KR. Reduction in cytochrome P-450 enzyme expression is associated with repression of CAR (constitutive androstane receptor) and PXR (pregnane X receptor) in mouse liver during the acute phase response. Biochem Biophys Res Commun 2002; 293(1): 145-9.

260. Fang C, Yoon S, Tindberg N, Jarvelainen HA, Lindros KO, Ingelman-Sundberg M. Hepatic expression of multiple acute phase proteins and down-regulation of nuclear receptors after acute endotoxin exposure. Biochem Pharmacol 2004; 67(7): 1389-97.

261. Teng S, Piquette-Miller M. The involvement of the pregnane X receptor in hepatic gene regulation during inflammation in mice. J Pharmacol Exp Ther 2005; 312(2): 841-8.

262. Donner MG, Warskulat U, Saha N, Haussinger D. Enhanced expression of basolateral multidrug resistance protein isoforms Mrp3 and Mrp5 in rat liver by LPS. Biol Chem 2004; 385(3-4): 331-9.

263. Cherrington NJ, Slitt AL, Li N, Klaassen CD. Lipopolysaccharide-mediated regulation of hepatic transporter mRNA levels in rats. Drug Metab Dispos 2004; 32(7): 734-41.

264. Hartmann G, Cheung AK, Piquette-Miller M. Inflammatory cytokines, but not bile acids, regulate expression of murine hepatic anion transporters in endotoxemia. J Pharmacol Exp Ther 2002; 303(1): 273-81.

265. Shneider BL, Fox VL, Schwarz KB, Watson CL, Ananthanarayanan M, Thevananther S, Christie DM, et al. Hepatic basolateral sodium-dependent-bile acid transporter expression in two unusual cases of hypercholanemia and in extrahepatic biliary atresia. Hepatology 1997; 25(5): 1176-83.

266. Kogan D, Ananthanarayanan M, Emre S. The bile salt export pump (BSEP/SPGP) is not down-regulated in human cholestasis associated with extrahepatic biliary atresia. Hepatology 1999; 30, 468A.

267. Shoda J, Kano M, Oda K, Kamiya J, Nimura Y, Suzuki H, Sugiyama Y, et al. The expression levels of plasma membrane transporters in the cholestatic liver of patients undergoing biliary drainage and their association with the impairment of biliary secretory function. Am J Gastroenterol 2001; 96(12): 3368-78.

268. Oswald M, Kullak-Ublick GA, Paumgartner G, Beuers U. Expression of hepatic transporters OATP-C and MRP2 in primary sclerosing cholangitis. Liver 2001; 21(4): 247-53.

269. Gartung C, Ananthanarayanan M, Rahman MA, Schuele S, Nundy S, Soroka CJ, Stolz A, et al. Down-regulation of expression and function of the rat liver Na+/bile acid cotransporter in extrahepatic cholestasis. Gastroenterology 1996; 110(1): 199-209.

270. Dumont M, Jacquemin E, D’Hont C, Descout C, Cresteil D, Haouzi D, Desrochers M, et al. Expression of the liver Na+ -independent organic anion transporting polypeptide (oatp-1) in rats with bile duct ligation. J Hepatol 1997; 27(6): 1051-6.

271. Wagner M, Fickert P, Zollner G, Fuchsbichler A, Silbert D, Tsybrovskyy O, Zatloukal K, et al. Role of farnesoid X receptor in determining hepatic ABC transporter expression and liver injury in bile duct-ligated mice. Gastroenterology 2003; 125(3): 825-38.

272. Donner MG, Keppler D. Up-regulation of basolateral multidrug resistance protein 3 (Mrp3) in cholestatic rat liver. Hepatology 2001; 34(2): 351-9.

273. Denk GU, Soroka CJ, Takeyama Y, Chen WS, Schuetz JD, Boyer JL. Multidrug resistance-associated protein 4 is up-regulated in liver but down-regulated in kidney in obstructive cholestasis in the rat. J Hepatol 2004; 40(4): 585-91.

274. Sewnath ME, Van Der PT, Ten Kate FJ, Van Noorden CJ, Gouma DJ. Interleukin-1 receptor type I gene-deficient bile duct-ligated mice are partially protected against endotoxin. Hepatology 2002; 35(1): 149-58.

275. Plebani M, Panozzo MP, Basso D, De PM, Biasin R, Infantolino D. Cytokines and the progression of liver damage in experimental bile duct ligation. Clin Exp Pharmacol Physiol 1999; 26(4): 358-63.

276. Liu TZ, Lee KT, Chern CL, Cheng JT, Stern A, Tsai LY. Free radical-triggered hepatic injury of experimental obstructive jaundice of rats involves overproduction of proinflammatory cytokines and enhanced activation of nuclear factor kappaB. Ann Clin Lab Sci 2001; 31(4): 383-90.

277. Denson LA, Bohan A, Held MA, Boyer JL. Organ-specific alterations in RAR alpha:RXR alpha abundance regulate rat Mrp2 (Abcc2) expression in obstructive cholestasis. Gastroenterology 2002; 123(2): 599-607.

278. Fickert P, Fuchsbichler A, Wagner M, Zollner G, Kaser A, Tilg H, Krause R, et al. Regurgitation of bile acids from leaky bile ducts causes sclerosing cholangitis in Mdr2 (Abcb4) knockout mice. Gastroenterology 2004; 127(1): 261-74.

279. Wagner M, Fickert P, Zollner G, Fuchsbichler A, Gumhold J, Silbert D, Zatloukal K et al. Mdr2-/- Mice with chronic cholestasis due to sclerosing cholangitis lack adaptive regulation of ABC transporters and bile acid synthetic enzymes. Gastroenterology 2004: [Suppl1].

280. Chisholm JW, Dolphin PJ. Abnormal lipoproteins in the ANIT-treated rat: a transient and reversible animal model of intrahepatic cholestasis. J Lipid Res 1996; 37(5): 1086-98.

281. Desmet VJ, Krstulovic B, Van DB. Histochemical study of rat liver in alpha-naphthyl isothiocyanate (ANIT) induced cholestasis. Am J Pathol 1968; 52(2): 401-21.

282. Liu Y, Binz J, Numerick MJ, Dennis S, Luo G, Desai B, MacKenzie KI, et al. Hepatoprotection by the farnesoid X receptor agonist GW4064 in rat models of intra- and extrahepatic cholestasis. J Clin Invest 2003; 112(11): 1678-87.

283. Paumgartner G, Beuers U. Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited. Hepatology 2002; 36(3): 525-31.

284. Paumgartner G, Beuers U. Mechanisms of action and therapeutic efficacy of ursodeoxycholic acid in cholestatic liver disease. Clin Liver Dis 2004; 8(1): 67-81, vi.

285. Gerk PM, Vore M. Tauroursodeoxycholate and taurocholate are transported by human MRP2 in the presence of certain MRP2 substrates. Hepatology 2004: 40, Suppl. 1.

286. Trauner M, Graziadei IW. Review article: mechanisms of action and therapeutic applications of ursodeoxycholic acid in chronic liver diseases. Aliment Pharmacol Ther 1999; 13(8): 979-96.

287. Fickert P, Zollner G, Fuchsbichler A, Stumptner C, Pojer C, Zenz R, Lammert F, et al. Effects of ursodeoxycholic and cholic acid feeding on hepatocellular transporter expression in mouse liver. Gastroenterology 2001; 121(1): 170-83.

288. Zollner G, Halilbasic E, Wagner M, Fickert P, Silbert D, Gumhold J, Fuchsbichler A, et al. Cholic acid (CA) and ursodeoxycholic acid (UDCA) exert different effects on hepatic and renal expression of Mrp4 and Mrp6, two novel ABC transporters. Gastroenterology 2004: 126[4, Suppl. 2], A-16.

289. Marschall HU, Wagner M, Zollner G, Fickert P, Gumhold J, Silbert D, Diczfalusy U, et al. Comprehensive study of the effects of rifampicin and ursodeoxycholic acid on genes and proteins involved in regulation, transport and detoxification of biliary compounds in gallstone patients. Gastroenterology 2004: 126[4, Suppl 2], A746.

290. Bodin K, Bretillon L, Aden Y, Bertilsson L, Broome U, Einarsson C, Diczfalusy U. Antiepileptic drugs increase plasma levels of 4beta-hydroxycholesterol in humans: evidence for involvement of cytochrome p450 3A4. J Biol Chem 2001; 276(42): 38685-9.

291. Ellis E, Axelson M, Abrahamsson A, Eggertsen G, Thorne A, Nowak G, Ericzon BG, et al. Feedback regulation of bile acid synthesis in primary human hepatocytes: evidence that CDCA is the strongest inhibitor. Hepatology 2003; 38(4): 930-8.

292. Marschall HU, Wagner M, Zollner G, Fickert P, Gumhold J, Silbert D, Fuchsbichler A, et al. FXR-/- mice defend against bile acid injury by enhanced phase I detoxification. Gastroenterology 2004; 126[4, Suppl. 2], A-674.

293. Kuipers F, Claudel T, Sturm E, Staels B. The Farnesoid X Receptor (FXR) as Modulator of Bile Acid Metabolism. Rev Endocr Metab Disord 2004; 5(4): 319-26.

294. Pellicciari R, Fiorucci S, Camaioni E, Clerici C, Costantino G, Maloney PR, Morelli A, et al. 6alpha-ethyl-chenodeoxycholic acid (6-ECDCA), a potent and selective FXR agonist endowed with anticholestatic activity. J Med Chem 2002; 45(17): 3569-72.

295. Fiorucci S, Clerici C, Antonelli E, Orlandi S, Goodwin B, Sadeghpour B, Sabatino G, et al. Protective Effects of 6-Ethyl Chenodeoxycholic Acid, A Farnesoid X receptor (FXR) Ligand, In Estrogen Induced Cholestasis. J Pharmacol Exp Ther 2005.

296. Fiorucci S, Antonelli E, Rizzo G, Renga B, Mencarelli A, Ricardi L, Orlandi S, et al. The nuclear receptor SHP mediates inhibition of hepatic stellate cells by FXR and protects against liver fibrosis. Gastroenterology 2004: in press.

297. Bachs L, Pares A, Elena M, Piera C, Rodes J. Comparison of rifampicin with phenobarbitone for treatment of pruritus in biliary cirrhosis. Lancet 1989; 1(8638): 574-6.

298. Bachs L, Pares A, Elena M, Piera C, Rodes J. Effects of long-term rifampicin administration in primary biliary cirrhosis. Gastroenterology 1992; 102(6): 2077-80.

299. Prince MI, Burt AD, Jones DE. Hepatitis and liver dysfunction with rifampicin therapy for pruritus in primary biliary cirrhosis. Gut 2002; 50(3): 436-9.

300. Becker M, von BK, Rotthauwe HW, Leiss O. Effects of phenobarbital on biliary lipid metabolism in children with chronic intrahepatic cholestasis. Eur J Pediatr 1984; 143(1): 41-4.

301. Bloomer JR, Boyer JL. Phenobarbital effects in cholestatic liver diseases. Ann Intern Med 1975; 82(3): 310-7.

302. Stiehl A, Thaler MM, Admirand WH. Effects of phenobarbital on bile salt metabolism in cholestasis due to intrahepatic bile duct hypoplasia. Pediatrics 1973; 51(6): 992-7.

303. Stiehl A, Thaler MM, Admirand WH. The effects of phenobarbital on bile salts and bilirubin in patients with intrahepatic and extrahepatic cholestasis. N Engl J Med 1972; 286(16): 858-61.

304. Sharp HL, Mirkin BL. Effect of phenobarbital on hyperbilirubinemia, bile acid metabolism, and microsomal enzyme activity in chronic intrahepatic cholestasis of childhood. J Pediatr 1972; 81(1): 116-26.

305. Marek CJ, Tucker SJ, Konstantinou DK, Elrick LJ, Haefner D, Sigalas C, Murray GI, et al. Pregnenolone 16alpha carbonitrile inhibits rodent liver fibrogenesis via PXR-dependent and PXR-independent mechanisms. Biochem J 2004.

306. Guo GL, Moffit J, Nicol CJ, Ward JM, Aleksunes L, Slitt A, Kliewer SA, et al. Enhanced Acetaminophen Toxicity by Activation of the Pregnane X Receptor. Toxicol Sci 2004.

307. Maglich JM, Watson J, McMillen PJ, Goodwin B, Willson TM, Moore JT. The nuclear receptor CAR is a regulator of thyroid hormone metabolism during caloric restriction. J Biol Chem 2004; 279(19): 19832-8.

308. Yamamoto Y, Moore R, Goldsworthy TL, Negishi M, Maronpot RR. The orphan nuclear receptor constitutive active/androstane receptor is essential for liver tumor promotion by phenobarbital in mice. Cancer Res 2004; 64(20): 7197-200.

309. Masuyama H, Hiramatsu Y, Kodama J, Kudo T. Expression and potential roles of pregnane X receptor in endometrial cancer. J Clin Endocrinol Metab 2003; 88(9): 4446-54.

310. Ritzel U, Leonhardt U, Nather M, Schafer G, Armstrong VW, Ramadori G. Simvastatin in primary biliary cirrhosis: effects on serum lipids and distinct disease markers. J Hepatol 2002; 36(4): 454-8.

311. Ohmoto K, Mitsui Y, Yamamoto S. Effect of bezafibrate in primary biliary cirrhosis: a pilot study. Liver 2001; 21(3): 223-4.

312. Kanda T, Yokosuka O, Imazeki F, Saisho H. Bezafibrate treatment: a new medical approach for PBC patients? J Gastroenterol 2003; 38(6): 573-8.

313. Nakai S, Masaki T, Kurokohchi K, Deguchi A, Nishioka M. Combination therapy of bezafibrate and ursodeoxycholic acid in primary biliary cirrhosis: a preliminary study. Am J Gastroenterol 2000; 95(1): 326-7.

314. Miranda S, Vollrath V, Wielandt AM, Loyola G, Bronfman M, Chianale J. Overexpression of mdr2 gene by peroxisome proliferators in the mouse liver. J Hepatol 1997; 26(6): 1331-9.

315. Hooiveld GJ, Vos TA, Scheffer GL, Van GH, Koning H, Bloks V, Loot AE, et al. 3-Hydroxy-3-methylglutaryl-coenzyme A reductase inhibitors (statins) induce hepatic expression of the phospholipid translocase mdr2 in rats. Gastroenterology 1999; 117(3): 678-87.

316. Carrella M, Feldman D, Cogoi S, Csillaghy A, Weinhold PA. Enhancement of mdr2 gene transcription mediates the biliary transfer of phosphatidylcholine supplied by an increased biosynthesis in the pravastatin-treated rat. Hepatology 1999; 29(6): 1825-32.

317. Kok T, Bloks VW, Wolters H, Havinga R, Jansen PL, Staels B, Kuipers F. Peroxisome proliferator-activated receptor alpha (PPARalpha)-mediated regulation of multidrug resistance 2 (Mdr2) expression and function in mice. Biochem J 2003; 369(Pt 3): 539-47.

318. Post SM, Groenendijk M, Solaas K, Rensen PC, Princen HM. Cholesterol 7alpha-hydroxylase deficiency in mice on an APOE*3-Leiden background impairs very-low-density lipoprotein production. Arterioscler Thromb Vasc Biol 2004; 24(4): 768-74.

319. Weitz-Schmidt G. Statins as anti-inflammatory agents. Trends Pharmacol Sci 2002; 23(10): 482-6.

320. Leuschner M, Guldutuna S, You T, Hubner K, Bhatti S, Leuschner U. Ursodeoxycholic acid and prednisolone versus ursodeoxycholic acid and placebo in the treatment of early stages of primary biliary cirrhosis. J Hepatol 1996; 25(1): 49-57.

321. Leuschner M, Maier KP, Schlichting J, Strahl S, Herrmann G, Dahm HH, Ackermann H, et al. Oral budesonide and ursodeoxycholic acid for treatment of primary biliary cirrhosis: results of a prospective double-blind trial. Gastroenterology 1999; 117(4): 918-25.

322. Mitchison HC, Palmer JM, Bassendine MF, Watson AJ, Record CO, James OF. A controlled trial of prednisolone treatment in primary biliary cirrhosis. Three-year results. J Hepatol 1992; 15(3): 336-44.

323. Warskulat U, Kubitz R, Wettstein M, Stieger B, Meier PJ, Haussinger D. Regulation of bile salt export pump mRNA levels by dexamethasone and osmolarity in cultured rat hepatocytes. Biol Chem 1999; 380(11): 1273-9.

324. Jung D, Fantin AC, Scheurer U, Fried M, Kullak-Ublick GA. Human ileal bile acid transporter gene ASBT (SLC10A2) is transactivated by the glucocorticoid receptor. Gut 2004; 53(1): 78-84.