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Assem M, Schuetz EG, Leggas M, et al. Interactions between hepatic Mrp4 and Sult2a as revealed by the constitutive androstane receptor and Mrp4 knockout mice. J Biol Chem 2004; 279:22250-22257. Sult2a also appears to play a role in the excretion of toxic bile acids during cholestasis.
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Gissen P, Johnson CA, Morgan NV, et al. Mutations in VPS33B, encoding a regulator of SNARE-dependent membrane fusion, cause arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome. Nat Genet 2004; 36:400-404.
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Glantz A, Marschall HU, Mattsson LA. Intrahepatic cholestasis of pregnancy. relationships between bile acid levels and fetal complication rates. Hepatology 2004; 40:467-474. Study suggesting it may be possible to identify patients with ICP at highest risk for fetal complications.
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Selmi C, Mayo MJ, Bach N, et al. Primary biliary cirrhosis in monozygotic and dizygotic twins:genetics, epigenetics, and environment. Gastroenterology 2004; 127:485-492.
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Cloning the human betaretrovirus praviral genome from patients with primary biliary cirrhosis
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Xu L, Sakalian M, Shen Z. Cloning the human betaretrovirus praviral genome from patients with primary biliary cirrhosis. Hepatology 2004; 39:151-156. Suggests a role for a viral infection in triggering PBC.
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Xu, L.1
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Selmi C, Ross SR, Ansari AA. Lack of immunological or molecular evidence for a role of mouse mammary tumor retrovirus in primary biliary cirrhosis. Gastroenterology 2004; 127:493-501. A study refuting an association between human betaretrovirus and PBC.
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Plamer J, Robe AJ, Burt AD, et al. Covalent modification as a mechanism for the breakdown of immune tolerance to pyruvate dehydrogenase complex in the mouse. Hepatology 2004; 39:1583-1592. This study showed how xenobiotics could produce altered self-antigens and loss of immune tolerance.
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Plamer, J.1
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Matsumura S, Van de Water J, Leung P. Caspase induction by IgA antimitochondrial antibody. IgA-mediated biliary injury in primary biliary cirrhosis. Hepatology 2004; 39:1415-1422. First study to suggest a possible pathogenic role for AMA.
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Reddy A, Prince M, James OF, et al. Tamoxifen: a novel treatment for primary biliary cirrhosis. Liver Int 2004; 24:194-197.
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Kaplan MM, Cheng S, Price LL, Bonis PAL. A randomized controlled trial of colchicine plus ursodiol versus methotrexate plus ursodiol in primary biliary cirrhosis: ten-year results. Hepatology 2004; 39:915-923. Results of a long-term study comparing colchicine to methotrexate in PBC.
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Kingham JG, Kochar N, Gravenor MB. Incidence, clinical patterns, and outcomes of primary sclerosing cholangitis in South Wales, United Kingdom. Gastroenterology 2004; 126:1929-1930.
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Rost D, Rudolph G, Kloeters-Plachky P, Stiehl A. Effect of high-dose ursodeoxycholic acid on its biliary enrichment in primary sclerosing cholangitis. Hepatology 2004; 40:693-698. Shows that biliary enrichment with UDCA plateaus at a dose of 22-25 mg/kg and that this may represent the optimal dose in patients with PSC.
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