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Homozygous disruption of the murine mdr2 p-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease
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Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis
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of outstanding interest. This study is the first molecular characterization of a hereditary form of cholestatic liver disease. It highlights the importance of biliary phospholipids as protective agents against bile duct damage by bile acids.
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de Vree JML, Jacquemin E, Sturm E, Cresteil D, Bosma PJ, Aten J, Deleuze J-F, Desrochers M, Burdelski M, Bernard O, et al. Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis. of outstanding interest Proc Natl Acad Sci USA. 95:1998;282-287 This study is the first molecular characterization of a hereditary form of cholestatic liver disease. It highlights the importance of biliary phospholipids as protective agents against bile duct damage by bile acids.
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MDR1 p-glycoprotein is a lipid translocase of broad specificity, while MDR3 p-glycoprotein specifically translocates phosphatidycholine
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Hepatic secretion of phospholipid vesicles in the mouse critically depends on mdr2 or MDR3 p-glycoprotein expression
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of special interest. This study presents an interesting model of the mechanism of bile acid induced lipid secretion into bile and supports it with experimental evidence obtained from genetically altered mice.
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34
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of special interest. This is the description of the molecular defect in the MRP2 gene of a patient with Dubin - Johnson syndrome. The mutation leads to the premature termination of the protein and hence to its absence from the canalicular plasma membrane.
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Paulusma CC, Kool M, Bosma PJ, Scheffer GL, ter Borg F, Scheper RJ, Tytgat GNJ, Borst P, Baas F, Oude Elferink RPJ. A mutation in the human canalicular multispecific organic anion transporter gene causes the Dubin-Johnson syndrome. of special interest Hepatology. 25:1997;1539-1542 This is the description of the molecular defect in the MRP2 gene of a patient with Dubin - Johnson syndrome. The mutation leads to the premature termination of the protein and hence to its absence from the canalicular plasma membrane.
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A human canalicular multispecific organic anion transporter (cMOAT) gene is overexpressed in cisplatin-resistant human cancer cell lines with decreased drug accumulation
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Taniguchi K, Wada M, Kohno K, Nakamura T, Kawabe T, Kawakami M, Kagotani K, Okumura K, Akiyama S, Kuwano M. A human canalicular multispecific organic anion transporter (cMOAT) gene is overexpressed in cisplatin-resistant human cancer cell lines with decreased drug accumulation. Cancer Res. 56:1996;4124-4129.
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Mutations in the canalicular multispecific organic anion transporter (cMOAT) gene, a novel ABC transporter, in patients with hyperbilirubinemia II/Dubin-Johnson syndrome
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The sister of p-glycoprotein represents the canalicular bile salt export pump of mammalian liver
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of special interest. This paper describes the cloning and localization of an ATP-dependent bile acid transport system in the canalicular plasma membrane of rat hepatocytes. This is the first bile acid transporter isolated from the canalicular plasma membrane of mammalian liver.
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Gerloff T, Stieger B, Hagenbuch B, Madon J, Landmann L, Roth J, Hofmann AF, Meier PJ. The sister of p-glycoprotein represents the canalicular bile salt export pump of mammalian liver. of special interest J Biol Chem. 273:1998;10046-10050 This paper describes the cloning and localization of an ATP-dependent bile acid transport system in the canalicular plasma membrane of rat hepatocytes. This is the first bile acid transporter isolated from the canalicular plasma membrane of mammalian liver.
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Gerloff, T.1
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Identification of a locus for progressive familial intrahepatic cholestasis PFIC2 on chromosome 2q24
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A gene encoding a p-type ATPase mutated in two forms of hereditary cholestasis
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of special interest. In this paper, the gene responsible for a hereditary form of cholestatic liver disease has been identified by positional cloning. The protein encoded is related to a P-type ATPase thought to be an aminophospholipid translocator.
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Bull LN, van Eijk MJT, Pawlikowska L, DeYoung JA, Juijn JA, Liao M, Klomp LWJ, Lomri N, Berger R, Scharschmidt BF, et al. A gene encoding a p-type ATPase mutated in two forms of hereditary cholestasis. of special interest Nat Genet. 18:1998;219-224 In this paper, the gene responsible for a hereditary form of cholestatic liver disease has been identified by positional cloning. The protein encoded is related to a P-type ATPase thought to be an aminophospholipid translocator.
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Kubitz R, D'Urso D, Keppler D, Häussinger D. Osmodependent dynamic localization of the multidrug resistance protein 2 in the rat hepatocyte canalicular membrane. Gastroenterology. 113:1997;1438-1442.
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Mitogen-activated protein kinases mediate the stimulation of bile acid secretion by tauroursodeoxycholate in rat liver
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50
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0030845302
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Hepatocellular ATP-binding cassette protein expression enhances ATP release and autocrine regulation of cell volume
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Roman RM, Wang Y, Lidofsky SD, Feranchak AP, Lomri N, Scharschmidt BF, Fitz JG. Hepatocellular ATP-binding cassette protein expression enhances ATP release and autocrine regulation of cell volume. J Biol Chem. 272:1997;21970-21976.
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51
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Identification of a new p-glycoprotein-like ATP-binding cassette transporter gene that is overexpressed during hepatocarcinogenesis
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Furuya KN, Bradley G, Sun D, Schuetz EG, Schuetz JD. Identification of a new p-glycoprotein-like ATP-binding cassette transporter gene that is overexpressed during hepatocarcinogenesis. Cancer Res. 57:1997;3708-3716.
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52
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0030810683
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Phenotypic characterization of Lith genes that determine susceptibility to cholesterol cholelithiasis in inbred mice: Physical-chemistry of gall bladder bile
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Wang DQ-H, Paigen B, Carey MC. Phenotypic characterization of Lith genes that determine susceptibility to cholesterol cholelithiasis in inbred mice: physical-chemistry of gall bladder bile. J Lipid Res. 38:1997;1395-1411.
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A new pathway for vacuolar cadmium sequestration in Saccharomyces cerevisiae: YCF1-catalyzed transport of bis(glutathionato) cadmium
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Li Z-S, Lu Y-P, Zhen R-G, Szczypka M, Thiele DJ, Rea PA. A new pathway for vacuolar cadmium sequestration in Saccharomyces cerevisiae: YCF1-catalyzed transport of bis(glutathionato) cadmium. Proc Natl Acad Sci USA. 94:1997;42-47.
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54
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0030971840
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Structure of the multidrug resistance p-glycoprotein to 2.5 nm resolution determined by electron microscopy and image analysis
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Rosenberg MF, Callaghan R, Ford RC, Higgins CF. Structure of the multidrug resistance p-glycoprotein to 2.5 nm resolution determined by electron microscopy and image analysis. J Biol Chem. 272:1997;10685-10694.
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Rosenberg, M.F.1
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A general pattern for substrate recognition by p-glycoprotein
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Seelig A. A general pattern for substrate recognition by p-glycoprotein. Eur J Biochem. 251:1998;252-261.
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