title: Erythrocyte membrane transport of glutathione conjugates and oxidized glutathione in the Dubin-Johnson syndrome and in rats with hereditary hyperbilirubinemia.
abstract: The Dubin-Johnson syndrome is manifested by conjugated hyperbilirubinemia and pigment accumulation in hepatocellular lysosomes. The TR-rat model is a phenotypic model of the Dubin-Johnson syndrome and is characterized by defective ATP-dependent transport of a group of nonbile acid organic anions, including glutathione-S-conjugates and oxidized glutathione, across the bile canaliculus. Similar ATP-dependent transport mechanisms have been described in erythrocytes. Intact erythrocytes and inverted erythrocyte membrane vesicles from Dubin-Johnson patients, TR-rats and appropriate controls were studied with regard to ATP-dependent transport of dinitrophenyl glutathione and oxidized glutathione. No significant differences were observed, indicating that the erythrocyte and canalicular ATP-dependent transporters for these substrates are functionally and potentially genetically distinct.
authors.affiliation: Department of Physiology, Tufts University School of Medicine, Boston, Massachusetts 02111.
authors.forename: P
authors.initials: P
authors.lastname: Board
authors.forename: T
authors.initials: T
authors.lastname: Nishida
authors.forename: Z
authors.initials: Z
authors.lastname: Gatmaitan
authors.forename: M
authors.initials: M
authors.lastname: Che
authors.forename: I M
authors.initials: IM
authors.lastname: Arias
mesh_terms: D000255:Adenosine Triphosphate / Q000494:pharmacology; D000818:Animals; D001692:Biological Transport / Q000187:drug effects; D004910:Erythrocyte Membrane / Q000378:metabolism*; D005260:Female; D005978:Glutathione / Q000031:analogs & derivatives* / Q000097:blood / Q000378:metabolism*; D019803:Glutathione Disulfide; D006801:Humans; D006933:Hyperbilirubinemia, Hereditary / Q000097:blood*; D007566:Jaundice, Chronic Idiopathic / Q000097:blood*; D008297:Male; D051381:Rats; D011919:Rats, Inbred Strains; D013379:Substrate Specificity
chemical_list: C030190:S-(2,4-dinitrophenyl)glutathione; D000255:Adenosine Triphosphate; D005978:Glutathione; D019803:Glutathione Disulfide