Altered coproporphyrin-isomer excretion in patients with the Dubin-Johnson syndrome
โ Scribed by Pentti Koskelo; Pertti Mustajoki
- Publisher
- Elsevier Science
- Year
- 1980
- Tongue
- English
- Weight
- 409 KB
- Volume
- 12
- Category
- Article
- ISSN
- 0020-711X
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๐ SIMILAR VOLUMES
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, in
Dubin-Johnson patients, mutant Corriedale sheep and TRand EHBR mutant rats have recessively inherited defective bile canalicular secretion of many nonbile acid organic anions. The human and ovine mutants have black livers and lysosomal pigment accumulation. The livers in TR-and EHBR mutant rats are