Chronic idiopathic jaundice with unidentified pigment in liver cells: A new clinico-pathologic entity with a report of twelve cases

Dubin, I. N.; Johnson, Frank B.

doi:10.1016/0002-9343(54)90461-1

Cited by 77 publications

(106 citation statements)

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“…The phenotype of the human Dubin-Johnson syndrome strongly suggests that cBAT and cMOAT are separate transport systems which mediate the hepatobiliary transport of bile salt and nonbile salt organic anions, respectively. Dubin-Johnson syndrome is a rare congenital autosomal recessive defect characterized by a chronic conjugated hyperbilirubinemia [12,13]. In these patients it has been demonstrated that the hepatobiliary transport of bilirubin and other nonbile salt organic anions is severely impaired [16,17].…”

Section: Hepatobiliary Transport Of Bile Salt and Nonbile Salt Organimentioning

confidence: 99%

“…Two syndromes have been described which are characterized by high serum levels of, predominantly conjugated bilirubin: Dubin-Johnson's syndrome [12,13] and Rotor's syndrome [81]. The genetic defect underlying the impaired hepatobiliary transport in Dubin-Johnson patients has not been identified yet, but the cloning of the human cMOAT cDNA will elucidate whether this gene is involved in Dubin-Johnson syndrome.…”

Section: Hereditary Chronic Hyperbilirubinemias In Manmentioning

confidence: 99%

“…Dubin-Johnson syndrome is a rare and benign disorder first described in 1954 [12,13]. The syndrome is frequently observed among Persian Jews (1:1300) [83] in whom it is often linked to coagulation factor VII deficiency [84,85].…”

Section: Dubin-johnson Syndromementioning

confidence: 99%

“…An important phenotypic feature of the Dubin-Johnson syndrome is the lysosomal pigment accumulation in the hepatocyte [12,13]. Subcellular fractionation experiments on liver biopsies demonstrate that lysosomes accumulate melanin [94].…”

Section: Dubin-johnson Syndromementioning

confidence: 99%

“…These rats have an autosomal recessive defect in the hepatobiliary excretion of a wide variety of negatively charged, amphiphilic organic anions. TR -rats have a similar phenotype as patients with Dubin-Johnson syndrome; this syndrome is characterized by a chronic conjugated hyperbilirubinemia which results in jaundice and is caused by impaired hepatobiliary excretion of many organic compounds [12,13].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

The canalicular multispecific organic anion transporter and conjugated hyperbilirubinemia in rat and man

Paulusma

Elferink

1997

Journal of Molecular Medicine

104

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The canalicular multispecific organic anion transporter and conjugated hyperbilirubinemia in rat and man Paulusma, C.C.; Oude Elferink, R.P.J. Disclaimer/Complaints regulationsIf you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library: http://uba.uva.nl/en/contact, or a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible. Download date: 22 Mar 2019& p . 1 : Abstract The human Dubin-Johnson syndrome is an autosomal recessive liver disease characterized by a chronic conjugated hyperbilirubinemia. Patients have impaired hepatobiliary transport of many endogenous and xenobiotic compounds. A similar disease phenotype has been described for a naturally occurring mutant Wistar rat strain, the TR -rat, which is defective in the, functionally defined, canalicular multispecific organic anion transporter (cMOAT). The complementary DNA encoding this protein has been cloned from rat and recently from human liver. cMOAT is a new member of the ATPbinding cassette transporter family, and homologous to the multidrug resistance-associated protein 1. A mutation in the cMOAT gene is responsible for the phenotype observed in TR -rats. This information should soon lead to a complete genetic characterization of the human DubinJohnson syndrome.

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Section: Hepatobiliary Transport Of Bile Salt and Nonbile Salt Organimentioning

confidence: 99%

Section: Hereditary Chronic Hyperbilirubinemias In Manmentioning

confidence: 99%