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Defective ABCC2 causes DJS
Stable Identifier
R-HSA-5679001
Type
Pathway
Species
Homo sapiens
Synonyms
Defective ABCC2 causes Dubin-Johnson syndrome
ReviewStatus
5/5
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Defective ABCC2 causes DJS (Homo sapiens)
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Canalicular multispecific organic anion transporter 1 (ABCC2 aka multidrug resistance-associated protein 2, MRP2), in addition to transporting many organic anions, mediates the ATP-dependent transport of glutathione and glucuronate conjugates from hepatocytes into bile. ABCC2 transports with high affinity and efficiency mono- and di-glucuronated bilirubin into bile. Bilirubin, the end product of heme breakdown, is an important constituent of bile and is responsible for its characteristic colour. Defects in ABCC2 can cause Dubin-Johnson syndrome (DJS; MIM:237500), an autosomal recessive disorder characterised by conjugated hyperbilirubinemia (Dubin & Johnson 1954, Keppler 2014, Erlinger et al. 2014).
Literature References
PubMed ID
Title
Journal
Year
24459177
The roles of MRP2, MRP3, OATP1B1, and OATP1B3 in conjugated hyperbilirubinemia
Keppler, D
Drug Metab. Dispos.
2014
24704527
Inherited disorders of bilirubin transport and conjugation: new insights into molecular mechanisms and consequences
Erlinger, S
,
Dhumeaux, D
,
Arias, IM
Gastroenterology
2014
13193360
Chronic idiopathic jaundice with unidentified pigment in liver cells; a new clinicopathologic entity with a report of 12 cases
DUBIN, IN
,
JOHNSON, FB
Medicine (Baltimore)
1954
Participants
Events
Defective ABCC2 does not transport BMG,BDG from cytosol to extracellular region
(Homo sapiens)
Participates
as an event of
ABC transporter disorders (Homo sapiens)
Disease
Name
Identifier
Synonyms
Dubin-Johnson syndrome
DOID:12308
chronic idiopathic jaundice, Dubin Johnson syndrome
Authored
Jassal, B (2015-02-27)
Reviewed
Shukla, S (2015-09-15)
Created
Jassal, B (2015-02-27)
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