Canalicular multispecific organic anion transporter 1

ATP-dependent transporter of the ATP-binding cassette (ABC) family that binds and hydrolyzes ATP to enable active transport of various substrates including many drugs, toxicants and endogenous compound across cell membranes. Transports a wide variety of conjugated organic anions such as sulfate-, glucuronide- and glutathione (GSH)-conjugates of endo- and xenobiotics substrates (PubMed:10220572, PubMed:10421658, PubMed:11500505, PubMed:16332456). Mediates hepatobiliary excretion of mono- and bis-glucuronidated bilirubin molecules and therefore play an important role in bilirubin detoxification (PubMed:10421658). Mediates also hepatobiliary excretion of others glucuronide conjugates such as 17beta-estradiol 17-glucosiduronic acid and leukotriene C4 (PubMed:11500505). Transports sulfated bile salt such as taurolithocholate sulfate (PubMed:16332456). Transports various anticancer drugs, such as anthracycline, vinca alkaloid and methotrexate and HIV-drugs such as protease inhibitors (PubMed:10220572, PubMed:11500505, PubMed:12441801). Confers resistance to several anti-cancer drugs including cisplatin, doxorubicin, epirubicin, methotrexate, etoposide and vincristine (PubMed:10220572, PubMed:11500505).

Dubin-Johnson syndrome

DJS

Autosomal recessive disorder characterized by conjugated hyperbilirubinemia, an increase in the urinary excretion of coproporphyrin isomer I, deposition of melanin-like pigment in hepatocytes, and prolonged retention of sulfobromophthalein, but otherwise normal liver function.

None

The disease is caused by variants affecting the gene represented in this entry.

Belongs to the ABC transporter superfamily. ABCC family. Conjugate transporter (TC 3.A.1.208) subfamily.

Expressed by polarized cells in liver, kidney and intestine. The highest expression is found in liver. Expressed in small intestine (PubMed:28408210).

• Apical cell membrane
• Multi-pass membrane protein
• Localized to the apical membrane of enterocytes.

CMOAT, CMOAT1, CMRP, MRP2, ABCC2, ATP-binding cassette sub-family C member 2, Canalicular multidrug resistance protein, Canalicular multispecific organic anion transporter 1, Multidrug resistance-associated protein 2

• entrezGene:1244
• omim:601107
• swissprot:Q92887

Proteins

Metabolism

174207Da