ABCC2
The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MRP subfamily which is involved in multi-drug resistance. This protein is expressed in the canalicular (apical) part of the hepatocyte and functions in biliary transport. Substrates include anticancer drugs such as vinblastine; therefore, this protein appears to contribute to drug resistance in mammalian cells. Several different mutations in this gene have been observed in patients with Dubin-Johnson syndrome (DJS), an autosomal recessive disorder characterized by conjugated hyperbilirubinemia. [provided by RefSeq, Jul 2008]
Full Name
ATP Binding Cassette Subfamily C Member 2
Function
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. Mediates hepatobiliary excretion of mono- and bis-glucuronidated bilirubin molecules and therefore play an important role in bilirubin detoxification. Mediates also hepatobiliary excretion of others glucuronide conjugates such as 17beta-estradiol 17-glucosiduronic acid and leukotriene C4. Transports sulfated bile salt such as taurolithocholate sulfate. Transport various anticancer drugs, such as anthracycline, vinca alkaloid and methotrexate and HIV-drugs such as protease inhibitors. Confers resistance to several anti-cancer drugs including cisplatin, doxorubicin, epirubicin, methotrexate, etoposide and vincristine.
Biological Process
Benzylpenicillin metabolic process
Bile acid and bile salt transport
Bile acid signaling pathway
Bilirubin transport
Canalicular bile acid transport
Cellular chloride ion homeostasis
Cellular response to dexamethasone stimulus
Cellular response to drug
Cellular response to interleukin-1
Cellular response to interleukin-6
Cellular response to lipopolysaccharide
Cellular response to tumor necrosis factor
Drug export
Drug transmembrane transport
Female pregnancy
Heme catabolic process
Inflammatory response
Leukotriene transport
Mercury ion transport
Negative regulation of gene expression
Prostaglandin transport
Regulation of bile acid secretion
Response to 17alpha-ethynylestradiol
Response to antineoplastic agent
Response to arsenic-containing substance
Response to estradiol
Response to estrogen
Response to glucagon
Response to heat
Response to methotrexate
Response to oxidative stress
Thyroid hormone transport
Transepithelial transport
Transmembrane transport
Transport across blood-brain barrier
Xenobiotic catabolic process
Xenobiotic detoxification by transmembrane export across the plasma membrane
Xenobiotic transport across blood-brain barrier
Cellular Location
Apical cell membrane
Involvement in disease
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.
Topology
Extracellular: 1-27 aa
Helical: 28-48 aa
Cytoplasmic: 49-68 aa
Helical: 69-89 aa
Extracellular: 90-93 aa
Helical: 94-114 aa
Cytoplasmic: 115-126 aa
Helical: 127-147 aa
Extracellular: 148-165 aa
Helical: 166-186 aa
Cytoplasmic: 187-313 aa
Helical: 314-334 aa
Extracellular: 335-360 aa
Helical: 361-381 aa
Cytoplasmic: 382-437 aa
Helical: 438-458 aa
Extracellular: 459-461 aa
Helical: 462-482 aa
Cytoplasmic: 483-544 aa
Helical: 545-565 aa
Extracellular: 566-587 aa
Helical: 588-608 aa
Cytoplasmic: 609-971 aa
Helical: 972-992 aa
Extracellular: 993-1033 aa
Helical: 1034-1054 aa
Cytoplasmic: 1055-1097 aa
Helical: 1098-1118 aa
Extracellular: 1119 aa
Helical: 1120-1140 aa
Cytoplasmic: 1141-1211 aa
Helical: 1212-1232 aa
Extracellular: 1233-1234 aa
Helical: 1235-1255 aa
Cytoplasmic: 1256-1545 aa