Mediates ATP-dependent export of organic anions and drugs from the cytoplasm. Hydrolyzes ATP with low efficiency. Human MDR3 is not capable of conferring drug resistance. Mediates the translocation of phosphatidylcholine across the canalicular membrane of the hepatocyte. Defects in ABCB4 are the cause of progressive familial intrahepatic cholestasis type 3 (PFIC3). PFIC3 is an autosomal recessive liver disorder presenting with early onset cholestasis that progresses to cirrhosis and liver failure before adulthood. It is characterized by elevated serum gamma-glutamyltransferase levels. Defects in ABCB4 are a cause of intrahepatic cholestasis of pregnancy (ICP); also known as obstetric cholestasis. ICP causes fetal distress, spontaneous premature delivery and intrauterine death. ICP patients have spontaneous and progressive disappearance of cholestasis after delivery. Defects in ABCB4 are a cause of gallbladder disease type 1 (GBD1). It is one of the major digestive diseases.
Clone
Polyclonal
Isotype
IgG
Host species
Rabbit
Species Reactivity
Human, mouse, rat
Cellular Localization
membrane
Positive Control
HCC, heart tissue lysates
Applications
IHC, Flow Cyt., ICC/IF, WB
Intended Use
Research Use Only