LMPD Database

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LMP000621

UniProt Annotations

Entry Information
Gene NameATPase, aminophospholipid transporter, class I, type 8B, member 1
Protein EntryAT8B1_HUMAN
UniProt IDO43520
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityATP + H(2)O + phospholipid(Side 1) = ADP + phosphate + phospholipid(Side 2).
DiseaseCholestasis, benign recurrent intrahepatic, 1 (BRIC1) [MIM
DiseaseCholestasis of pregnancy, intrahepatic 1 (ICP1) [MIM
DiseaseCholestasis, progressive familial intrahepatic, 1 (PFIC1) [MIM
FunctionCatalytic component of a P4-ATPase flippase complex which catalyzes the hydrolysis of ATP coupled to the transport of aminophospholipids from the outer to the inner leaflet of various membranes and ensures the maintenance of asymmetric distribution of phospholipids. Phospholipid translocation seems also to be implicated in vesicle formation and in uptake of lipid signaling molecules. May play a role in asymmetric distribution of phospholipids in the canicular membrane. May have a role in transport of bile acids into the canaliculus, uptake of bile acids from intestinal contents into intestinal mucosa or both. In cooperation with ABCB4 may be involved in establishing integrity of the canalicular membrane thus protecting hepatocytes from bile salts. Together with TMEM30A is involved in uptake of the synthetic drug alkylphospholipid perifosine. Involved in the microvillus formation in polarized epithelial cells; the function seems to be independent from its flippase activity. Required for the preservation of cochlear hair cells in the inner ear. May act as cardiolipin transporter during inflammatory injury. {ECO
InteractionQ9NV96:TMEM30A; NbExp=3; IntAct=EBI-9524729, EBI-2836942;
SimilarityBelongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IV subfamily.
Subcellular LocationCell membrane; Multi-pass membrane protein. Apical cell membrane. Cell projection, stereocilium . Endoplasmic reticulum. Golgi apparatus. Note=Exit from the endoplasmic reticulum requires the presence of TMEM30A or TMEM30B. Localizes to apical membranes in epithelial cells.
SubunitComponent of a P4-ATPase flippase complex which consists of a catalytic alpha subunit and an accessory beta subunit (Probable). The probable flippase ATP8B1:TMEM30A complex can form an intermediate phosphoenzyme in vitro. Also interacts with beta subunit TMEM30B. {ECO
Tissue SpecificityFound in most tissues except brain and skeletal muscle. Most abundant in pancreas and small intestine.