SLC51A
Function
Essential component of the Ost-alpha/Ost-beta complex, a heterodimer that acts as the intestinal basolateral transporter responsible for bile acid export from enterocytes into portal blood (PubMed:16317684). Efficiently transports the major species of bile acids (taurocholate) (PubMed:16317684). Taurine conjugates are transported more efficiently across the basolateral membrane than glycine-conjugated bile acids (By similarity). Can also transport steroids such as estrone 3-sulfate and dehydroepiandrosterone 3-sulfate, therefore playing a role in the enterohepatic circulation of sterols (PubMed:16317684). Able to transport eicosanoids such as prostaglandin E2 (By similarity).
Involvement in disease
Cholestasis, progressive familial intrahepatic, 6
PFIC6
An autosomal recessive form of progressive cholestasis, a disorder characterized by early onset of cholestasis that progresses to hepatic fibrosis, cirrhosis, and end-stage liver disease. PFIC6 patients have elevated liver transaminases and congenital diarrhea.
None
The disease is caused by variants affecting the gene represented in this entry.
Sequence Similarities
Belongs to the OST-alpha family.
Tissue Specificity
Widely expressed with a high expression in ileum. Expressed in testis, colon, liver, small intestine, kidney, ovary and adrenal gland; and at low levels in heart, lung, brain, pituitary, thyroid gland, uterus, prostate, mammary gland and fat.
Cellular localization
- Cell membrane
- Multi-pass membrane protein
- Endoplasmic reticulum membrane
- Multi-pass membrane protein
- Transported from the endoplasmic reticulum to the plasma membrane upon interacting with SLC51B (By similarity). Mainly restricted to the lateral and basal membranes of ileal enterocytes.
Alternative names
OSTA, SLC51A, Organic solute transporter subunit alpha, OST-alpha, Solute carrier family 51 subunit alpha