OCTN1 antibodyOCTN1 抗体

Catalog Number :RS-88260R Quantity size :?? 0.1ml?（dilute with pH 7.4??? 0.01 M PBS or antibody diluent ） background: Carnitine (β-hydroxy-γ-trimethylaminobutyrate) is a small, highly polar compound that aids in the β-oxidation of long-chain fatty acids. Organic cation/carnitine transporters (OCTN) assist in the elimination of cationic compounds,including xenobiotics, and transport carnitine for reabsorption in the kidney.Similar to organic cation transporters (OCT), OCTN proteins localize to the plasma membrane of epithelial cells. OCTN1 is expressed in kidney, trachea,bone marrow and fetal liver. OCTN2 is abundantly expressed in kidney, skeletal muscle, placenta and heart. OCTN3 is strongly expressed in testis and weakly expressed in kidney. Mutations in the gene encoding OCTN2 leads to systemic carnitine deficiency (SCD), an autosomal recessive disorder characterized by cardiomyopathy, skeletal myopathy, lethargy, hypoglycemia and hyperammonemia.