Anti-SPTLC1 Antibody serum, from rabbit

Serine palmitoyltransferase 1 (EC 2.3.1.50; UniProt O15269; also known as LCB 1, Long chain base biosynthesis protein 1, Serine-palmitoyl-CoA transferase 1, SPT 1, SPT1) is encoded by the SPTLC1 (also known as HSAN1, HSAN1A, LCB1) gene (Gene ID 10558) in human. Serine palmitoyltransferase (SPT) catalyzes pyridoxal 5-phosphate-/PLP-dependent condensation of L-serine with palmitoyl-CoA to generate 3-ketodihydrosphingosine, the rate-limiting step for the de novo synthesis of sphingolipids. SPT belongs to the POAS (PLP-dependent alpha-oxoamine synthase) family of enzymes, members of this family also include 5-amino-levulinic acid synthase, KBL (2-amino-3 ketobutyrate ligase) and AONS (8-amino-7-oxononanoate synthase). Unlike the other known POAS family members that all exist as soluble homodimers, SPT is bound to the outer membrane of the endoplasmic reticulum (ER) as a multisubunit complex composed of three distinct subunits, SPTLC1, SPTLC2 and SPTLC3. SPTLC1 is a 473-amino acid sigle-transmembrane (a.a. 16-36) protein with a short N-terminal lumenal domain (a.a. 1-15) and a large C-terminal cytoplasmic domain (a.a. 37-473). Alternative splicing generates an isoform with a truncated cytoplasmic domain (D143 switched to E143, a.a. 144-473 truncated).