Serine palmitoyltransferase, which consists of two different subunits, is the key enzyme in sphingolipid biosynthesis. It converts L-serine and palmitoyl-CoA to 3-oxosphinganine with pyridoxal 5'-phosphate as a cofactor. SPT1 is the long chain base subunit 1 of mammalian serine palmitoyltransferase. SPT1 is not catalytically active but is necessary for the stabilization of the SPT2 subunit and anchoring the holoenzyme to the cytosolic face of the endoplasmic reticulum. Missense mutations in this gene have been identified in patients with hereditary sensory neuropathy type 1 (HSAN1). These mutations induce a shift in the substrate specificity of the holoenzyme, leading to the formation and accumulation of two neurotoxic sphingolipids.
应用类型
ELISA,Immunohistochemistry,Western Blot,
免疫原
Anti-SPT1 antibody was prepared from whole rabbit serum produced by repeated immunizations with a 17 amino acid synthetic peptide near the C-terminus of human SPT1.
来源宿主
Rabbit
反应性
H. sapiens (Human)
保存建议
Store vial at -20° C prior to opening. Aliquot contents and freeze at -20° C or below for extended storage. Avoid cycles of freezing and thawing. Centrifuge product if not completely clear after standing at room temperature. This product is stable for several weeks at 4° C as an undiluted liquid. Dilute only prior to immediate use.
