基因名称(Gene Name)
OXCT1 OXCT SCOT
免疫原
Synthesized peptide derived from human SCOT1 AA range: 105-155
特异性
This antibody detects endogenous levels of SCOT1 at Human/Mouse/Rat
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse serum by affinity-chromatography using specific immunogen.
背景
This gene encodes a member of the 3-oxoacid CoA-transferase gene family. The encoded protein is a homodimeric mitochondrial matrix enzyme that plays a central role in extrahepatic ketone body catabolism by catalyzing the reversible transfer of coenzyme A from succinyl-CoA to acetoacetate. Mutations in this gene are associated with succinyl CoA:3-oxoacid CoA transferase deficiency. [provided by RefSeq, Jul 2008],
功能
catalytic activity:Succinyl-CoA + a 3-oxo acid = succinate + a 3-oxoacyl-CoA.,disease:Defects in OXCT1 are a cause of ketoacidosis [MIM:245050].,function:Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate.,pathway:Ketone metabolism; succinyl-CoA degradation; acetoacetyl-CoA from succinyl-CoA: step 1/1.,similarity:Belongs to the 3-oxoacid CoA-transferase family.,subunit:Homodimer.,tissue specificity:Abundant in heart, followed in order by kidney, brain, and muscle, whereas in liver it is undetectable; also detectable in leukocytes and fibroblasts.,
