Succinyl CoA:3 ketoacid coenzyme A transferase 1 mitochondrial
Succinyl CoA:3 oxoacid CoA transferase
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
功能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.
组织特异性Abundant in heart, followed in order by kidney, brain, and muscle, whereas in liver it is undetectable; also detectable in leukocytes and fibroblasts.
通路Ketone metabolism; succinyl-CoA degradation; acetoacetyl-CoA from succinyl-CoA: step 1/1.
疾病相关Succinyl-CoA:3-oxoacid CoA transferase deficiency
序列相似性Belongs to the 3-oxoacid CoA-transferase family.