Succinyl CoA:3 ketoacid coenzyme A transferase 1 mitochondrial
Succinyl CoA:3 oxoacid CoA transferase
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
FunctionKey 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.
Tissue specificityAbundant in heart, followed in order by kidney, brain, and muscle, whereas in liver it is undetectable; also detectable in leukocytes and fibroblasts.
PathwayKetone metabolism; succinyl-CoA degradation; acetoacetyl-CoA from succinyl-CoA: step 1/1.
Involvement in diseaseSuccinyl-CoA:3-oxoacid CoA transferase deficiency
Sequence similaritiesBelongs to the 3-oxoacid CoA-transferase family.