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.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Mitochondrion .
组织表达： Abundant in heart, followed in order by brain, kidney, skeletal muscle, and lung, whereas in liver it is undetectable. Expressed (at protein level) in all tissues (except in liver), most abundant in myocardium, then brain, kidney, adrenal glands, skeletal muscle and lung; also detectable in leukocytes and fibroblasts.
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.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Mitochondrion .
组织表达： Abundant in heart, followed in order by brain, kidney, skeletal muscle, and lung, whereas in liver it is undetectable. Expressed (at protein level) in all tissues (except in liver), most abundant in myocardium, then brain, kidney, adrenal glands, skeletal muscle and lung; also detectable in leukocytes and fibroblasts.
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