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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.
2610008O03Rik; 3-oxoacid CoA transferase 1; 3-oxoacid CoA-transferase 1; 3-oxoacid-CoA transferase 1; EMBL:AAI66478.1}; OXCT; Oxct1; oxct1 {ECO:0000312; Oxct2a; SCOT; SCOT-s; somatic-type succinyl CoA:3-oxoacid CoA-transferase; somatic-type succinyl-CoA:3-oxoacid CoA-transferase; somatic-type succinyl-CoA:3-oxoacid-CoA-transferase; succinyl CoA:3-oxoacid CoA transferase; succinyl-CoA:3-ketoacid CoA transferase; succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; succinyl-CoA:3-ketoacid-CoA transferase; succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial; Succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial-like protein; Unknown (protein for MGC:137519)
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