OXCT1encodes 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.OXCT1 is a mitochondrial matrix enzyme that functions as a homodimer. From human heart cDNA libraries, Kassovska-Bratinova et al. (1996) cloned cDNAs spanning the entire 1,560-bp coding sequence using pig heart OXCT1 cDNA as a probe. They reported that a single mRNA transcript of 3.2 kb was present in human tissues. OXCT1 mRNA was most abundant in heart and was also detectable in leukocytes and fibroblasts.