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The mitochondrial matrix enzyme 3-oxoacid CoA transferase is homodimeric. It is a key enzyme in the extrahepatic utilization of ketone bodies, catalyzing the reversible transfer of coenzyme A from succinyl-CoA to acetoacetate, a necessary step in ketolytic energy production. Deficiencies can result in intermittent ketoacidosis.
Gene Name: | 3-oxoacid CoA transferase 1 |
Synonyms: | OXCT1, 3-oxoacid CoA transferase 1, 3-oxoacid CoA-transferase 1, 3-oxoacid-CoA transferase 1, 3-oxoacid CoA transferase, OXCT, SCOT, SCOT-s |
Target Sequences: | NM_000436 NP_000427.1 P55809 |
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