Gene:
3-oxoacid CoA transferase 1
Gene ID:
201191
Symbol:
OXCT1
Species:
1000
Description:
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.