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. * CATALYTIC ACTIVITY: Succinyl-CoA + a 3-oxo acid = succinate + a 3-oxo-acyl-CoA. * PATHWAY: FIRST STEP OF KETOLYSIS IN EXTRAHEPATIC TISSUES. * SUBUNIT: Homodimer. * SUBCELLULAR LOCATION: Mitochondrial matrix. * 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. * DISEASE: DEFECTS IN OXCT ARE THE CAUSE OF A FORM OF HYPERKETONEMIA WITH EPISODIC SEVERE KETOACIDOSIS. * SIMILARITY: BELONGS TO THE 3-OXOACID COA-TRANSFERASE FAMILY. |
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23-Jan-03
Last Update 13-Mar-03