FUNCTION: THE PYRUVATE DEHYDROGENASE COMPLEX CATALYZES THE OVERALL CONVERSION OF PYRUVATE TO ACETYL-COA & CO(2). IT CONTAINS MULTIPLE COPIES OF THREE ENZYMATIC COMPONENTS: PYRUVATE DEHYDROGENASE (E1), DIHYDROLIPOAMIDE ACETYLTRANSFERASE (E2) & LIPOAMIDE DEHYDROGENASE (E3). CATALYTIC ACTIVITY: PYRUVATE + LIPOAMIDE = S-ACETYL-DIHYDRO- LIPOAMIDE + CO(2). COFACTOR: THIAMINE PYROPHOSPHATE. SUBUNIT: TETRAMER OF TWO ALPHA AND TWO BETA SUBUNITS. SUBCELLULAR LOCATION: MITOCHONDRIAL MATRIX. DISEASE: PYRUVATE DEHYDROGENASE DEFICIENCY IS THE MOST COMMON ENZYME DEFECT IN PATIENTS WITH PRIMARY LACTIC ACIDOSIS. CAUTION: REF.5 SEQUENCE DIFFERS FROM THAT SHOWN DUE TO FRAMESHIFTS. |
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06-JAN-01
Last Update 13-Mar-03