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- BCKDHA abstract "2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene.The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that consists of 3 catalytic components: a heterotetrameric (alpha2, beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2; MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331). The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase. The BCKDHA gene encodes the alpha subunit of E1, and the BCKDHB gene (MIM 248611) encodes the beta subunit of E1.[supplied by OMIM]".
- BCKDHA entrezgene "593".
- BCKDHA wikiPageID "14760208".
- BCKDHA wikiPageRevisionID "462157697".
- BCKDHA hasPhotoCollection BCKDHA.
- BCKDHA requireManualInspection "no".
- BCKDHA summaryText "The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is catalyzed by the branched-chain alpha-keto acid dehydrogenase complex , an inner-mitochondrial enzyme complex that consists of 3 catalytic components: a heterotetrameric branched-chain alpha-keto acid decarboxylase , a homo-24-meric dihydrolipoyl transacylase , and a homodimeric dihydrolipoamide dehydrogenase . The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase. The BCKDHA gene encodes the alpha subunit of E1, and the BCKDHB gene encodes the beta subunit of E1.[supplied by OMIM]".
- BCKDHA updateCitations "yes".
- BCKDHA updatePage "yes".
- BCKDHA updateProteinBox "yes".
- BCKDHA updateSummary "yes".
- BCKDHA type Biomolecule.
- BCKDHA type Protein.
- BCKDHA type BiologicalObject.
- BCKDHA comment "2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene.The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that consists of 3 catalytic components: a heterotetrameric (alpha2, beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2; MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331). ".
- BCKDHA label "BCKDHA".
- BCKDHA sameAs m.03gx04l.
- BCKDHA sameAs Q4835178.
- BCKDHA sameAs Q4835178.
- BCKDHA sameAs BCKDHA.
- BCKDHA wasDerivedFrom BCKDHA?oldid=462157697.
- BCKDHA isPrimaryTopicOf BCKDHA.