2-Oxoisovalerate Dehydrogenase Subunit Alpha, Mitochondrial

Preferred Label : 2-Oxoisovalerate Dehydrogenase Subunit Alpha, Mitochondrial;

NCIt synonyms : BCKDH E1-Alpha; 2-Oxoisovalerate Dehydrogenase Subunit Alpha; Branched Chain Keto Acid Dehydrogenase E1 Subunit Alpha; BCKDHA; BCKDE1A; EC 1.2.4.4; Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Alpha Chain;

NCIt definition : 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (445 aa, 50 kDa) is encoded by the human BCKDHA gene. This protein plays a role in the decarboxylation of alpha-ketoacids derived from branched-chain amino acids.;

Détails

Origin ID

C215323;

OMIM relation
- Branched-chain keto acid dehydrogenase e1, alpha polypeptide [OMIM gene]
gene_encodes_gene_product
- BCKDHA Gene [NCIt concept]
- Enzyme Gene [NCIt concept]
- Oxidoreductase Gene [NCIt concept]
gene_product_has_associated_anatomy
- Mitochondrial Matrix [NCIt concept]
gene_product_has_structural_domain_or_motif
- Substrate Interaction Domain [NCIt concept]
gene_product_is_element_in_pathway
- Lipoic Acid Metabolism Pathway [NCIt concept]
- Propanoate Metabolism Pathway [NCIt concept]
- Valine, Leucine and Isoleucine Biosynthesis Pathway [NCIt concept]
gene_product_plays_role_in_biological_process
- Amino Acid Metabolic Process [NCIt concept]
- Catabolic Process [NCIt concept]
- Detoxification [NCIt concept]
- Metal Ion Binding [NCIt concept]
- Oxidation [NCIt concept]
- Oxidation/Reduction [NCIt concept]

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