2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial

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

NCIt synonyms : Testis Secretory Sperm-Binding Protein Li 240mP; Branched-Chain Beta-Keto Acid Dehydrogenase E1 Component Beta Chain; BCKDHB; BCKDE1B; Branched Chain Keto Acid Dehydrogenase E1 Subunit Beta; 2-Oxoisovalerate Dehydrogenase Subunit Beta; EC 1.2.4.4; BCKDH E1-Beta;

NCIt definition : 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (392 aa, 43 kDa) is encoded by the human BCKDHB gene. This protein is involved in the catabolism of alpha-ketoacids derived from branched chain amino acids.;

Details

Origin ID

C215326;

OMIM relation
- Branched-chain keto acid dehydrogenase e1, beta polypeptide [OMIM gene]
gene_encodes_gene_product
- BCKDHB 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]
- Lipid Metabolic Process [NCIt concept]
- Oxidation [NCIt concept]
- Oxidation/Reduction [NCIt concept]

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