BCKDHA

BCKDHA
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1DTW, 1OLS, 1OLU, 1OLX, 1U5B, 1V11, 1V16, 1V1M, 1V1R, 1WCI, 1X7W, 1X7X, 1X7Y, 1X7Z, 1X80, 2BEU, 2BEV, 2BEW, 2BFB, 2BFC, 2BFD, 2BFE, 2BFF, 2J9F
Identifiers
Aliases	BCKDHA, BCKDE1A, MSU, MSUD1, OVD1A, branched chain keto acid dehydrogenase E1, alpha polypeptide, branched chain keto acid dehydrogenase E1 subunit alpha
External IDs	OMIM: 608348; MGI: 107701; HomoloGene: 569; GeneCards: BCKDHA; OMA:BCKDHA - orthologs
Gene location (Human)
Chr.	Chromosome 19 (human)
End	41,425,002 bp
Gene location (Mouse)
Chr.	Chromosome 7 (mouse)
End	25,358,406 bp
RNA expression pattern
	Top expressed in
	right adrenal gland; ; apex of heart; ; right adrenal cortex; ; right lobe of liver; ; left adrenal gland; ; left adrenal cortex; ; left ventricle; ; gastrocnemius muscle; ; muscle of thigh; ; body of stomach;
	Top expressed in
	right kidney; ; muscle of thigh; ; brown adipose tissue; ; right ventricle; ; human kidney; ; lacrimal gland; ; left lobe of liver; ; myocardium of ventricle; ; proximal tubule; ; salivary gland;
	More reference expression data
	n/a
Gene ontology
Molecular function	oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor; protein binding; metal ion binding; oxidoreductase activity; carboxy-lyase activity; 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity;
Cellular component	mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion;
Biological process	metabolism; branched-chain amino acid catabolic process; cellular nitrogen compound metabolic process;
	Sources:Amigo / QuickGO
Orthologs
	593
	12039
	ENSG00000248098
	ENSMUSG00000060376
	P12694
	P50136
	NM_001164783; NM_000709
	NM_007533
	NP_000700; NP_001158255
	n/a
	Wikidata
View/Edit Human	View/Edit Mouse

A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene.

BCKDHA is a coding gene that is part of the BCKD complex (branched-chain alpha-keto acid dehydrogenase).