Gene Information
Gene NameBCKDHA
OrganismHomo sapiens (Human)
Gene Length445
Protein Names2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (EC 1.2.4.4) (Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDE1A) (BCKDH E1-alpha)
Target NameNA
Target TypeNA
Gene Age1714 ( ENSG00000248098 )
Evolutionary StageCellular_organisms
AlphaFoldDBP12694
Gene CardBCKDHA
Uniprot IDP12694
PfamPF00676
In ASDno
Allosteric Predictionno (-0.103155655)
Ortholog in AnimalsAlpaca_ BCKDHA ( ortholog_one2one )
Dog_ TMEM91 ( ortholog_one2one )
GuineaPig_ BCKDHA ( ortholog_one2one )
Macaque_ BCKDHA ( ortholog_one2one )
Mouse_ Bckdha ( ortholog_one2one )
Pig_ BCKDHA ( ortholog_one2one )
Rat_ Bckdha ( ortholog_one2one )
Rabbit_ BCKDHA ( ortholog_one2one )
OhnologsNA
ParalogsPDHA1, PDHA2
TissueNA
Tissue SpecificityNA
Pharmacological Animal ModelsNULL(Chimpanzee);NULL(Mouse);0.004990(Rat);NULL(Rabbit)
Gene Ontology
(biological process)
Gene Ontology
(cellular component)
Gene Ontology
(molecular function)
PDB 1DTW  (X-ray  2.70A  1OLS  (X-ray  1.85A  1OLU  (X-ray  1.90A  1OLX  (X-ray  2.25A  1U5B  (X-ray  1.83A  1V11  (X-ray  1.95A  1V16  (X-ray  1.90A  1V1M  (X-ray  2.00A  1V1R  (X-ray  1.80A  1WCI  (X-ray  1.84A  1X7W  (X-ray  1.73A  1X7X  (X-ray  2.10A  1X7Y  (X-ray  1.57A  1X7Z  (X-ray  1.72A  1X80  (X-ray  2.00A  2BEU  (X-ray  1.89A  2BEV  (X-ray  1.80A  2BEW  (X-ray  1.79A  2BFB  (X-ray  1.77A  2BFC  (X-ray  1.64A  2BFD  (X-ray  1.39A  2BFE  (X-ray  1.69A  2BFF  (X-ray  1.46A  2J9F  (X-ray  1.88A 
DBSNP rs11549936  rs34442879  rs769688327  rs398123503  rs375785084  rs199599175  rs137852871  rs182923857  rs137852872  rs137852870 
Subcellular location [CC]Mitochondrion matrix.
Mouse PG classificationNA
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