BCKDHA - 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial - human protein (Function)
 
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BCKDHA »  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial   [ EC 1.2.4.4 ]
 
Protein also known as:  Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain (BCKDE1A).
Gene name:  BCKDHA
Family name: BCKDHA
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 46 2
GENE REF ISO

Function

 show evidences
Overview 
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).  
1
  • UniProtKB
GO molecular function 
3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activitydefinition[GO:0003863]  
1
  • EC 2 GO
Alpha-ketoacid dehydrogenase activitydefinition[GO:0003826]  
1
  • HGNC
Carboxy-lyase activitydefinition[GO:0016831]  
1
  • HGNC
Metal ion bindingdefinition[GO:0046872]  
1
  • UniProtKB KW
Protein bindingdefinition[GO:0005515]  
4
  • IntAct
GO biological process 
Branched-chain amino acid catabolic processdefinition[GO:0009083]  
1
  • HGNC
Oxidation-reduction processdefinition[GO:0055114]  
1
  • UniProtKB KW
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 1.2.4.4: 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO(2).  
1
  • UniProtKB
It requires the following cofactor
 thiamine diphosphate  
1
  • UniProtKB
Pathways 
According to KEGG, this protein belongs to the following pathways:
Metabolic pathways  hsa01100+593  
Valine, leucine and isoleucine degradation  hsa00280+593  
According to Reactome, this protein belongs to the following pathway:
Branched-chain amino acid catabolism  REACT_197  
 
More information is available from:
Note 
Bound potassium ions stabilize the protein structure.  
1
  • UniProtKB
 

Keywords

Molecular function 
Oxidoreductase  definition   [KW-0560]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: P12694
Other
GeneWiki: BCKDHA
GenomeRNAi: 593
PRO: PR:P12694