UniProt Accession

 ODBB_HUMAN; P21953; Q9BQL0 

Genbank Protein ID

 M55575; D90391; AK289977; BT020063; AL049696; AL391595; AL391595; AL049696; CH471051; CH471051; BC040139; U50708; X52446 

Genbank Nucleotide ID

 AAA51812.1; BAA14389.1; BAF82666.1; AAV38866.1; CAC36881.2; CAC36881.2; CAI15049.1; CAI15049.1; EAW48696.1; EAW48697.1; AAH40139.1; AAB16763.1; CAA36685.1 

Protein Name

 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial 

Protein Synonyms/Alias

 Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; BCKDE1B; BCKDH E1-beta 

Gene Name

 BCKDHB 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
241	PCIFFEPKILYRAAA	acetylation	[1, 2]
294	VASMAKEKLGVSCEV	ubiquitination	[3]
317	WDVDTICKSVIKTGR	ubiquitination	[3]
321	TICKSVIKTGRLLIS	ubiquitination	[3]

Reference

 [1] Lysine acetylation targets protein complexes and co-regulates major cellular functions.
 Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M.
 Science. 2009 Aug 14;325(5942):834-40. [PMID: 19608861]
 [2] Proteomic investigations of lysine acetylation identify diverse substrates of mitochondrial deacetylase sirt3.
 Sol EM, Wagner SA, Weinert BT, Kumar A, Kim HS, Deng CX, Choudhary C.
 PLoS One. 2012;7(12):e50545. [PMID: 23236377]
 [3] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961] 

Functional Description

 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). 

Sequence Annotation

 MOD_RES 241 241 N6-acetyllysine.  

Keyword

 3D-structure; Acetylation; Complete proteome; Direct protein sequencing; Disease mutation; Maple syrup urine disease; Mitochondrion; Oxidoreductase; Polymorphism; Reference proteome; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 392 AA 

Protein Sequence

Gene Ontology

 GO:0005947; C:mitochondrial alpha-ketoglutarate dehydrogenase complex; IMP:HGNC.
 GO:0003863; F:3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity; TAS:ProtInc.
 GO:0003826; F:alpha-ketoacid dehydrogenase activity; IEA:Compara.
 GO:0016831; F:carboxy-lyase activity; TAS:HGNC.
 GO:0009083; P:branched-chain amino acid catabolic process; IMP:HGNC.
 GO:0034641; P:cellular nitrogen compound metabolic process; TAS:Reactome.
 GO:0051591; P:response to cAMP; IEA:Compara.
 GO:0051384; P:response to glucocorticoid stimulus; IEA:Compara.
 GO:0007584; P:response to nutrient; IEA:Compara. 

Interpro

 IPR009014; Transketo_C/Pyr-ferredox_oxred.
 IPR015941; Transketolase-like_C.
 IPR005475; Transketolase-like_Pyr-bd.
 IPR005476; Transketolase_C. 

Pfam

 PF02779; Transket_pyr
 PF02780; Transketolase_C 

SMART

 SM00861; Transket_pyr 

PROSITE

  

PRINTS