UniProt Accession

 BCKD_HUMAN; O14874; Q6FGL4; Q96IN5 

Genbank Protein ID

 AF026548; CR542093; CH471192; BC007363 

Genbank Nucleotide ID

 AAB82714.1; CAG46890.1; EAW52161.1; AAH07363.1 

Protein Name

 [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial 

Protein Synonyms/Alias

 Branched-chain alpha-ketoacid dehydrogenase kinase; BCKD-kinase; BCKDHKIN 

Gene Name

 BCKDK 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
48	EMARERSKTVTSFYN	ubiquitination	[1, 2]
184	RKHIEDEKLVRYFLD	acetylation	[3]
192	LVRYFLDKTLTSRLG	acetylation	[3]
233	SPKKIIEKWVDFARR	acetylation	[3]
245	ARRLCEHKYGNAPRV	acetylation	[3]

Reference

 [1] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [2] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789]
 [3] 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] 

Functional Description

 Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex (By similarity). 

Sequence Annotation

 DOMAIN 159 404 Histidine kinase.
 MOD_RES 31 31 Phosphoserine (By similarity).
 MOD_RES 35 35 Phosphothreonine (By similarity).
 MOD_RES 52 52 Phosphoserine; by autocatalysis (By
 MOD_RES 192 192 N6-acetyllysine.
 MOD_RES 233 233 N6-acetyllysine.  

Keyword

 Acetylation; ATP-binding; Complete proteome; Disease mutation; Kinase; Mitochondrion; Nucleotide-binding; Phosphoprotein; Reference proteome; Serine/threonine-protein kinase; Transferase; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 412 AA 

Protein Sequence

Gene Ontology

 GO:0005947; C:mitochondrial alpha-ketoglutarate dehydrogenase complex; ISS:HGNC.
 GO:0047323; F:[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity; IEA:EC.
 GO:0005524; F:ATP binding; ISS:HGNC.
 GO:0004674; F:protein serine/threonine kinase activity; ISS:HGNC.
 GO:0009083; P:branched-chain amino acid catabolic process; ISS:HGNC. 

Interpro

 IPR018955; BCDHK/PDK_N.
 IPR003594; HATPase_ATP-bd.
 IPR004358; Sig_transdc_His_kin-like_C.
 IPR005467; Sig_transdc_His_kinase_core. 

Pfam

 PF10436; BCDHK_Adom3
 PF02518; HATPase_c 

SMART

 SM00387; HATPase_c 

PROSITE

 PS50109; HIS_KIN 

PRINTS

 PR00344; BCTRLSENSOR.