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

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Reviewed, UniProtKB/Swiss-Prot O14874 (BCKD_HUMAN)

Last modified June 16, 2009. Version 84. History...

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Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Binary interactions · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

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Names and origin

Protein names

Recommended name:
    [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
    EC=2.7.11.4
Alternative name(s):
    Branched-chain alpha-ketoacid dehydrogenase kinase
      Short name=BCKD-kinase
      Short name=BCKDHKIN

Gene names

Name:

BCKDK

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

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Protein attributes

Sequence length	412 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level.

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General annotation (Comments)

Function	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.
Catalytic activity	ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] = ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate.
Subunit structure	Monomer By similarity.
Subcellular location	Mitochondrion matrix.
Tissue specificity	Ubiquitous.
Post-translational modification	Autophosphorylated By similarity.
Sequence similarities	Belongs to the PDK/BCKDK protein kinase family. Contains 1 histidine kinase domain.

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Ontologies

Keywords
Cellular component	Mitochondrion
Domain	Transit peptide
Ligand	ATP-binding Nucleotide-binding
Molecular function	Kinase Transferase
PTM	Phosphoprotein
Gene Ontology (GO)
Biological process	branched chain family amino acid catabolic process Traceable author statement. Source: HGNC peptidyl-histidine phosphorylation Inferred from electronic annotation. Source: InterPro signal transduction Inferred from electronic annotation. Source: InterPro
Cellular component	mitochondrial alpha-ketoglutarate dehydrogenase complex Traceable author statement. Source: HGNC
Molecular function	ATP binding Inferred from sequence or structural similarity. Source: HGNC [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity Inferred from electronic annotation. Source: EC protein binding Inferred from physical interaction. Source: IntAct protein serine/threonine kinase activity Inferred from sequence or structural similarity. Source: HGNC two-component sensor activity Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

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Binary interactions

With	Entry	#Exp.	IntAct
BCKDHA	P12694	1	EBI-1046765,EBI-1029053
CAB39	Q9Y376	1	EBI-1046765,EBI-306905
CAB39L	Q9H9S4	1	EBI-1046765,EBI-1047244
FAM98B	Q52LJ0	1	EBI-1046765,EBI-1043130
RTCD1	Q5VSU9	1	EBI-1046765,EBI-1045306

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Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Transit peptide

1 – 30

Mitochondrion By similarity

Chain

31 – 412

382

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

PRO_0000023452

Regions

Domain

159 – 404

246

Histidine kinase

Amino acid modifications

Modified residue

Phosphoserine Ref.4

Modified residue

Phosphoserine Ref.3

Modified residue

Phosphothreonine By similarity

Modified residue

Phosphoserine; by autocatalysis By similarity

Experimental info

Sequence conflict

218

V → F in AAB82714. Ref.1

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Sequences

Sequence

Length

Mass (Da)

Tools

O14874-1 [UniParc].

Last modified June 6, 2002. Version 2.
Checksum: AC97CF5D151FEFB4
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FASTA

412

46,360

        10         20         30         40         50         60 
MILASVLRSG PGGGLPLRPL LGPALALRAR STSATDTHHV EMARERSKTV TSFYNQSAID 

        70         80         90        100        110        120 
AAAEKPSVRL TPTMMLYAGR SQDGSHLLKS ARYLQQELPV RIAHRIKGFR CLPFIIGCNP 

       130        140        150        160        170        180 
TILHVHELYI RAFQKLTDFP PIKDQADEAQ YCQLVRQLLD DHKDVVTLLA EGLRESRKHI 

       190        200        210        220        230        240 
EDEKLVRYFL DKTLTSRLGI RMLATHHLAL HEDKPDFVGI ICTRLSPKKI IEKWVDFARR 

       250        260        270        280        290        300 
LCEHKYGNAP RVRINGHVAA RFPFIPMPLD YILPELLKNA MRATMESHLD TPYNVPDVVI 

       310        320        330        340        350        360 
TIANNDVDLI IRISDRGGGI AHKDLDRVMD YHFTTAEAST QDPRISPLFG HLDMHSGAQS 

       370        380        390        400        410 
GPMHGFGFGL PTSRAYAEYL GGSLQLQSLQ GIGTDVYLRL RHIDGREESF RI

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References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	Chuang J.C., Cox R.P., Chuang D.T. Submitted (SEP-1997) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[2]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Placenta.
[3]	"Combining protein-based IMAC, peptide-based IMAC, and MudPIT for efficient phosphoproteomic analysis." Cantin G.T., Yi W., Lu B., Park S.K., Xu T., Lee J.-D., Yates J.R. III J. Proteome Res. 7:1346-1351(2008) [PubMed: 18220336] [Abstract] Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-33, MASS SPECTROMETRY.
[4]	"Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle." Daub H., Olsen J.V., Bairlein M., Gnad F., Oppermann F.S., Korner R., Greff Z., Keri G., Stemmann O., Mann M. Mol. Cell 31:438-448(2008) [PubMed: 18691976] [Abstract] Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-31, MASS SPECTROMETRY.

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Cross-references

Sequence databases
	AF026548 mRNA. Translation: AAB82714.1. BC007363 mRNA. Translation: AAH07363.1.
IPI	IPI00298612.
RefSeq	NP_005872.2.
UniGene	Hs.513520
3D structure databases
HSSP	HSSP built from PDB template 1GKZ based on UniProtKB Q00972.
SMR	O14874. Positions 68-408.
ModBase	Search...
Protein-protein interaction databases
IntAct	O14874. 6 interactions.
PTM databases
PhosphoSite	O14874.
Proteomic databases
PRIDE	O14874.
Genome annotation databases
Ensembl	ENSG00000103507. Homo sapiens. [Contig view]
GeneID	10295.
Organism-specific databases
GeneCards	GC16P031024.
HGNC	HGNC:16902. BCKDK.
HPA	HPA017995.
PharmGKB	PA134899581.
GenAtlas	Search...
Phylogenomic databases
HOGENOM	O14874.
HOVERGEN	O14874.
OMA	O14874. RIKGFRS.
Enzyme and pathway databases
BRENDA	2.7.11.4. 247.
Gene expression databases
ArrayExpress	O14874.
Bgee	O14874.
CleanEx	HS_BCKDK.
GermOnline	ENSG00000103507. Homo sapiens.
Family and domain databases
InterPro	IPR003594. ATP_bd_ATPase. IPR018955. BCDHK/PDK_mit. IPR004358. Sig_transdc_His_kin-like_C. IPR005467. Sig_transdc_His_kinase_core. [Graphical view]
Gene3D	G3DSA:3.30.565.10. ATP_bd_ATPase. 1 hit.
Pfam	PF10436. BCDHK_Adom3. 1 hit. PF02518. HATPase_c. 1 hit. [Graphical view]
PRINTS	PR00344. BCTRLSENSOR.
SMART	SM00387. HATPase_c. 1 hit. [Graphical view]
PROSITE	PS50109. HIS_KIN. 1 hit. [Graphical view]
ProtoNet	Search...
Other Resources
NextBio	39014.

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Entry information

Entry name

BCKD_HUMAN

Accession

Primary (citable) accession number: O14874
Secondary accession number(s): Q96IN5

Entry history

Integrated into UniProtKB/Swiss-Prot:	July 15, 1998
Last sequence update:	June 6, 2002
Last modified:	June 16, 2009
This is version 84 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)

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Relevant documents

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Human chromosome 16: entries, gene names and cross-references to MIM

Human and mouse protein kinases

Human and mouse protein kinases: classification and index

SIMILARITY comments

Index of protein domains and families

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Binary interactions

With

Entry

#Exp.

IntAct

Notes

Sequence annotation (Features)

Molecule processing

Regions

Amino acid modifications

Experimental info

Sequences

References

Cross-references

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Proteomic databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Gene expression databases

Family and domain databases

Other Resources

Entry information

Relevant documents