[1]	NUCLEOTIDE SEQUENCE [MRNA]. STRAIN=C57BL/6J; TISSUE=Liver; PubMed=8761456 [NCBI, ExPASy, EBI, Israel, Japan] Costeas P.A., Chinsky J.M.; "Effects of insulin on the regulation of branched-chain alpha-keto acid dehydrogenase E1 alpha subunit gene expression."; Biochem. J. 318:85-92(1996).
[2]	PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-344, AND MASS SPECTROMETRY. TISSUE=Liver; DOI=10.1021/pr0604155; PubMed=17203969 [NCBI, ExPASy, EBI, Israel, Japan] Dai J., Jin W.-H., Sheng Q.-H., Shieh C.-H., Wu J.-R., Zeng R.; "Protein phosphorylation and expression profiling by Yin-yang multidimensional liquid chromatography (Yin-yang MDLC) mass spectrometry."; J. Proteome Res. 6:250-262(2007).
[3]	PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-334, AND MASS SPECTROMETRY. TISSUE=Liver; DOI=10.1074/mcp.M600218-MCP200; PubMed=17208939 [NCBI, ExPASy, EBI, Israel, Japan] Lee J., Xu Y., Chen Y., Sprung R., Kim S.C., Xie S., Zhao Y.; "Mitochondrial phosphoproteome revealed by an improved IMAC method and MS/MS/MS."; Mol. Cell. Proteomics 6:669-676(2007).
[4]	PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-344, AND MASS SPECTROMETRY. TISSUE=Liver; DOI=10.1073/pnas.0609836104; PubMed=17242355 [NCBI, ExPASy, EBI, Israel, Japan] Villen J., Beausoleil S.A., Gerber S.A., Gygi S.P.; "Large-scale phosphorylation analysis of mouse liver."; Proc. Natl. Acad. Sci. U.S.A. 104:1488-1493(2007).

Comments

FUNCTION: 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).
CATALYTIC ACTIVITY: 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO₂.
COFACTOR: Thiamine pyrophosphate.
SUBUNIT: Heterotetramer of alpha and beta chains (By similarity).
SUBCELLULAR LOCATION: Mitochondrion matrix.
MISCELLANEOUS: Bound potassium ions stabilize the protein structure (By similarity).
SIMILARITY: Belongs to the BCKDHA family.

Copyright

Copyrighted by the UniProt Consortium, see http://www.uniprot.org/terms. Distributed under the Creative Commons Attribution-NoDerivs License.

Cross-references

Sequence databases

EMBL

L47335; AAB38422.1; -; mRNA.

[EMBL / GenBank / DDBJ] [CoDingSequence]

PIR

S71881; S71881.

UniGene

Mm.25848

3D structure databases

HSSP

P12694; 1DTW. [HSSP ENTRY / PDB]

P50136; 48-442.

2D gel databases

P50136; -.

Organism-specific databases

MGI

MGI:107701; Bckdha.

Gene expression databases

GermOnline

ENSMUSG00000060376; Mus musculus.

Ontologies

GO:0005947;	Cellular component: mitochondrial alpha-ketoglutarate dehydrogenase complex (inferred from sequence or structural similarity from HGNC).
GO:0003826;	Molecular function: alpha-ketoacid dehydrogenase activity (inferred from sequence or structural similarity from HGNC).
GO:0016536;	Molecular function: cyclin-dependent protein kinase 5 activator regulator activity (inferred from direct assay from MGI).
GO:0009083;	Biological process: branched chain family amino acid catabolic process (inferred from sequence or structural similarity from HGNC).
GO:0045893;	Biological process: positive regulation of transcription, DNA-dependent (inferred from direct assay from MGI).
QuickGo view.

Family and domain databases

InterPro

IPR001017; DHase_E1.
Graphical view of domain structure.

Pfam

PF00676; E1_dh; 1.
Pfam graphical view of domain structure.

BLOCKS

P50136.

Genome annotation databases

Ensembl

ENSMUSG00000060376; Mus musculus. [Contig view]

Phylogenomic databases

HOVERGEN

P50136; -.

Other

SOURCE

Bckdha; Mus musculus.

ProtoNet

P50136.

UniRef

View cluster of proteins with at least 50% / 90% / 100% identity.

Keywords

Metal-binding; Mitochondrion; Oxidoreductase; Phosphoprotein; Potassium; Thiamine pyrophosphate; Transit peptide.

Features

Feature table viewer

Feature aligner

`Key`	`From To`	`Length`	`Description`	`FTId`
`TRANSIT`	`1 42`	`42`	`Mitochondrion (By similarity).`
`CHAIN`	`43 442`	`400`	`2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial.`	`PRO_0000020467`
`REGION`	`154 156`	`3`	`Thiamine pyrophosphate binding (By similarity).`
`METAL`	`203 203`		`Potassium (By similarity).`
`METAL`	`208 208`		`Potassium (By similarity).`
`METAL`	`209 209`		`Potassium (By similarity).`
`MOD_RES`	`334 334`		`Phosphoserine.`
`MOD_RES`	`342 342`		`Phosphotyrosine (By similarity).`
`MOD_RES`	`344 344`		`Phosphoserine.`

Sequence information

Length: 442 AA [This is the length of the unprocessed precursor]

Molecular weight: 50371 Da [This is the MW of the unprocessed precursor]

CRC64: 3388213D88BC7C92 [This is a checksum on the sequence]

        10         20         30         40         50         60 
MSAAKIWRPS RGLRQAALLL LGRSGVRGLA RSHPSRQQQQ QFPSLDDKPQ FPGASAEFVD 

        70         80         90        100        110        120 
KLEFIQPNVI SGIPIYRVMD RQGQIINPSE DPHLPQEEVL KFYRSMTLLN TMDRILYESQ 

       130        140        150        160        170        180 
REGRISFYMT NYGEEGTHVG SAAALERTDL VFGQYREAGV LMYRDYPLEL FMSQCYGNVN 

       190        200        210        220        230        240 
DPGKGRQMPV HYGCKERHFV TISSPLATQI PQAVGAAYAA KRANANRIVI CYFGEGAASE 

       250        260        270        280        290        300 
GDAHAGFNFA ATLECPIIFF CRNNGYAIST PTSEQYRGDG IAARGPGYGI KSIRVDGNDV 

       310        320        330        340        350        360 
FAVYNATKEA RRRAVAENQP FLIEAMTYRI GHHSTSDDSS AYRSVDEVNY WDKQDHPISR 

       370        380        390        400        410        420 
LRQYLLNQGW WDEEQEKAWR KQSRKKVMEA FEQAERKLKP NPSLLFSDVY QEMPAQLRRQ 

       430        440 
QESLARHLQT YGEHYPLDHF EK

P50136 in FASTA format

View entry in original UniProtKB/Swiss-Prot format
View entry in raw text format (no links)
Report form for errors/updates in this UniProtKB/Swiss-Prot entry

	BLAST submission on ExPASy/SIB or at NCBI (USA)		Sequence analysis tools: ProtParam, ProtScale, Compute pI/Mw, PeptideMass, PeptideCutter, Dotlet (Java)
	ScanProsite, MotifScan		Submit a homology modeling request to SWISS-MODEL
	NPSA Sequence analysis tools