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Reviewed, UniProtKB/Swiss-Prot Q6P3A8 (ODBB_MOUSE)

Last modified February 9, 2010. Version 47. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (1) | Third-party data | Customize display text xml rdf/xml gff fasta
Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Alternative products · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

Names and origin

Protein namesRecommended name:
    2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
    EC=1.2.4.4
Alternative name(s):
    Branched-chain alpha-keto acid dehydrogenase E1 component beta chain
      Short name=BCKDH E1-beta
      Short name=BCKDE1B
Gene names
Name: Bckdhb
OrganismMus musculus (Mouse)
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMus

Protein attributes

Sequence length390 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at transcript level.

General annotation (Comments)

Function

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. 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.

Subunit structure

Heterodimer of an alpha and a beta chain.

Subcellular location

Mitochondrion matrix.

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q6P3A8-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.
Isoform 2 (identifier: Q6P3A8-2)

The sequence of this isoform differs from the canonical sequence as follows:
     1-68: Missing.
Note: No experimental confirmation available.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 4848Mitochondrion Potential
Chain49 – 3903422-oxoisovalerate dehydrogenase subunit beta, mitochondrial
PRO_0000312367

Amino acid modifications

Modified residue2391N6-acetyllysine By similarity

Natural variations

Alternative sequence1 – 6868Missing in isoform 2.
VSP_029841

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified December 4, 2007. Version 2.
Checksum: 507E04F01BCCD298

FASTA39042,880
        10         20         30         40         50         60 
MAAVAARAGG LLWLRAAGAE RRRCGLRCAA LVQGFLQPGG EDTAQKRRVA HFTFHPDPES 

        70         80         90        100        110        120 
LQYGQTQKMN LFQSITSALD NSLAKDPTAV IFGEDVAFGG VFRCTVGLRD KYGKDRVFNT 

       130        140        150        160        170        180 
PLCEQGIVGF GIGIAVTGAT AIAEIQFADY IFPAFDQIVN EAAKYRYRSG DLFNCGSLTI 

       190        200        210        220        230        240 
RAPWGCVGHG ALYHSQSPEA FFAHCPGIKV VIPRSPFQAK GLLLSCIEDK NPCIFFEPKI 

       250        260        270        280        290        300 
LYRAAVEQVP VEPYKIPLSQ AEVIQEGSDV TLVAWGTQVH VIREVASMAQ EKLGVSCEVI 

       310        320        330        340        350        360 
DLRTIVPWDV DTVCKSVIKT GRLLISHEAP LTGGFASEIS STVQEECFLN LEAPISRVCG 

       370        380        390 
YDTPFPHIFE PFYIPDKWKC YDALRKMINY 

« Hide

Isoform 2.

Checksum: 07349BDF80A03758
Show »

FASTA32235,505

References

« Hide 'large scale' references
[1]"Molecular cloning and analysis of the expression of the E1 beta subunit of branched chain alpha-ketoacid dehydrogenase in mice."
Chinsky J.M., Costeas P.A.
Biochim. Biophys. Acta 1216:499-503(1993) [PubMed: 8268236] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
[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] (ISOFORM 2).
Strain: C57BL/6J.
Tissue: Mammary gland.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
L16992 mRNA. No translation available.
BC064099 mRNA. Translation: AAH64099.1.
IPIIPI00115302.
IPI00661338.
RefSeqNP_954665.1.
XP_001481012.1.
UniGeneMm.12819

3D structure databases

HSSPHSSP built from PDB template 2BFF based on UniProtKB P21953.
SMRQ6P3A8. Positions 65-390.
ModBaseSearch...

Protein-protein interaction databases

STRINGQ6P3A8.

Proteomic databases

PRIDEQ6P3A8.

Genome annotation databases

EnsemblENSMUST00000034801; ENSMUSP00000034801; ENSMUSG00000032263; Mus musculus. [Genome view]
ENSMUST00000116537; ENSMUSP00000112236; ENSMUSG00000032263; Mus musculus. [Genome view]
GeneID100048676.
12040.
KEGGmmu:100048676.
mmu:12040.
UCSCuc009qws.1. mouse.

Organism-specific databases

CTD12040.
MGIMGI:88137. Bckdhb.

Phylogenomic databases

eggNOGroNOG05452.
HOGENOMHBG753264.
HOVERGENQ6P3A8.
InParanoidQ6P3A8.
OMALIPWDKE.
OrthoDBEOG91G5PH.

Enzyme and pathway databases

BRENDA1.2.4.4. 244.

Gene expression databases

ArrayExpressQ6P3A8.
BgeeQ6P3A8.
GenevestigatorQ6P3A8.

Family and domain databases

InterProIPR009014. Transketo_C/Pyr-ferredox_oxred.
IPR015941. Transketolase-like_C.
IPR005475. Transketolase-like_Pyr-bd.
IPR005476. Transketolase_C.
[Graphical view]
Gene3DG3DSA:3.40.50.920. Transketo_C_like. 2 hits.
PfamPF02779. Transket_pyr. 1 hit.
PF02780. Transketolase_C. 1 hit.
[Graphical view]
SMARTSM00861. Transket_pyr. 1 hit.
[Graphical view]
ProtoNetSearch...

Other Resources

NextBio462083.
SOURCESearch...

Entry information

Entry nameODBB_MOUSE
AccessionPrimary (citable) accession number: Q6P3A8
Entry history
Integrated into UniProtKB/Swiss-Prot: December 4, 2007
Last sequence update: December 4, 2007
Last modified: February 9, 2010
This is version 47 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation projectHPI (Human Proteome Initiative)

Relevant documents

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot

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