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Protein

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

Gene

Bckdhb

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

Functioni

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 activityi

3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2.

GO - Molecular functioni

GO - Biological processi

  • branched-chain amino acid catabolic process Source: HGNC
  • cellular amino acid catabolic process Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Enzyme and pathway databases

ReactomeiR-MMU-389661. Glyoxylate metabolism and glycine degradation.
R-MMU-70895. Branched-chain amino acid catabolism.

Names & Taxonomyi

Protein namesi
Recommended 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:
BCKDE1B
Short name:
BCKDH E1-beta
Gene namesi
Name:Bckdhb
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 9

Organism-specific databases

MGIiMGI:88137. Bckdhb.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 4848MitochondrionSequence analysisAdd
BLAST
Chaini49 – 3903422-oxoisovalerate dehydrogenase subunit beta, mitochondrialPRO_0000312367Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei230 – 2301N6-acetyllysineCombined sources
Modified residuei239 – 2391N6-acetyllysineCombined sources

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ6P3A8.
MaxQBiQ6P3A8.
PaxDbiQ6P3A8.
PRIDEiQ6P3A8.

PTM databases

iPTMnetiQ6P3A8.
SwissPalmiQ6P3A8.

Expressioni

Gene expression databases

BgeeiQ6P3A8.
GenevisibleiQ6P3A8. MM.

Interactioni

Subunit structurei

Heterotetramer of 2 alpha and 2 beta chains.By similarity

Protein-protein interaction databases

IntActiQ6P3A8. 1 interaction.
MINTiMINT-1860867.
STRINGi10090.ENSMUSP00000034801.

Structurei

3D structure databases

ProteinModelPortaliQ6P3A8.
SMRiQ6P3A8. Positions 62-390.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0525. Eukaryota.
COG0022. LUCA.
GeneTreeiENSGT00530000063423.
HOGENOMiHOG000281451.
HOVERGENiHBG108210.
InParanoidiQ6P3A8.
KOiK00167.
OMAiLAWGAQM.
OrthoDBiEOG79SDXF.
PhylomeDBiQ6P3A8.
TreeFamiTF105947.

Family and domain databases

Gene3Di3.40.50.920. 1 hit.
3.40.50.970. 1 hit.
InterProiIPR029061. THDP-binding.
IPR009014. Transketo_C/PFOR_II.
IPR005475. Transketolase-like_Pyr-bd.
IPR033248. Transketolase_C.
IPR033247. Transketolase_fam.
[Graphical view]
PANTHERiPTHR11624. PTHR11624. 1 hit.
PfamiPF02779. Transket_pyr. 1 hit.
PF02780. Transketolase_C. 1 hit.
[Graphical view]
SMARTiSM00861. Transket_pyr. 1 hit.
[Graphical view]
SUPFAMiSSF52518. SSF52518. 1 hit.
SSF52922. SSF52922. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q6P3A8-1) [UniParc]FASTAAdd to basket

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.

« Hide

        10         20         30         40         50
MAAVAARAGG LLWLRAAGAE RRRCGLRCAA LVQGFLQPGG EDTAQKRRVA
60 70 80 90 100
HFTFHPDPES LQYGQTQKMN LFQSITSALD NSLAKDPTAV IFGEDVAFGG
110 120 130 140 150
VFRCTVGLRD KYGKDRVFNT PLCEQGIVGF GIGIAVTGAT AIAEIQFADY
160 170 180 190 200
IFPAFDQIVN EAAKYRYRSG DLFNCGSLTI RAPWGCVGHG ALYHSQSPEA
210 220 230 240 250
FFAHCPGIKV VIPRSPFQAK GLLLSCIEDK NPCIFFEPKI LYRAAVEQVP
260 270 280 290 300
VEPYKIPLSQ AEVIQEGSDV TLVAWGTQVH VIREVASMAQ EKLGVSCEVI
310 320 330 340 350
DLRTIVPWDV DTVCKSVIKT GRLLISHEAP LTGGFASEIS STVQEECFLN
360 370 380 390
LEAPISRVCG YDTPFPHIFE PFYIPDKWKC YDALRKMINY
Length:390
Mass (Da):42,880
Last modified:December 4, 2007 - v2
Checksum:i507E04F01BCCD298
GO
Isoform 2 (identifier: Q6P3A8-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-68: Missing.

Note: No experimental confirmation available.
Show »
Length:322
Mass (Da):35,505
Checksum:i07349BDF80A03758
GO

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 6868Missing in isoform 2. 1 PublicationVSP_029841Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L16992 mRNA. No translation available.
BC064099 mRNA. Translation: AAH64099.1.
CCDSiCCDS23378.1. [Q6P3A8-2]
CCDS81045.1. [Q6P3A8-1]
RefSeqiNP_001292864.1. NM_001305935.1. [Q6P3A8-1]
NP_954665.1. NM_199195.1. [Q6P3A8-2]
UniGeneiMm.12819.

Genome annotation databases

EnsembliENSMUST00000034801; ENSMUSP00000034801; ENSMUSG00000032263. [Q6P3A8-2]
ENSMUST00000190166; ENSMUSP00000140598; ENSMUSG00000032263. [Q6P3A8-2]
ENSMUST00000190637; ENSMUSP00000139684; ENSMUSG00000032263. [Q6P3A8-1]
GeneIDi12040.
KEGGimmu:12040.
UCSCiuc009qwr.1. mouse. [Q6P3A8-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L16992 mRNA. No translation available.
BC064099 mRNA. Translation: AAH64099.1.
CCDSiCCDS23378.1. [Q6P3A8-2]
CCDS81045.1. [Q6P3A8-1]
RefSeqiNP_001292864.1. NM_001305935.1. [Q6P3A8-1]
NP_954665.1. NM_199195.1. [Q6P3A8-2]
UniGeneiMm.12819.

3D structure databases

ProteinModelPortaliQ6P3A8.
SMRiQ6P3A8. Positions 62-390.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ6P3A8. 1 interaction.
MINTiMINT-1860867.
STRINGi10090.ENSMUSP00000034801.

PTM databases

iPTMnetiQ6P3A8.
SwissPalmiQ6P3A8.

Proteomic databases

EPDiQ6P3A8.
MaxQBiQ6P3A8.
PaxDbiQ6P3A8.
PRIDEiQ6P3A8.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000034801; ENSMUSP00000034801; ENSMUSG00000032263. [Q6P3A8-2]
ENSMUST00000190166; ENSMUSP00000140598; ENSMUSG00000032263. [Q6P3A8-2]
ENSMUST00000190637; ENSMUSP00000139684; ENSMUSG00000032263. [Q6P3A8-1]
GeneIDi12040.
KEGGimmu:12040.
UCSCiuc009qwr.1. mouse. [Q6P3A8-1]

Organism-specific databases

CTDi594.
MGIiMGI:88137. Bckdhb.

Phylogenomic databases

eggNOGiKOG0525. Eukaryota.
COG0022. LUCA.
GeneTreeiENSGT00530000063423.
HOGENOMiHOG000281451.
HOVERGENiHBG108210.
InParanoidiQ6P3A8.
KOiK00167.
OMAiLAWGAQM.
OrthoDBiEOG79SDXF.
PhylomeDBiQ6P3A8.
TreeFamiTF105947.

Enzyme and pathway databases

ReactomeiR-MMU-389661. Glyoxylate metabolism and glycine degradation.
R-MMU-70895. Branched-chain amino acid catabolism.

Miscellaneous databases

PROiQ6P3A8.
SOURCEiSearch...

Gene expression databases

BgeeiQ6P3A8.
GenevisibleiQ6P3A8. MM.

Family and domain databases

Gene3Di3.40.50.920. 1 hit.
3.40.50.970. 1 hit.
InterProiIPR029061. THDP-binding.
IPR009014. Transketo_C/PFOR_II.
IPR005475. Transketolase-like_Pyr-bd.
IPR033248. Transketolase_C.
IPR033247. Transketolase_fam.
[Graphical view]
PANTHERiPTHR11624. PTHR11624. 1 hit.
PfamiPF02779. Transket_pyr. 1 hit.
PF02780. Transketolase_C. 1 hit.
[Graphical view]
SMARTiSM00861. Transket_pyr. 1 hit.
[Graphical view]
SUPFAMiSSF52518. SSF52518. 1 hit.
SSF52922. SSF52922. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  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] [Europe PMC] [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] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
    Strain: C57BL/6J.
    Tissue: Mammary gland.
  3. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Brain, Brown adipose tissue, Heart, Kidney, Liver, Lung, Pancreas, Spleen and Testis.
  4. "Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways."
    Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.
    Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-230 AND LYS-239, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiODBB_MOUSE
AccessioniPrimary (citable) accession number: Q6P3A8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 4, 2007
Last sequence update: December 4, 2007
Last modified: June 8, 2016
This is version 96 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.