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Protein

2-oxoisovalerate dehydrogenase subunit alpha

Gene

Bckdha

Organism
Mus musculus (Mouse)
Status
Unreviewed-Annotation score: -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).UniRule annotation

Catalytic activityi

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

Cofactori

thiamine diphosphateUniRule annotation

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductaseUniRule annotation
LigandThiamine pyrophosphateUniRule annotation

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 alphaUniRule annotation (EC:1.2.4.4UniRule annotation)
Alternative name(s):
Branched-chain alpha-keto acid dehydrogenase E1 component alpha chainUniRule annotation
Gene namesi
Name:BckdhaImported
ORF Names:mCG_7651Imported
OrganismiMus musculus (Mouse)Imported
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 7

Organism-specific databases

MGIiMGI:107701 Bckdha

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Expressioni

Gene expression databases

BgeeiENSMUSG00000060376

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini107 – 406E1_dhInterPro annotationAdd BLAST300

Sequence similaritiesi

Belongs to the BCKDHA family.UniRule annotation

Phylogenomic databases

eggNOGiKOG1182 Eukaryota
COG1071 LUCA
GeneTreeiENSGT00530000063174
HOGENOMiHOG000281337
HOVERGENiHBG002459
KOiK00166
OMAiNTMDKIL
OrthoDBiEOG091G073F
TreeFamiTF300863

Family and domain databases

InterProiView protein in InterPro
IPR034616 BCKDH_E1-a
IPR001017 DH_E1
IPR029061 THDP-binding
PANTHERiPTHR43380:SF1 PTHR43380:SF1, 1 hit
PfamiView protein in Pfam
PF00676 E1_dh, 1 hit
SUPFAMiSSF52518 SSF52518, 1 hit

Sequencei

Sequence statusi: Complete.

Q3U3J1-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAIVMSAAKI WRPSRGLRQA ALLLLGRSGV RGLARSHPSR QQQQQFPSLD
60 70 80 90 100
DKPQFPGASA EFVDKLEFIQ PNVISGIPIY RVMDRQGQII NPSEDPHLPQ
110 120 130 140 150
EEVLKFYRSM TLLNTMDRIL YESQRQGRIS FYMTNYGEEG THVGSAAALE
160 170 180 190 200
RTDLVFGQYR EAGVLMYRDY PLELFMSQCY GNVNDPGKGR QMPVHYGCKE
210 220 230 240 250
RHFVTISSPL ATQIPQAVGA AYAAKRANAN RIVICYFGEG AASEGDAHAG
260 270 280 290 300
FNFAATLECP IIFFCRNNGY AISTPTSEQY RGDGIAARGP GYGIMSIRVD
310 320 330 340 350
GNDVFAVYNA TKEARRRAVA ENQPFLIEAM TYRIGHHSTS DDSSAYRSVD
360 370 380 390 400
EVNYWDKQDH PISRLRQYLL NQGWWDEEQE KAWRKQSRKK VMEAFEQAER
410 420 430 440
KLKPNPSLLF SDVYQEMPAQ LRRQQESLAR HLQTYGEHYP LDHFDK
Length:446
Mass (Da):50,773
Last modified:October 11, 2005 - v1
Checksum:i110D5F3FA8A3108D
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC162614 Genomic DNA No translation available.
AK154734 mRNA Translation: BAE32795.1
CH466593 Genomic DNA Translation: EDL24248.1
RefSeqiNP_031559.3, NM_007533.5
UniGeneiMm.25848

Genome annotation databases

EnsembliENSMUST00000071329; ENSMUSP00000071292; ENSMUSG00000060376
GeneIDi12039
KEGGimmu:12039
UCSCiuc009fth.2 mouse

Similar proteinsi

Entry informationi

Entry nameiQ3U3J1_MOUSE
AccessioniPrimary (citable) accession number: Q3U3J1
Entry historyiIntegrated into UniProtKB/TrEMBL: October 11, 2005
Last sequence update: October 11, 2005
Last modified: May 23, 2018
This is version 115 of the entry and version 1 of the sequence. See complete history.
Entry statusiUnreviewed (UniProtKB/TrEMBL)

Miscellaneousi

Keywords - Technical termi

Complete proteome, Proteomics identificationCombined sources, Reference proteomeImported

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