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Protein

2-oxoisovalerate dehydrogenase subunit alpha

Gene

Bckdha

Organism
Mus musculus (Mouse)
Status
Unreviewed-Annotation score: -Experimental evidence at transcript 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

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
OrganismiMus musculus (Mouse)Imported
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus

Organism-specific databases

MGIiMGI:107701 Bckdha

Structurei

3D structure databases

ProteinModelPortaliQ99L69
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini103 – 402E1_dhInterPro annotationAdd BLAST300

Sequence similaritiesi

Belongs to the BCKDHA family.UniRule annotation

Phylogenomic databases

eggNOGiKOG1182 Eukaryota
COG1071 LUCA
HOVERGENiHBG002459

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.

Q99L69-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSAAKIWRPS RGLRQAALLL LGRSGVRGLA RSHPSRQQQQ QFPSLDDKPQ
60 70 80 90 100
FPGASAEFVD KLEFIQPNVI SGIPIYRVMD RQGQIINPSE DPHLPQEEVL
110 120 130 140 150
KFYRSMTLLN TMDRILYESQ RQGRISFYMT NYGEEGTHVG SAAALERTDL
160 170 180 190 200
VFGQYREAGV LMYRDYPLEL FMSQCYGNVN DPGKGRQMPV HYGCKERHFV
210 220 230 240 250
TISSPLATQI PQAVGAAYAA KRANANRIVI CYFGEGAASE GDAHAGFNFA
260 270 280 290 300
ATLECPIIFF CRNNGYAIST PTSEQYRGDG IAARGPGYGI MSIRVDGNDV
310 320 330 340 350
FAVYNATKEA RRRAVAENQP FLIEAMTYRI GHHSTSDDSS AYRSVDEVNY
360 370 380 390 400
WDKQDHPISR LRQYLLNQGW WDEEQEKAWR KQSRKKVMEA FEQAERKLKP
410 420 430 440
NPSLLFSDVY QEMPAQLRRQ QESLARHLQT YGEHYPLDHF DK
Length:442
Mass (Da):50,359
Last modified:June 1, 2001 - v1
Checksum:i1BF54B9C99E858FA
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC003787 mRNA Translation: AAH03787.1
UniGeneiMm.25848

Similar proteinsi

Entry informationi

Entry nameiQ99L69_MOUSE
AccessioniPrimary (citable) accession number: Q99L69
Entry historyiIntegrated into UniProtKB/TrEMBL: June 1, 2001
Last sequence update: June 1, 2001
Last modified: April 25, 2018
This is version 102 of the entry and version 1 of the sequence. See complete history.
Entry statusiUnreviewed (UniProtKB/TrEMBL)
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health