UniProtKB - Q99L69 (Q99L69_MOUSE)
Protein
2-oxoisovalerate dehydrogenase subunit alpha
Gene
Bckdha
Organism
Mus musculus (Mouse)
Status
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 annotationARBA annotation
Catalytic activityi
- 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]-(R)-N6-lipoyl-L-lysine + H+ = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]-(R)-N6-(S8-2-methylpropanoyldihydrolipoyl)-L-lysine + CO2UniRule annotationARBA annotationEC:1.2.4.4UniRule annotationARBA annotation
Cofactori
thiamine diphosphateUniRule annotationARBA annotation
GO - Molecular functioni
- 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity Source: UniProtKB-EC
- alpha-ketoacid dehydrogenase activity Source: InterPro
GO - Biological processi
- branched-chain amino acid catabolic process Source: InterPro
Keywordsi
Molecular function | OxidoreductaseUniRule annotationARBA annotation |
Ligand | Thiamine pyrophosphateUniRule annotationARBA 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 |
Organismi | Mus musculus (Mouse)Imported |
Taxonomic identifieri | 10090 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus |
Organism-specific databases
MGIi | MGI:107701, Bckdha |
Interactioni
Subunit structurei
Heterotetramer of alpha and beta chains.
ARBA annotationFamily & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 103 – 402 | E1_dhInterPro annotationAdd BLAST | 300 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 30 – 50 | DisorderedSequence analysisAdd BLAST | 21 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 31 – 46 | PolarSequence analysisAdd BLAST | 16 |
Sequence similaritiesi
Keywords - Domaini
Transit peptideARBA annotationFamily and domain databases
InterProi | View protein in InterPro IPR034616, BCKDH_E1-a IPR001017, DH_E1 IPR029061, THDP-binding |
PANTHERi | PTHR43380:SF1, PTHR43380:SF1, 1 hit |
Pfami | View protein in Pfam PF00676, E1_dh, 1 hit |
SUPFAMi | SSF52518, SSF52518, 1 hit |
i Sequence
Sequence statusi: Complete.
Q99L69-1 [UniParc]FASTAAdd to basket
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
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | BC003787 mRNA Translation: AAH03787.1 |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | BC003787 mRNA Translation: AAH03787.1 |
3D structure databases
ModBasei | Search... |
SWISS-MODEL-Workspacei | Submit a new modelling project... |
Proteomic databases
PRIDEi | Q99L69 |
Organism-specific databases
MGIi | MGI:107701, Bckdha |
Miscellaneous databases
ChiTaRSi | Bckdha, mouse |
SOURCEi | Search... |
Family and domain databases
InterProi | View protein in InterPro IPR034616, BCKDH_E1-a IPR001017, DH_E1 IPR029061, THDP-binding |
PANTHERi | PTHR43380:SF1, PTHR43380:SF1, 1 hit |
Pfami | View protein in Pfam PF00676, E1_dh, 1 hit |
SUPFAMi | SSF52518, SSF52518, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | Q99L69_MOUSE | |
Accessioni | Q99L69Primary (citable) accession number: Q99L69 | |
Entry historyi | Integrated into UniProtKB/TrEMBL: | June 1, 2001 |
Last sequence update: | June 1, 2001 | |
Last modified: | February 10, 2021 | |
This is version 113 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Unreviewed (UniProtKB/TrEMBL) |