UniProtKB - Q3U3J1 (Q3U3J1_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 |
Proteomesi |
|
Organism-specific databases
MGIi | MGI:107701, Bckdha |
Expressioni
Gene expression databases
Bgeei | ENSMUSG00000060376, Expressed in brown adipose tissue and 307 other tissues |
Interactioni
Subunit structurei
Heterotetramer of alpha and beta chains.
ARBA annotationFamily & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 107 – 406 | E1_dhInterPro annotationAdd BLAST | 300 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 34 – 54 | DisorderedSequence analysisAdd BLAST | 21 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 35 – 50 | PolarSequence analysisAdd BLAST | 16 |
Sequence similaritiesi
Keywords - Domaini
Transit peptideARBA annotationPhylogenomic databases
GeneTreei | ENSGT00530000063174 |
HOGENOMi | CLU_029393_1_2_1 |
OMAi | NTMDKIL |
OrthoDBi | 623111at2759 |
TreeFami | TF300863 |
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 |
(1+)i Sequence
Sequence statusi: Complete.
This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All
Q3U3J1-1 [UniParc]FASTAAdd to basket
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
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketP50136 | ODBA_MOUSE | 2-oxoisovalerate dehydrogenase subu... | Bckdha | 442 | Annotation score: | ||
A0A0U1RPK5 | A0A0U1RPK5_MOUSE | 2-oxoisovalerate dehydrogenase subu... | Bckdha | 99 | Annotation score: |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AC162614 Genomic DNA No translation available. AK154734 mRNA Translation: BAE32795.1 |
RefSeqi | NP_031559.3, NM_007533.5 |
Genome annotation databases
Ensembli | ENSMUST00000071329; ENSMUSP00000071292; ENSMUSG00000060376 |
GeneIDi | 12039 |
KEGGi | mmu:12039 |
UCSCi | uc009fth.2, mouse |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AC162614 Genomic DNA No translation available. AK154734 mRNA Translation: BAE32795.1 |
RefSeqi | NP_031559.3, NM_007533.5 |
3D structure databases
SMRi | Q3U3J1 |
ModBasei | Search... |
PTM databases
SwissPalmi | Q3U3J1 |
Proteomic databases
PRIDEi | Q3U3J1 |
Genome annotation databases
Ensembli | ENSMUST00000071329; ENSMUSP00000071292; ENSMUSG00000060376 |
GeneIDi | 12039 |
KEGGi | mmu:12039 |
UCSCi | uc009fth.2, mouse |
Organism-specific databases
CTDi | 593 |
MGIi | MGI:107701, Bckdha |
Phylogenomic databases
GeneTreei | ENSGT00530000063174 |
HOGENOMi | CLU_029393_1_2_1 |
OMAi | NTMDKIL |
OrthoDBi | 623111at2759 |
TreeFami | TF300863 |
Miscellaneous databases
BioGRID-ORCSi | 12039, 2 hits in 17 CRISPR screens |
ChiTaRSi | Bckdha, mouse |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSMUSG00000060376, Expressed in brown adipose tissue and 307 other tissues |
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 | Q3U3J1_MOUSE | |
Accessioni | Q3U3J1Primary (citable) accession number: Q3U3J1 | |
Entry historyi | Integrated into UniProtKB/TrEMBL: | October 11, 2005 |
Last sequence update: | October 11, 2005 | |
Last modified: | February 10, 2021 | |
This is version 130 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Unreviewed (UniProtKB/TrEMBL) |