UniProtKB - P50136 (ODBA_MOUSE)
Protein
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
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).
Miscellaneous
Bound potassium ions stabilize the protein structure.By similarity
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 + CO2EC:1.2.4.4
Cofactori
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 203 | PotassiumBy similarity | 1 | |
Metal bindingi | 208 | PotassiumBy similarity | 1 | |
Metal bindingi | 209 | PotassiumBy similarity | 1 |
GO - Molecular functioni
- 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity Source: UniProtKB-EC
- alpha-ketoacid dehydrogenase activity Source: HGNC-UCL
- metal ion binding Source: UniProtKB-KW
- protein-containing complex binding Source: MGI
GO - Biological processi
- branched-chain amino acid catabolic process Source: HGNC-UCL
Keywordsi
Molecular function | Oxidoreductase |
Ligand | Metal-binding, Potassium, Thiamine pyrophosphate |
Enzyme and pathway databases
Reactomei | R-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 alpha, mitochondrial (EC:1.2.4.4)Alternative name(s): Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain Short name: BCKDE1A Short name: BCKDH E1-alpha |
Gene namesi | Name:Bckdha |
Organismi | Mus musculus (Mouse) |
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 |
Subcellular locationi
Mitochondrion
Mitochondrion
- mitochondrial alpha-ketoglutarate dehydrogenase complex Source: HGNC-UCL
- mitochondrion Source: MGI
Keywords - Cellular componenti
MitochondrionPTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 42 | MitochondrionBy similarityAdd BLAST | 42 | |
ChainiPRO_0000020467 | 43 – 442 | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrialAdd BLAST | 400 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 334 | PhosphoserineCombined sources | 1 | |
Modified residuei | 335 | PhosphothreonineCombined sources | 1 | |
Modified residuei | 336 | PhosphoserineCombined sources | 1 | |
Modified residuei | 344 | PhosphoserineCombined sources | 1 | |
Modified residuei | 353 | N6-acetyllysine; alternateCombined sources | 1 | |
Modified residuei | 353 | N6-succinyllysine; alternateCombined sources | 1 | |
Modified residuei | 377 | N6-succinyllysineCombined sources | 1 |
Keywords - PTMi
Acetylation, PhosphoproteinProteomic databases
EPDi | P50136 |
jPOSTi | P50136 |
MaxQBi | P50136 |
PaxDbi | P50136 |
PeptideAtlasi | P50136 |
PRIDEi | P50136 |
2D gel databases
SWISS-2DPAGEi | P50136 |
PTM databases
iPTMneti | P50136 |
PhosphoSitePlusi | P50136 |
Interactioni
Subunit structurei
Heterotetramer of alpha and beta chains.
By similarityProtein-protein interaction databases
IntActi | P50136, 5 interactors |
MINTi | P50136 |
STRINGi | 10090.ENSMUSP00000071292 |
Miscellaneous databases
RNActi | P50136, protein |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 154 – 156 | Thiamine pyrophosphate bindingBy similarity | 3 |
Sequence similaritiesi
Belongs to the BCKDHA family.Curated
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG1182, Eukaryota |
InParanoidi | P50136 |
PhylomeDBi | P50136 |
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.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All
P50136-1 [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 REGRISFYMT 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 KSIRVDGNDV
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 EK
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketQ3U3J1 | Q3U3J1_MOUSE | 2-oxoisovalerate dehydrogenase subu... | Bckdha | 446 | Annotation score: | ||
A0A0U1RPK5 | A0A0U1RPK5_MOUSE | 2-oxoisovalerate dehydrogenase subu... | Bckdha | 99 | Annotation score: |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | L47335 mRNA Translation: AAB38422.1 |
PIRi | S71881 |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | L47335 mRNA Translation: AAB38422.1 |
PIRi | S71881 |
3D structure databases
SMRi | P50136 |
ModBasei | Search... |
Protein-protein interaction databases
IntActi | P50136, 5 interactors |
MINTi | P50136 |
STRINGi | 10090.ENSMUSP00000071292 |
PTM databases
iPTMneti | P50136 |
PhosphoSitePlusi | P50136 |
2D gel databases
SWISS-2DPAGEi | P50136 |
Proteomic databases
EPDi | P50136 |
jPOSTi | P50136 |
MaxQBi | P50136 |
PaxDbi | P50136 |
PeptideAtlasi | P50136 |
PRIDEi | P50136 |
Organism-specific databases
MGIi | MGI:107701, Bckdha |
Phylogenomic databases
eggNOGi | KOG1182, Eukaryota |
InParanoidi | P50136 |
PhylomeDBi | P50136 |
Enzyme and pathway databases
Reactomei | R-MMU-389661, Glyoxylate metabolism and glycine degradation R-MMU-70895, Branched-chain amino acid catabolism |
Miscellaneous databases
ChiTaRSi | Bckdha, mouse |
PROi | PR:P50136 |
RNActi | P50136, protein |
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 | ODBA_MOUSE | |
Accessioni | P50136Primary (citable) accession number: P50136 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | October 1, 1996 |
Last sequence update: | October 1, 1996 | |
Last modified: | August 12, 2020 | |
This is version 137 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families