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UniProtKB - A5A6H9 (ODBA_PANTR)
Protein
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Gene
BCKDHA
Organism
Pan troglodytes (Chimpanzee)
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
- (R)-N6-lipoyl-L-lysyl-[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] + 3-methyl-2-oxobutanoate + H+ = (R)-N6-(S8-2-methylpropanoyldihydrolipoyl)-L-lysyl-[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] + CO2EC:1.2.4.4
Cofactori
thiamine diphosphateBy similarity
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 206 | PotassiumBy similarity | 1 | |
Metal bindingi | 211 | PotassiumBy similarity | 1 | |
Metal bindingi | 212 | PotassiumBy similarity | 1 |
GO - Molecular functioni
- 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity Source: UniProtKB-EC
- metal ion binding Source: UniProtKB-KW
GO - Biological processi
- branched-chain amino acid catabolic process Source: InterPro
Keywordsi
Molecular function | Oxidoreductase |
Ligand | Metal-binding, Potassium, Thiamine pyrophosphate |
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 | Pan troglodytes (Chimpanzee) |
Taxonomic identifieri | 9598 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Pan |
Proteomesi |
|
Subcellular locationi
Mitochondrion
- Mitochondrion matrix By similarity
Mitochondrion
- mitochondrial matrix Source: UniProtKB-SubCell
Keywords - Cellular componenti
MitochondrionPTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 45 | MitochondrionBy similarityAdd BLAST | 45 | |
ChainiPRO_0000296643 | 46 – 445 | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrialAdd BLAST | 400 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 337 | PhosphoserineBy similarity | 1 | |
Modified residuei | 338 | PhosphothreonineBy similarity | 1 | |
Modified residuei | 339 | PhosphoserineBy similarity | 1 | |
Modified residuei | 347 | PhosphoserineBy similarity | 1 | |
Modified residuei | 356 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 356 | N6-succinyllysine; alternateBy similarity | 1 | |
Modified residuei | 380 | N6-succinyllysineBy similarity | 1 |
Keywords - PTMi
Acetylation, PhosphoproteinProteomic databases
PaxDbi | A5A6H9 |
Interactioni
Subunit structurei
Heterotetramer of alpha and beta chains.
By similarityProtein-protein interaction databases
STRINGi | 9598.ENSPTRP00000018933 |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 33 – 54 | DisorderedSequence analysisAdd BLAST | 22 | |
Regioni | 157 – 159 | Thiamine pyrophosphate bindingBy similarity | 3 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 37 – 51 | Polar residuesSequence analysisAdd BLAST | 15 |
Sequence similaritiesi
Belongs to the BCKDHA family.Curated
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG1182, Eukaryota |
InParanoidi | A5A6H9 |
OrthoDBi | 623111at2759 |
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 |
i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
A5A6H9-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MAVAIAAARV WRLNRGLSQA ALLLLRRPGA RGLARSHPRR QQQQFSSLDD
60 70 80 90 100
KPQFPGASAE FIDKLEFIQP NVISGIPIYR VMDRQGQIIN PSEDPHLPKE
110 120 130 140 150
KVLKLYKSMT LLNTMDRILY ESQRQGRISF YMTNYGEEGT HVGSAAALDN
160 170 180 190 200
TDLVFGQYRE AGVLMYRDYP LELFMAQCYG NISDLGKGRQ MPVHYGCKER
210 220 230 240 250
HFVTISSPLA TQIPQAVGAA YAAKRANANR VVICYFGEGA ASEGDAHAGF
260 270 280 290 300
NFAATLECPI IFFCRNNGYA ISTPTSEQYR GDGIAARGPG YGIMSIRVDG
310 320 330 340 350
NDVFAVYNAT KEARRRAVAE NQPFLIEAMT YRIGHHSTSD DSSAYRSVDE
360 370 380 390 400
VNYWDKQDHP VSRLRHYLLS QGWWDEEQEK AWRKQSRKKV MEAFEQAERK
410 420 430 440
PKPNPNLLFS DVYQEMPAQL RKQQESLARH LQTYGEHYPL DHFDK
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB222107 mRNA Translation: BAF62352.1 |
RefSeqi | NP_001092034.1, NM_001098564.1 |
Genome annotation databases
GeneIDi | 468889 |
KEGGi | ptr:468889 |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB222107 mRNA Translation: BAF62352.1 |
RefSeqi | NP_001092034.1, NM_001098564.1 |
3D structure databases
AlphaFoldDBi | A5A6H9 |
SMRi | A5A6H9 |
ModBasei | Search... |
Protein-protein interaction databases
STRINGi | 9598.ENSPTRP00000018933 |
Proteomic databases
PaxDbi | A5A6H9 |
Genome annotation databases
GeneIDi | 468889 |
KEGGi | ptr:468889 |
Organism-specific databases
CTDi | 593 |
Phylogenomic databases
eggNOGi | KOG1182, Eukaryota |
InParanoidi | A5A6H9 |
OrthoDBi | 623111at2759 |
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 |
MobiDBi | Search... |
Entry informationi
Entry namei | ODBA_PANTR | |
Accessioni | A5A6H9Primary (citable) accession number: A5A6H9 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | July 24, 2007 |
Last sequence update: | June 12, 2007 | |
Last modified: | May 25, 2022 | |
This is version 72 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
- SIMILARITY comments
Index of protein domains and families