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Protein

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Gene

Bckdha

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein 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).

Miscellaneous

Bound potassium ions stabilize the protein structure.By similarity

Catalytic activityi

3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2.

Cofactori

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi202PotassiumBy similarity1
Metal bindingi207PotassiumBy similarity1
Metal bindingi208PotassiumBy similarity1

GO - Molecular functioni

GO - Biological processi

  • branched-chain amino acid catabolic process Source: HGNC
  • response to cAMP Source: RGD
  • response to glucocorticoid Source: RGD
  • response to nutrient Source: RGD

Keywordsi

Molecular functionOxidoreductase
LigandMetal-binding, Potassium, Thiamine pyrophosphate

Enzyme and pathway databases

SABIO-RKiP11960.

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
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi2196. Bckdha.

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei‹1 – 41MitochondrionBy similarityAdd BLAST›41
ChainiPRO_000002046842 – 4412-oxoisovalerate dehydrogenase subunit alpha, mitochondrialAdd BLAST400

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei333PhosphoserineCombined sources1
Modified residuei334PhosphothreonineBy similarity1
Modified residuei335PhosphoserineBy similarity1
Modified residuei343PhosphoserineCombined sources1
Modified residuei352N6-acetyllysine; alternateBy similarity1
Modified residuei352N6-succinyllysine; alternateBy similarity1
Modified residuei376N6-succinyllysineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

PaxDbiP11960.
PRIDEiP11960.

PTM databases

iPTMnetiP11960.
PhosphoSitePlusiP11960.

Interactioni

Subunit structurei

Heterotetramer of alpha and beta chains.By similarity

GO - Molecular functioni

  • protein complex binding Source: RGD

Protein-protein interaction databases

IntActiP11960. 1 interactor.
STRINGi10116.ENSRNOP00000027995.

Structurei

3D structure databases

ProteinModelPortaliP11960.
SMRiP11960.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni153 – 155Thiamine pyrophosphate bindingBy similarity3

Sequence similaritiesi

Belongs to the BCKDHA family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1182. Eukaryota.
COG1071. LUCA.
HOVERGENiHBG002459.
InParanoidiP11960.
KOiK00166.
PhylomeDBiP11960.

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: Fragment.

Sequence processingi: The displayed sequence is further processed into a mature form.

P11960-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
SAAKIWRPSR GLRQAALLLL GRPGARGLAR FHPSRQQQQQ FPSLDDKPQF
60 70 80 90 100
PGASAEFVDK LEFIQPNVIS GIPIYRVMDR QGQIINPSED PHLPQEEVLK
110 120 130 140 150
LYRSMTLLNT MDRILYESQR QGRISFYMTN YGEEGTHVGS AAALERTDLV
160 170 180 190 200
FGQYREAGVL MYRDYPLELF MAQCYGNVSD PGKGRQMPVH YGCKERHFVT
210 220 230 240 250
ISSPLATQIP QAVGAAYAAK RANANQIVIC YFGEGAASEG DAHAGFNFAA
260 270 280 290 300
TLECPIIFFC RNNGYAISTP TSEQYRGDGI AARGPGYGIM SIRVDGNDVF
310 320 330 340 350
AVYNATKEAR RRAVAENQPF LIEAMTYRIG HHSTSDDSSA YRSVDEVNYW
360 370 380 390 400
DKQDHPISRL RQYLLNQGWW DEEQEKAWRK QSRKKVMEAF EQAERKLKPN
410 420 430 440
PSLLFSDVYQ EMPAQLRRQQ ESLARHLQTY GEHYPLDHFD K
Length:441
Mass (Da):50,164
Last modified:October 1, 1989 - v1
Checksum:iFFD7709A94094C8E
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Non-terminal residuei11

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J02827 mRNA. Translation: AAA40811.1.
PIRiA29468. DERTXA.
RefSeqiNP_036914.1. NM_012782.1.
UniGeneiRn.49145.

Genome annotation databases

GeneIDi25244.
KEGGirno:25244.
UCSCiRGD:2196. rat.

Similar proteinsi

Entry informationi

Entry nameiODBA_RAT
AccessioniPrimary (citable) accession number: P11960
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1989
Last sequence update: October 1, 1989
Last modified: August 30, 2017
This is version 140 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families