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Protein

[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial

Gene

Bckdk

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex.By similarity

Catalytic activityi

ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] = ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionKinase, Transferase
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-MMU-70895 Branched-chain amino acid catabolism

Names & Taxonomyi

Protein namesi
Recommended name:
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial (EC:2.7.11.4)
Alternative name(s):
Branched-chain alpha-ketoacid dehydrogenase kinase
Short name:
BCKD-kinase
Short name:
BCKDHKIN
Gene namesi
Name:Bckdk
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 7

Organism-specific databases

MGIiMGI:1276121 Bckdk

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

Pathology & Biotechi

Disruption phenotypei

Mutant animals are born at the expected Mendelian ratio, but they exhibit a decreased fertility. They are healthy at birth. At 3 weeks of age, they start showing growth retardation. At 12 weeks, they are 15% smaller than their wild-type littermates. Adults develop neurological abnormalities, such as tremors, epileptic seizures and hindlimb clasping. These neurological deficits can be completely abolished when mice are fed with a diet enriched in branched amino acids.2 Publications

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 30MitochondrionBy similarityAdd BLAST30
ChainiPRO_000002345331 – 412[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrialAdd BLAST382

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei31PhosphoserineBy similarity1
Modified residuei52Phosphoserine; by autocatalysisBy similarity1
Modified residuei192N6-acetyllysineCombined sources1
Modified residuei233N6-acetyllysineBy similarity1
Modified residuei356PhosphoserineCombined sources1
Modified residuei360PhosphoserineBy similarity1

Post-translational modificationi

Autophosphorylated.By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiO55028
PaxDbiO55028
PeptideAtlasiO55028
PRIDEiO55028

PTM databases

iPTMnetiO55028
PhosphoSitePlusiO55028

Expressioni

Tissue specificityi

Ubiquitous.

Inductioni

Expression in female liver is influenced by circadian rhythm.

Gene expression databases

BgeeiENSMUSG00000030802
CleanExiMM_BCKDK
ExpressionAtlasiO55028 baseline and differential
GenevisibleiO55028 MM

Interactioni

Subunit structurei

Monomer.By similarity

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000070345

Structurei

3D structure databases

ProteinModelPortaliO55028
SMRiO55028
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini159 – 404Histidine kinasePROSITE-ProRule annotationAdd BLAST246

Sequence similaritiesi

Belongs to the PDK/BCKDK protein kinase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0787 Eukaryota
COG0642 LUCA
GeneTreeiENSGT00550000074574
HOGENOMiHOG000164315
HOVERGENiHBG004829
InParanoidiO55028
KOiK00905
OMAiADEAQYC
OrthoDBiEOG091G07VC
PhylomeDBiO55028
TreeFamiTF331303

Family and domain databases

CDDicd00075 HATPase_c, 1 hit
Gene3Di1.20.140.20, 1 hit
3.30.565.10, 1 hit
InterProiView protein in InterPro
IPR036784 AK/P_DHK_N_sf
IPR018955 BCDHK/PDK_N
IPR003594 HATPase_C
IPR036890 HATPase_C_sf
IPR005467 His_kinase_dom
IPR004358 Sig_transdc_His_kin-like_C
PfamiView protein in Pfam
PF10436 BCDHK_Adom3, 1 hit
PF02518 HATPase_c, 1 hit
PRINTSiPR00344 BCTRLSENSOR
SMARTiView protein in SMART
SM00387 HATPase_c, 1 hit
SUPFAMiSSF55874 SSF55874, 1 hit
SSF69012 SSF69012, 1 hit
PROSITEiView protein in PROSITE
PS50109 HIS_KIN, 1 hit

Sequencei

Sequence statusi: Complete.

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

O55028-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MILTSVLGSG PRSWSSLWPL LGSSLSLRAR STSATDTHHV ELARERSKTV
60 70 80 90 100
TSFYNQSAID VAAEKPSVRL TPTMMLYSGR SQDGSHLLKS GRYLQQELPV
110 120 130 140 150
RIAHRIKGFR SLPFIIGCNP TILHVHELYI RAFQKLTDFP PIKDQADEAQ
160 170 180 190 200
YCQLVRQLLD DHKDVVTLLA EGLRESRKHI QDEKLVRYFL DKTLTSRLGI
210 220 230 240 250
RMLATHHLAL HEDKPDFVGI ICTRLSPKKI IEKWVDFARR LCEHKYGNAP
260 270 280 290 300
RVRINGHVAA RFPFIPMPLD YILPELLKNA MRATMESHLD TPYNVPDVVI
310 320 330 340 350
TIANNDIDLI IRISDRGGGI AHKDLDRVMD YHFTTAEAST QDPRINPLFG
360 370 380 390 400
HLDMHSGGQS GPMHGFGFGL PTSRAYAEYL GGSLQLQSLQ GIGTDVYLRL
410
RHIDGREESF RI
Length:412
Mass (Da):46,588
Last modified:June 1, 1998 - v1
Checksum:i80B3B173AAC8AAD3
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF043070 mRNA Translation: AAB97689.1
BC046595 mRNA Translation: AAH46595.1
CCDSiCCDS21884.1
RefSeqiNP_033869.1, NM_009739.3
UniGeneiMm.425495

Genome annotation databases

EnsembliENSMUST00000071056; ENSMUSP00000070345; ENSMUSG00000030802
GeneIDi12041
KEGGimmu:12041
UCSCiuc009jxf.1 mouse

Similar proteinsi

Entry informationi

Entry nameiBCKD_MOUSE
AccessioniPrimary (citable) accession number: O55028
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: June 1, 1998
Last modified: June 20, 2018
This is version 151 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

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