Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

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

Gene

Bckdk

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

Complete GO annotation...

Keywords - Molecular functioni

Kinase, Transferase

Keywords - Ligandi

ATP-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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 7

Organism-specific databases

MGIiMGI:1276121. Bckdk.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

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
Contains 1 histidine kinase domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

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

Family and domain databases

Gene3Di1.20.140.20. 1 hit.
3.30.565.10. 1 hit.
InterProiIPR018955. BCDHK/PDK_N.
IPR003594. HATPase_C.
IPR005467. His_kinase_dom.
IPR004358. Sig_transdc_His_kin-like_C.
[Graphical view]
PfamiPF10436. BCDHK_Adom3. 1 hit.
PF02518. HATPase_c. 1 hit.
[Graphical view]
PRINTSiPR00344. BCTRLSENSOR.
SMARTiSM00387. HATPase_c. 1 hit.
[Graphical view]
SUPFAMiSSF55874. SSF55874. 1 hit.
SSF69012. SSF69012. 1 hit.
PROSITEiPS50109. HIS_KIN. 1 hit.
[Graphical view]

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.

Cross-referencesi

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.

3D structure databases

ProteinModelPortaliO55028.
SMRiO55028.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000070345.

PTM databases

iPTMnetiO55028.
PhosphoSitePlusiO55028.

Proteomic databases

EPDiO55028.
PaxDbiO55028.
PeptideAtlasiO55028.
PRIDEiO55028.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi10295.
MGIiMGI:1276121. Bckdk.

Phylogenomic databases

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

Enzyme and pathway databases

ReactomeiR-MMU-70895. Branched-chain amino acid catabolism.

Miscellaneous databases

PROiO55028.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000030802.
CleanExiMM_BCKDK.
ExpressionAtlasiO55028. baseline and differential.
GenevisibleiO55028. MM.

Family and domain databases

Gene3Di1.20.140.20. 1 hit.
3.30.565.10. 1 hit.
InterProiIPR018955. BCDHK/PDK_N.
IPR003594. HATPase_C.
IPR005467. His_kinase_dom.
IPR004358. Sig_transdc_His_kin-like_C.
[Graphical view]
PfamiPF10436. BCDHK_Adom3. 1 hit.
PF02518. HATPase_c. 1 hit.
[Graphical view]
PRINTSiPR00344. BCTRLSENSOR.
SMARTiSM00387. HATPase_c. 1 hit.
[Graphical view]
SUPFAMiSSF55874. SSF55874. 1 hit.
SSF69012. SSF69012. 1 hit.
PROSITEiPS50109. HIS_KIN. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiBCKD_MOUSE
AccessioniPrimary (citable) accession number: O55028
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: June 1, 1998
Last modified: November 2, 2016
This is version 144 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. Human and mouse protein kinases
    Human and mouse protein kinases: classification and index
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.