EKS-HOS-00457
Eukaryotic Protein Kinase & Protein Phosphatase Database
TagContent
EKPD IDEKS-HOS-00457
Classification
Group/FamilyScoreE-ValueStartEndDomain Length
Atypical/PDHK189.71.0E-55264400137
StatusReviewed
Ensembl ProteinENSP00000219794
UniProt AccessionO14874; Q6FGL4; Q96IN5;
Protein Name[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
Protein Synonyms/Alias Branched-chain alpha-ketoacid dehydrogenase kinase; BCKD-kinase; BCKDHKIN;
Gene NameBCKDK
Gene Synonyms/Alias BCKDK;
Ensembl Information
Ensembl Gene IDEnsembl Protein IDEnsembl Transcript ID
ENSG00000103507ENSP00000455866ENST00000561755
ENSG00000103507ENSP00000454947ENST00000567676
ENSG00000103507ENSP00000219794ENST00000219794
ENSG00000103507ENSP00000287507ENST00000287507
ENSG00000103507ENSP00000457821ENST00000567682
ENSG00000103507ENSP00000378405ENST00000394951
ENSG00000103507ENSP00000457226ENST00000484226
ENSG00000103507ENSP00000378404ENST00000394950
ENSG00000103507ENSP00000456479ENST00000567530
OrganismHomo sapiens
Functional DescriptionCatalyzes the phosphorylation and inactivation of thebranched-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).
Protein Length412
Protein Sequence
(FASTA)
MILASVLRSG PGGGLPLRPL LGPALALRAR STSATDTHHV EMARERSKTV TSFYNQSAID 60
AAAEKPSVRL TPTMMLYAGR SQDGSHLLKS ARYLQQELPV RIAHRIKGFR CLPFIIGCNP 120
TILHVHELYI RAFQKLTDFP PIKDQADEAQ YCQLVRQLLD DHKDVVTLLA EGLRESRKHI 180
EDEKLVRYFL DKTLTSRLGI RMLATHHLAL HEDKPDFVGI ICTRLSPKKI IEKWVDFARR 240
LCEHKYGNAP RVRINGHVAA RFPFIPMPLD YILPELLKNA MRATMESHLD TPYNVPDVVI 300
TIANNDVDLI IRISDRGGGI AHKDLDRVMD YHFTTAEAST QDPRISPLFG HLDMHSGAQS 360
GPMHGFGFGL PTSRAYAEYL GGSLQLQSLQ GIGTDVYLRL RHIDGREESF RI 412
Nucleotide Sequence
(FASTA)
ATGATCCTGG CGTCGGTGCT GAGGAGCGGT CCCGGGGGCG GGCTTCCGCT CCGGCCCCTC 60
CTGGGACCCG CACTCGCGCT CCGGGCCCGC TCGACGTCGG CCACCGACAC ACACCACGTG 120
GAGATGGCTC GGGAGCGCTC CAAGACCGTC ACCTCCTTTT ACAACCAGTC GGCCATCGAC 180
GCGGCAGCGG AGAAGCCCTC AGTCCGCCTA ACGCCCACCA TGATGCTCTA CGCTGGCCGC 240
TCTCAGGACG GCAGCCACCT TCTGAAAAGT GCTCGGTACC TGCAGCAAGA ACTTCCAGTG 300
AGGATTGCTC ACCGCATCAA GGGCTTCCGC TGCCTTCCTT TCATCATTGG CTGCAACCCC 360
ACCATACTGC ACGTGCATGA GCTATATATC CGTGCCTTCC AGAAGCTGAC AGACTTCCCT 420
CCGATCAAGG ACCAGGCGGA CGAGGCCCAG TACTGCCAGC TGGTGCGACA GCTGCTGGAT 480
GACCACAAGG ATGTGGTGAC CCTCTTGGCA GAGGGCCTAC GTGAGAGCCG GAAGCACATA 540
GAGGATGAAA AGCTCGTCCG CTACTTCTTG GACAAGACGC TGACTTCGAG GCTTGGAATC 600
CGCATGTTGG CCACGCATCA CCTGGCGCTG CATGAGGACA AGCCTGACTT TGTCGGCATC 660
ATCTGTACTC GTCTCTCACC AAAGAAGATT ATTGAGAAGT GGGTGGACTT TGCCAGACGC 720
CTGTGTGAGC ACAAGTATGG CAATGCGCCC CGTGTCCGCA TCAATGGCCA TGTGGCTGCC 780
CGGTTCCCCT TCATCCCTAT GCCACTGGAC TACATCCTGC CGGAGCTGCT CAAGAATGCC 840
ATGAGAGCCA CAATGGAGAG TCACCTAGAC ACTCCCTACA ATGTCCCAGA TGTGGTCATC 900
ACCATCGCCA ACAATGATGT CGATCTGATC ATCAGGATCT CAGACCGTGG TGGAGGAATC 960
GCTCACAAAG ATCTGGACCG GGTCATGGAC TACCACTTCA CTACTGCTGA GGCCAGCACA 1020
CAGGACCCCC GGATCAGCCC CCTCTTTGGC CATCTGGACA TGCATAGTGG CGCCCAGTCA 1080
GGACCCATGC ACGGCTTTGG CTTCGGGTTG CCCACGTCAC GGGCCTACGC GGAGTACCTC 1140
GGTGGGTCTC TGCAGCTGCA GTCCCTGCAG GGCATTGGCA CGGACGTCTA CCTGCGGCTC 1200
CGCCACATCG ATGGCCGGGA GGAAAGCTTC CGGATCTGA 1239
Domain Profile
S: 1     yvpshlyyllfellknamratveshedkkv.lpdvkvlvalgkedliikisdrGGGvplr  59
         ++p++l+y+l+ellknamrat+esh d+++ +pdv++++a++++dlii+isdrGGG++++
Q: 264   FIPMPLDYILPELLKNAMRATMESHLDTPYnVPDVVITIANNDVDLIIRISDRGGGIAHK  323
         8*****************************9*****************************
S: 60    kidrlldymystapapskdd..............araaplaGfGyGlpisrlyakylqGd  105
         ++dr++dy+++ta+a+++d+              a+++p++GfG+Glp+sr+ya+yl+G+
Q: 324   DLDRVMDYHFTTAEASTQDPrisplfghldmhsgAQSGPMHGFGFGLPTSRAYAEYLGGS  383
         ************************************************************
S: 106   lkllsleGiGtdaviylka  124
         l+l+sl+GiGtd+  yl++
Q: 384   LQLQSLQGIGTDV--YLRL  400
         ************9..8886
Domain Sequence
(FASTA)
FIPMPLDYIL PELLKNAMRA TMESHLDTPY NVPDVVITIA NNDVDLIIRI SDRGGGIAHK 60
DLDRVMDYHF TTAEASTQDP RISPLFGHLD MHSGAQSGPM HGFGFGLPTS RAYAEYLGGS 120
LQLQSLQGIG TDVYLRL 137
KeywordAcetylation; ATP-binding; Complete proteome; Kinase; Mitochondrion; Nucleotide-binding; Phosphoprotein; Reference proteome; Serine/threonine-protein kinase; Transferase; Transit peptide.
Sequence SourceEnsembl
Orthology
Ortholog group
Ailuropoda melanoleuca"; ?>Anolis carolinensis"; ?>Bos taurus"; ?>Callithrix jacchus"; ?>Canis familiaris"; ?>Cavia porcellus"; ?>Danio rerio"; ?>Dasypus novemcinctus"; ?>Dipodomys ordii"; ?>Echinops telfairi"; ?>Equus caballus"; ?>Felis catus"; ?>Gadus morhua"; ?>Gasterosteus aculeatus"; ?>Gorilla gorilla"; ?>Ictidomys tridecemlineatus"; ?>Latimeria chalumnae"; ?>Loxodonta africana"; ?>Macaca mulatta"; ?>Monodelphis domestica"; ?>Mus musculus"; ?>Mustela putorius furo"; ?>Myotis lucifugus"; ?>Nomascus leucogenys"; ?>Ochotona princeps"; ?>Oreochromis niloticus"; ?>Oryctolagus cuniculus"; ?>Oryzias latipes"; ?>Otolemur garnettii"; ?>Pan troglodytes"; ?>Pelodiscus sinensis"; ?>Pongo abelii"; ?>Procavia capensis"; ?>Pteropus vampyrus"; ?>Rattus norvegicus"; ?>Sarcophilus harrisii"; ?>Sus scrofa"; ?>Takifugu rubripes"; ?>Tetraodon nigroviridis"; ?>Xenopus tropicalis"; ?>Xiphophorus maculatus"; ?>
EKS-AIM-00420
EKS-ANC-00437
EKS-BOT-00450
EKS-CAJ-00455
EKS-CAF-00450
EKS-CAP-00483
EKS-DAR-00855
EKS-DAN-00024
EKS-DIO-00091
EKS-ECT-00054
EKS-EQC-00440
EKS-FEC-00429
EKS-GAM-00261
EKS-GAA-00546
EKS-GOG-00442
EKS-ICT-00431
EKS-LAC-00464
EKS-LOA-00458
EKS-MAM-00447
EKS-MOD-00439
EKS-MUM-00482
EKS-MUP-00443
EKS-MYL-00443
EKS-NOL-00409
EKS-OCP-00111
EKS-ORN-00569
EKS-ORC-00423
EKS-ORL-00520
EKS-OTG-00453
EKS-PAT-00425
EKS-PES-00396
EKS-POA-00439
EKS-PRC-00054
EKS-PTV-00158
EKS-RAN-00463
EKS-SAH-00416
EKS-SUS-00398
EKS-TAR-00561
EKS-TEN-00564
EKS-XET-00601
EKS-XIM-00561
Gene Ontology
GO:0005947; C:mitochondrial alpha-ketoglutarate dehydrogenase complex
GO:0047323; F:[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity
GO:0005524; F:ATP binding
GO:0004674; F:protein serine/threonine kinase activity
GO:0009083; P:branched-chain amino acid catabolic process
KEGG
hsa:10295;
InterPros
IPR003594; ATPase-like_ATP-bd.
IPR018955; BCDHK/PDK_N.
IPR004358; Sig_transdc_His_kin-like_C.
IPR005467; Sig_transdc_His_kinase_core.
Pfam
PF10436; BCDHK_Adom3; 1.
PF02518; HATPase_c; 1.
SMARTs
SM00387; HATPase_c; 1.
Prosites
PS50109; HIS_KIN; 1.
Prints
PR00344; BCTRLSENSOR.
Created Date20-Feb-2013