dbPAF Protein Information

Tag Content
dbPAF ID dbPAF-0000778
Uniprot Accession O14874; BCKD_HUMAN; A8MY43; Q6FGL4; Q96G95; Q96IN5;
Genbank Protein ID NP_001116429.1; NP_001258855.1; NP_005872.2;
Genbank Nucleotide ID NM_001122957.2; NM_001271926.1; NM_005881.3;
Protein Name [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
Protein Synonyms/Alias Branched-chain alpha-ketoacid dehydrogenase kinase;BCKD-kinase;BCKDHKIN;
Gene Name BCKDK
Gene Synonyms/Alias
Organism Homo sapiens(Human)
NCBI Taxa ID 9606
Functional Description
(View all)		 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.
Phosphorylation Sites
 dbPAF PTMs: 10
PositionPeptidesSourceReferences ( PMIDs )
31ALALRARSTSATDTHdbPTM 3.0;Phospho.ELM 9.0;HPRD 9;PHOSIDA;PhosphoPep 2.0;PhosphoSitePlus;SysPTM 2.0;curated18691976; 16381945; 23193290; 18220336; 20044836; 20068231;
32LALRARSTSATDTHHdbPTM 3.0;HPRD 9;PHOSIDA;PhosphoPep 2.0;PhosphoSitePlus;SysPTM 2.0;curated18707149; 20815410; 16381945; 23193290; 17081983; 20068231;
33ALRARSTSATDTHHVdbPTM 3.0;Phospho.ELM 9.0;HPRD 9;PHOSIDA;PhosphoSitePlus;SysPTM 2.0;curated18220336; 16381945; 23193290; 18707149; 19276368; 20815410;
35RARSTSATDTHHVEMdbPTM 3.0;HPRD 9;PHOSIDA;PhosphoSitePlus;curated19276368; 20044836; 20166139; 20815410; 16381945; 23193290;
37RSTSATDTHHVEMARPhosphoSitePlus;curated22135298; 20068231; 24702127
47VEMARERSKTVTSFYPhosphoSitePlus22135298
52ERSKTVTSFYNQSAIPhosphoPep 2.0;PhosphoSitePlus19060867; 22135298
57VTSFYNQSAIDAAAEPHOSIDA21081558
77LTPTMMLYAGRSQDGPhosphoSitePlus22135298
93HLLKSARYLQQELPVPhosphoSitePlus22135298
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 413
Keyword

KW-0007--Acetylation
KW-0025--Alternative splicing
KW-0067--ATP-binding
KW-1269--Autism
KW-1268--Autism spectrum disorder
KW-0181--Complete proteome
KW-0225--Disease mutation
KW-0418--Kinase
KW-0496--Mitochondrion
KW-0547--Nucleotide-binding
KW-0597--Phosphoprotein
KW-1185--Reference proteome
KW-0808--Transferase
KW-0809--Transit peptide
Interpro

IPR018955--BCDHK/PDK_N
IPR003594--HATPase_C
IPR005467--His_kinase_dom
IPR004358--Sig_transdc_His_kin-like_C
PROSITE

PS50109--HIS_KIN
Pfam

PF10436--BCDHK_Adom3
PF02518--HATPase_c
Gene Ontology

GO:0005947--C:mitochondrial alpha-ketoglutarate dehydrogenase complex
GO:0005759--C:mitochondrial matrix
GO:0005739--C:mitochondrion
GO:0047323--F:[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity
GO:0005524--F:ATP binding
GO:0016301--F:kinase activity
GO:0004674--F:protein serine/threonine kinase activity
GO:0009083--P:branched-chain amino acid catabolic process
GO:0009063--P:cellular amino acid catabolic process
GO:0034641--P:cellular nitrogen compound metabolic process
GO:0016310--P:phosphorylation
GO:0006468--P:protein phosphorylation
GO:0044281--P:small molecule metabolic process