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

Details

Name
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
Synonyms
  • 2.7.11.4
  • BCKD-kinase
  • BCKDHKIN
  • Branched-chain alpha-ketoacid dehydrogenase kinase
Gene Name
BCKDK
Organism
Humans
Amino acid sequence
>lcl|BSEQ0009492|[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
MILASVLRSGPGGGLPLRPLLGPALALRARSTSATDTHHVEMARERSKTVTSFYNQSAID
AAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNP
TILHVHELYIRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHI
EDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARR
LCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVI
TIANNDVDLIIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRISPLFGHLDMHSGAQS
GPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI
Number of residues
412
Molecular Weight
46359.955
Theoretical pI
Not Available
GO Classification
Functions
[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity / ATP binding / kinase activity / protein serine/threonine kinase activity
Processes
branched-chain amino acid catabolic process / cellular amino acid catabolic process / cellular nitrogen compound metabolic process / phosphorylation / protein phosphorylation / small molecule metabolic process
Components
mitochondrial alpha-ketoglutarate dehydrogenase complex / mitochondrial matrix / mitochondrion
General Function
Protein serine/threonine kinase activity
Specific Function
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.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Mitochondrion matrix
Gene sequence
>lcl|BSEQ0013196|[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial (BCKDK)
ATGATCCTGGCGTCGGTGCTGAGGAGCGGTCCCGGGGGCGGGCTTCCGCTCCGGCCCCTC
CTGGGACCCGCACTCGCGCTCCGGGCCCGCTCGACGTCGGCCACCGACACACACCACGTG
GAGATGGCTCGGGAGCGCTCCAAGACCGTCACCTCCTTTTACAACCAGTCGGCCATCGAC
GCGGCAGCGGAGAAGCCCTCAGTCCGCCTAACGCCCACCATGATGCTCTACGCTGGCCGC
TCTCAGGACGGCAGCCACCTTCTGAAAAGTGCTCGGTACCTGCAGCAAGAACTTCCAGTG
AGGATTGCTCACCGCATCAAGGGCTTCCGCTGCCTTCCTTTCATCATTGGCTGCAACCCC
ACCATACTGCACGTGCATGAGCTATATATCCGTGCCTTCCAGAAGCTGACAGACTTCCCT
CCGATCAAGGACCAGGCGGACGAGGCCCAGTACTGCCAGCTGGTGCGACAGCTGCTGGAT
GACCACAAGGATGTGGTGACCCTCTTGGCAGAGGGCCTACGTGAGAGCCGGAAGCACATA
GAGGATGAAAAGCTCGTCCGCTACTTCTTGGACAAGACGCTGACTTCGAGGCTTGGAATC
CGCATGTTGGCCACGCATCACCTGGCGCTGCATGAGGACAAGCCTGACTTTGTCGGCATC
ATCTGTACTCGTCTCTCACCAAAGAAGATTATTGAGAAGTGGGTGGACTTTGCCAGACGC
CTGTGTGAGCACAAGTATGGCAATGCGCCCCGTGTCCGCATCAATGGCCATGTGGCTGCC
CGGTTCCCCTTCATCCCTATGCCACTGGACTACATCCTGCCGGAGCTGCTCAAGAATGCC
ATGAGAGCCACAATGGAGAGTCACCTAGACACTCCCTACAATGTCCCAGATGTGGTCATC
ACCATCGCCAACAATGATGTCGATCTGATCATCAGGATCTCAGACCGTGGTGGAGGAATC
GCTCACAAAGATCTGGACCGGGTCATGGACTACCACTTCACTACTGCTGAGGCCAGCACA
CAGGACCCCCGGATCAGCCCCCTCTTTGGCCATCTGGACATGCATAGTGGCGCCCAGTCA
GGACCCATGCACGGGTGA
Chromosome Location
16
Locus
Not Available
External Identifiers
ResourceLink
UniProtKB IDO14874
UniProtKB Entry NameBCKD_HUMAN
HGNC IDHGNC:16902
General References
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  8. Olsen JV, Vermeulen M, Santamaria A, Kumar C, Miller ML, Jensen LJ, Gnad F, Cox J, Jensen TS, Nigg EA, Brunak S, Mann M: Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis. Sci Signal. 2010 Jan 12;3(104):ra3. doi: 10.1126/scisignal.2000475. [Article]
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  10. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. [Article]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB02930Adenosine 5'-[gamma-thio]triphosphateexperimentalunknownDetails