Identification
HMDB Protein ID CDBP01386
Secondary Accession Numbers Not Available
Name [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
Description Not Available
Synonyms
  1. BCKD-kinase
  2. BCKDHKIN
  3. Branched-chain alpha-ketoacid dehydrogenase kinase
Gene Name BCKDK
Protein Type Enzyme
Biological Properties
General Function Involved in ATP binding
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
GO Classification
Function
two-component sensor activity
protein kinase activity
binding
catalytic activity
transferase activity
transferase activity, transferring phosphorus-containing groups
kinase activity
nucleoside binding
purine nucleoside binding
adenyl nucleotide binding
adenyl ribonucleotide binding
atp binding
protein histidine kinase activity
Process
phosphorus metabolic process
phosphate metabolic process
metabolic process
peptidyl-histidine phosphorylation
biological regulation
regulation of biological process
cellular metabolic process
regulation of cellular process
signal transduction
protein amino acid phosphorylation
phosphorylation
Cellular Location
  1. Mitochondrion matrix
Pathways
Gene Properties
Chromosome Location Chromosome:1
Locus 16p11.2
SNPs BCKDK
Gene Sequence
>1239 bp
ATGATCCTGGCGTCGGTGCTGAGGAGCGGTCCCGGGGGCGGGCTTCCGCTCCGGCCCCTC
CTGGGACCCGCACTCGCGCTCCGGGCCCGCTCGACGTCGGCCACCGACACACACCACGTG
GAGATGGCTCGGGAGCGCTCCAAGACCGTCACCTCCTTTTACAACCAGTCGGCCATCGAC
GCGGCAGCGGAGAAGCCCTCAGTCCGCCTAACGCCCACCATGATGCTCTACGCTGGCCGC
TCTCAGGACGGCAGCCACCTTCTGAAAAGTGCTCGGTACCTGCAGCAAGAACTTCCAGTG
AGGATTGCTCACCGCATCAAGGGCTTCCGCTGCCTTCCTTTCATCATTGGCTGCAACCCC
ACCATACTGCACGTGCATGAGCTATATATCCGTGCCTTCCAGAAGCTGACAGACTTCCCT
CCGATCAAGGACCAGGCGGACGAGGCCCAGTACTGCCAGCTGGTGCGACAGCTGCTGGAT
GACCACAAGGATGTGGTGACCCTCTTGGCAGAGGGCCTACGTGAGAGCCGGAAGCACATA
GAGGATGAAAAGCTCGTCCGCTACTTCTTGGACAAGACGCTGACTTCGAGGCTTGGAATC
CGCATGTTGGCCACGCATCACCTGGCGCTGCATGAGGACAAGCCTGACTTTTTCGGCATC
ATCTGTACTCGTCTCTCACCAAAGAAGATTATTGAGAAGTGGGTGGACTTTGCCAGACGC
CTGTGTGAGCACAAGTATGGCAATGCGCCCCGTGTCCGCATCAATGGCCATGTGGCTGCC
CGGTTCCCCTTCATCCCTATGCCACTGGACTACATCCTGCCGGAGCTGCTCAAGAATGCC
ATGAGAGCCACAATGGAGAGTCACCTAGACACTCCCTACAATGTCCCAGATGTGGTCATC
ACCATCGCCAACAATGATGTCGATCTGATCATCAGGATCTCAGACCGTGGTGGAGGAATC
GCTCACAAAGATCTGGACCGGGTCATGGACTACCACTTCACTACTGCTGAGGCCAGCACA
CAGGACCCCCGGATCAGCCCCCTCTTTGGCCATCTGGACATGCATAGTGGCGCCCAGTCA
GGACCCATGCACGGCTTTGGCTTCGGGTTGCCCACGTCACGGGCCTACGCGGAGTACCTC
GGTGGGTCTCTGCAGCTGCAGTCCCTGCAGGGCATTGGCACGGACGTCTACCTGCGGCTC
CGCCACATCGATGGCCGGGAGGAAAGCTTCCGGATCTGA
Protein Properties
Number of Residues 412
Molecular Weight 46360.0
Theoretical pI 9.06
Pfam Domain Function
Signals
  • ["None"]
Transmembrane Regions
  • ["None"]
Protein Sequence
>[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
MILASVLRSGPGGGLPLRPLLGPALALRARSTSATDTHHVEMARERSKTVTSFYNQSAID
AAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNP
TILHVHELYIRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHI
EDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARR
LCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVI
TIANNDVDLIIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRISPLFGHLDMHSGAQS
GPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI
GenBank ID Protein 2583173
UniProtKB/Swiss-Prot ID O14874
UniProtKB/Swiss-Prot Entry Name BCKD_HUMAN
PDB IDs
GenBank Gene ID AF026548
GeneCard ID BCKDK
GenAtlas ID BCKDK
HGNC ID HGNC:16902
References
General References Not Available