Names & Taxonomy

Uniprot ID:
O14874
Entry Name:
BCKD_HUMAN
Status:
reviewed
Protein Names:
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial (EC 2.7.11.4) (Branched-chain alpha-ketoacid dehydrogenase kinase) (BCKD-kinase) (BCKDHKIN)
Gene Names:
BCKDK
Gene Names Primary:
BCKDK
Organism:
Homo sapiens (Human)

Structure

Length:
412
Sequence:
MILASVLRSGPGGGLPLRPLLGPALALRARSTSATDTHHVEMARERSKTVTSFYNQSAIDAAAEKPSVRLTPTMMLYAGRSQDGSHLLKSARYLQQELPVRIAHRIKGFRCLPFIIGCNPTILHVHELYIRAFQKLTDFPPIKDQADEAQYCQLVRQLLDDHKDVVTLLAEGLRESRKHIEDEKLVRYFLDKTLTSRLGIRMLATHHLALHEDKPDFVGIICTRLSPKKIIEKWVDFARRLCEHKYGNAPRVRINGHVAARFPFIPMPLDYILPELLKNAMRATMESHLDTPYNVPDVVITIANNDVDLIIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHGFGFGLPTSRAYAEYLGGSLQLQSLQGIGTDVYLRLRHIDGREESFRI
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion matrix. Mitochondrion

Function

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.
Catalytic Activity:
ATP + = ADP + phosphate.
Gene Ontology Go:
mitochondrial alpha-ketoglutarate dehydrogenase complex
mitochondrial matrix
mitochondrion
kinase activity
ATP binding
kinase activity
protein serine/threonine kinase activity
branched-chain amino acid catabolic process
cellular amino acid catabolic process
cellular nitrogen compound metabolic process
phosphorylation
small molecule metabolic process
Gene Ontology Biological Process:
branched-chain amino acid catabolic process
cellular amino acid catabolic process
cellular nitrogen compound metabolic process
phosphorylation
small molecule metabolic process
Gene Ontology Molecular Function:
kinase activity
ATP binding
kinase activity
protein serine/threonine kinase activity
Gene Ontology Cellular Component:
mitochondrial alpha-ketoglutarate dehydrogenase complex
mitochondrial matrix
mitochondrion
Keywords:
ATP-binding
Acetylation
Alternative splicing
Autism
Autism spectrum disorder
Complete proteome
Disease mutation
Kinase
Mitochondrion
Nucleotide-binding
Phosphoprotein
Reference proteome
Transferase
Transit peptide
Interacts With:
Q9WMX2; P40763

Publication

PubMed ID:
14702039 15616553 15489334 17081983 18220336 18691976 19608861 20068231 21406692 24275569 25944712 22956686 24449431