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