BCKDK phosphorylates BCKDH
BCKDH + 2 ATP → p-BCKDH + 2 ADP + H+
- Reactome
- R-HSA-5693148
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:17302
Inputs
- BCKDHR-HSA-70019, mitochondrial matrix
- 2 ATPCHEBI:30616, C10H12N5O13P3, mitochondrial matrix
Outputs
- p-BCKDHR-HSA-5693120, mitochondrial matrix
- 2 ADPCHEBI:456216, C10H12N5O10P2, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
Catalysed by
- protein kinase activity of BCKDK dimer [mitochondrial matrix]
Literature
- Cross-talk between thiamin diphosphate binding and phosphorylation loop conformation in human branched-chain alpha-keto acid decarboxylase/dehydrogenase J. Biol. Chem., 2004
- Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation Structure, 2004
- Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy Science, 2012
- Two novel mutations in the BCKDK (branched-chain keto-acid dehydrogenase kinase) gene are responsible for a neurobehavioral deficit in two pediatric unrelated patients Hum. Mutat., 2014