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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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BCKDK phosphorylates BCKDH

BCKDH + 2 ATP → p-BCKDH + 2 ADP + H+

Reactome
R-HSA-5693148
Compartment
mitochondrial matrix
Rhea
RHEA:17302

Inputs

Outputs

Catalysed by

  • protein kinase activity of BCKDK dimer [mitochondrial matrix]

Literature

  1. Cross-talk between thiamin diphosphate binding and phosphorylation loop conformation in human branched-chain alpha-keto acid decarboxylase/dehydrogenase J. Biol. Chem., 2004
  2. Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation Structure, 2004
  3. Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy Science, 2012
  4. 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