[3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] kinase
EC no.
CAS no.82391-38-6
IntEnzIntEnz view
ExPASyNiceZyme view
MetaCycmetabolic pathway
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO

In enzymology, a [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] (EC is an enzyme that catalyzes the chemical reaction

ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate

Thus, the two substrates of this enzyme are ATP and 3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring), whereas its 3 products are ADP, 3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring), and phosphate.

This enzyme belongs to the family of transferases, specifically those transferring a phosphate group to the sidechain oxygen atom of serine or threonine residues in proteins (protein-serine/threonine kinases). The systematic name of this enzyme class is ATP:[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphotransferase. Other names in common use include kinase, BCK, BCKD kinase, BCODH kinase, branched-chain alpha-ketoacid dehydrogenase kinase, branched-chain 2-oxo acid dehydrogenase kinase, branched-chain keto acid dehydrogenase kinase, branched-chain oxo acid dehydrogenase kinase (phosphorylating), and STK2.

In 2012, it was suggested that mutations in the gene which expresses this enzyme could be the cause of a rare form of autism.[1]


  1. ^ Novarino, G.; El-Fishawy, P.; Kayserili, H.; Meguid, N. A.; Scott, E. M.; Schroth, J.; Silhavy, J. L.; Kara, M.; Khalil, R. O.; Ben-Omran, T.; Ercan-Sencicek, A. G.; Hashish, A. F.; Sanders, S. J.; Gupta, A. R.; Hashem, H. S.; Matern, D.; Gabriel, S.; Sweetman, L.; Rahimi, Y.; Harris, R. A.; State, M. W.; Gleeson, J. G. (2012). "Mutations in BCKD-kinase Lead to a Potentially Treatable Form of Autism with Epilepsy". Science. 338 (6105): 394–397. doi:10.1126/science.1224631. PMC 3704165. PMID 22956686.