3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring) kinase
| [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] kinase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
| |||||||||
In
enzymology, a [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] (EC 2.7.11.4) 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
3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring), and phosphate
.
This enzyme belongs to the family of
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]
References
- PMID 22956686.
Literature
- Paxton R, Harris RA (1982). "Isolation of rabbit liver branched chain alpha-ketoacid dehydrogenase and regulation by phosphorylation". J. Biol. Chem. 257 (23): 14433–9. PMID 7142221.
- Wynn RM, Chuang JL, Cote CD, Chuang DT (2000). "Tetrameric assembly and conservation in the ATP-binding domain of rat branched-chain alpha-ketoacid dehydrogenase kinase". J. Biol. Chem. 275 (39): 30512–9. PMID 10903321.
- Chuang JL, Wynn RM, Chuang DT (2002). "The C-terminal hinge region of lipoic acid-bearing domain of E2b is essential for domain interaction with branched-chain alpha-keto acid dehydrogenase kinase". J. Biol. Chem. 277 (40): 36905–8. PMID 12189132.
- Popov KM, Hawes JW, Harris RA (1997). "Mitochondrial alpha-ketoacid dehydrogenase kinases: a new family of protein kinases". Adv. Second. Messenger. Phosphoprotein. Res. Advances in Second Messenger and Phosphoprotein Research. 31: 105–11. PMID 9344245.
