3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring) kinase

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[3-methyl-2-oxobutanoate dehydrogenase (lipoamide)] kinase
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EC no. 2.7.11.4
CAS no. 82391-38-6
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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 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]

Related Research Articles

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Branched chain ketoacid dehydrogenase kinase (BCKDK) is an enzyme encoded by the BCKDK gene on chromosome 16. This enzyme is part of the mitochondrial protein kinases family and it is a regulator of the valine, leucine, and isoleucine catabolic pathways. BCKDK is found in the mitochondrial matrix and the prevalence of it depends on the type of cell. Liver cells tend to have the lowest concentration of BCKDK, whereas skeletal muscle cells have the highest amount. Abnormal activity of this enzyme often leads to diseases such as maple syrup urine disease and cachexia.

(3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring))-phosphatase (EC 3.1.3.52, branched-chain oxo-acid dehydrogenase phosphatase, branched-chain 2-keto acid dehydrogenase phosphatase, branched-chain α-keto acid dehydrogenase phosphatase, BCKDH', [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)]-phosphatase, [3-methyl-2-oxobutanoate dehydrogenase (lipoamide)]-phosphate phosphohydrolase) is an enzyme with systematic name (3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring))-phosphate phosphohydrolase. This enzyme catalyses the following chemical reaction

References

  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.

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