InterPro domain: IPR039028
General Information
- Identifier IPR039028
- Description PDK/BCKDK protein kinase
- Number of genes 182
- Gene duplication stats Loading...
- Associated GO terms GO:0004672
Abstract
This entry includes [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase (BCKDK) and [Pyruvate dehydrogenase (acetyl-transferring)] kinase (PDK).
BCKDK catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways [ 1 ].
Pyruvate dehydrogenase kinase inhibits the pyruvate dehydrogenase complex by phosphorylation of the E1 alpha subunit, thus contributing to the regulation of glucose metabolism [ 2 ].
1. 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. 35, 470-7
2. Site specificity of four pyruvate dehydrogenase kinase isoenzymes toward the three phosphorylation sites of human pyruvate dehydrogenase. J. Biol. Chem. 276, 37223-9