InterPro domain: IPR011613

This entry represents a domain found in glycoside hydrolase family 15 members and in phosphorylase b kinase regulatory chains alpha and beta [ 1 ].

Glycoside hydrolase family 15 GH15 comprises enzymes with several known activities; glucoamylase ( 3.2.1.3 ); alpha-glucosidase ( 3.2.1.20 ); glucodextranase ( 3.2.1.70 ).

Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin. The beta chain acts as a regulatory unit and modulates the activity of the holoenzyme in response to phosphorylation [ 2 ].

1. 3D mapping of glycogenosis-causing mutations in the large regulatory alpha subunit of phosphorylase kinase. Biochim. Biophys. Acta 1782, 664-70
2. Muscle glycogenosis with low phosphorylase kinase activity: mutations in PHKA1, PHKG1 or six other candidate genes explain only a minority of cases. Eur. J. Hum. Genet. 11, 516-26