background:
Glycogen synthase catalyzes the rate-limiting step in glycogen synthesis. Its activity is regulated by a complex phosphorylation-dephosphorylation mechanism and by allosteric stimulators and inhibitors. Two isozymes of synthase, a skeletal muscle type (Glycogen synthase 1 - GYS1) and a liver type (Glycogen synthase 2 - GYS2), have been identified.
Function:
Transfers the glycosyl residue from UDP-Glc to the non-reducing end of alpha-1,4-glucan.
Post-translational modifications:
Primed phosphorylation at Ser-657 (site 5) by CSNK2A1 and CSNK2A2 is required for inhibitory phosphorylation at Ser-641 (site 3a), Ser-645 (site 3b), Ser-649 (site 3c) and Ser-653 (site 4) by GSK3A an GSK3B. Dephosphorylation at Ser-641 and Ser-645 by PP1 activates the enzyme (By similarity).
DISEASE:
Defects in GYS2 are the cause of glycogen storage disease type 0 (GSD0) [MIM:48600]; A metabolic disorder characterized by fasting hypoglycemia presenting in infancy or early childhood, high blood ketones and low alanine and lactate concentrations. Although feeding relieves symptoms, it often results in postprandial hyperglycemia and hyperlactatemia.
Similarity:
Belongs to the glycosyltransferase 3 family.
Database links:
Entrez Gene: 2998 Human
Entrez Gene: 232493 Mouse
Entrez Gene: 25623 Rat
Omim: 138571 Human
SwissProt: P54168 Human
SwissProt: Q8VCB3 Mouse
SwissProt: P17625 Rat
Unigene: 82614 Human
Unigene: 275975 Mouse
Unigene: 2906 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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