GYS1

Glycogen [starch] synthase, muscle ;

catalytic activity:UDP-glucose ((1->4)-alpha-D-glucosyl)(n) = UDP + ((1->4)-alpha-D-glucosyl)(n+1).,disease:Defects in GYS1 are the cause of muscle glycogen storage disease type 0 (GSD0b) [MIM:611556]; also called muscle glycogen synthase deficiency. GSD0 is a metabolic disorder characterized by fasting hypoglycemia presenting in infancy or early childhood. The role of muscle glycogen is to provide critical energy during bursts of activity and sustained muscle work.,enzyme regulation:Allosteric activation by glucose-6-phosphate. Phosphorylation reduces the activity towards UDP-glucose. When in the non-phosphorylated state, glycogen synthase does not require glucose-6-phosphate as an allosteric activator; when phosphorylated it does.,function:Transfers the glycosyl residue from UDP-Glc to the non-reducing end of alpha-1,4-glucan.,pathway:Glycan biosynthesis; glycogen biosynthesis.,similarity:Belongs to the glycosyltransferase 3 family.,

polysaccharide biosynthetic process, polysaccharide metabolic process, glycogen metabolic process, glycogen biosynthetic process, monosaccharide metabolic process, glucose metabolic process, cellular glucan metabolic process,generation of precursor metabolites and energy, energy reserve metabolic process, heart development, glucan biosynthetic process, energy derivation by oxidation of organic compounds, carbohydrate biosynthetic process,hexose metabolic process, cellular polysaccharide biosynthetic process, cellular carbohydrate biosynthetic process,glucan metabolic process, cellular polysaccharide metabolic process,