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MGP001386

UniProt Annotations

Entry Information
Gene Nameglycogen synthase 2 (liver)
Protein EntryGYS2_HUMAN
UniProt IDP54840
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityUDP-alpha-D-glucose + ((1->4)-alpha-D- glucosyl)(n) = UDP + ((1->4)-alpha-D-glucosyl)(n+1).
DiseaseGlycogen storage disease 0 (GSD0) [MIM:240600]: 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. {ECO:0000269|PubMed:9691087}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Enzyme RegulationAllosteric 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 (By similarity). {ECO:0000250}.
FunctionTransfers the glycosyl residue from UDP-Glc to the non- reducing end of alpha-1,4-glucan.
PathwayGlycan biosynthesis; glycogen biosynthesis.
PtmPrimed 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). Phosphorylation at Ser-8 is not required for interaction with GYG1 (By similarity). Interaction with GYG1 does not regulate the phosphorylation at Ser-8 and Ser-641 (By similarity). {ECO:0000250|UniProtKB:P13807, ECO:0000250|UniProtKB:P13834, ECO:0000250|UniProtKB:Q8VCB3}.
SimilarityBelongs to the glycosyltransferase 3 family. {ECO:0000305}.
SubunitInteracts with GYG1 (via C-terminus); required for GYS2- mediated glycogen synthesis. {ECO:0000250|UniProtKB:Q8VCB3}.
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