MGP Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins
MGP001385

UniProt Annotations

Entry Information
Gene Nameglycogen synthase 1 (muscle)
Protein EntryGYS1_HUMAN
UniProt IDP13807
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P13807-1; Sequence=Displayed; Name=2; IsoId=P13807-2; Sequence=VSP_042745; Note=No experimental confirmation available.;
Catalytic ActivityUDP-alpha-D-glucose + ((1->4)-alpha-D- glucosyl)(n) = UDP + ((1->4)-alpha-D-glucosyl)(n+1).
DiseaseMuscle glycogen storage disease 0 (GSD0b) [MIM:611556]: 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. {ECO:0000269|PubMed:17928598}. 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.
InteractionP49841:GSK3B; NbExp=3; IntAct=EBI-740553, EBI-373586; P46976:GYG1; NbExp=4; IntAct=EBI-740553, EBI-740533;
PathwayGlycan biosynthesis; glycogen biosynthesis.
PtmPhosphorylation at Ser-8 by AMPK inactivates the enzyme activity. 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 (By similarity). Phosphorylated at Ser-641 by DYRK2, leading to inactivation (By similarity). Phosphorylated at Ser-641 by PASK, leading to inactivation; phosphorylation by PASK is inhibited by glycogen. Dephosphorylation at Ser-641 and Ser-645 by PP1 activates the enzyme. {ECO:0000250, ECO:0000269|PubMed:16275910, ECO:0000269|PubMed:18669648, ECO:0000269|PubMed:19690332}.
SimilarityBelongs to the glycosyltransferase 3 family. {ECO:0000305}.
SubunitInteracts with GYG1. {ECO:0000269|PubMed:17055998}.
  logo