GYS1
entrez
2997
canonical
uniprot
P13807
canonical
ensembl
ENSG00000104812
resolver
ensp
ENSP00000263276
resolver
ensp
ENSP00000317904
resolver
genesymbol
GYS1
resolver
genesymbol_syn
GYS
resolver
hgnc
HGNC:4706
resolver
uniprot
Q9BTT9
resolver
uniprot_entry
GYS1_HUMAN
resolver
uniprot_sec
Q9BTT9
resolver
activity_regulation
ACTIVITY REGULATION: Allosteric activation by glucose-6-phosphate (PubMed:35690592, PubMed:35835870). Phosphorylation reduces enzyme activity by constraining a tense conformation of the tetramer through inter-subunit interaction (PubMed:35690592, PubMed:35835870). Phosphorylation reduces the activity towards UDP-glucose (By similarity). 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|UniProtKB:P13834, ECO:0000250|UniProtKB:P54840, ECO:0000269|PubMed:35690592, ECO:0000269|PubMed:35835870}.
uniprot
proteins
amino_acid_sequence
MPLNRTLSMSSLPGLEDWEDEFDLENAVLFEVAWEVANKVGGIYTVLQTKAKVTGDEWGDNYFLVGPYTEQGVRTQVELLEAPTPALKRTLDSMNSKGCKVYFGRWLIEGGPLVVLLDVGASAWALERWKGELWDTCNIGVPWYDREANDAVLFGFLTTWFLGEFLAQSEEKPHVVAHFHEWLAGVGLCLCRARRLPVATIFTTHATLLGRYLCAGAVDFYNNLENFNVDKEAGERQIYHRYCMERAAAHCAHVFTTVSQITAIEAQHLLKRKPDIVTPNGLNVKKFSAMHEFQNLHAQSKARIQEFVRGHFYGHLDFNLDKTLYFFIAGRYEFSNKGADVFLEALARLNYLLRVNGSEQTVVAFFIMPARTNNFNVETLKGQAVRKQLWDTANTVKEKFGRKLYESLLVGSLPDMNKMLDKEDFTMMKRAIFATQRQSFPPVCTHNMLDDSSDPILTTIRRIGLFNSSADRVKVIFHPEFLSSTSPLLPVDYEEFVRGCHLGVFPSYYEPWGYTPAECTVMGIPSISTNLSGFGCFMEEHIADPSAYGIYILDRRFRSLDDSCSQLTSFLYSFCQQSRRQRIIQRNRTERLSDLLDWKYLGRYYMSARHMALSKAFPEHFTYEPNEADAAQGYRYPRPASVPPSPSLSRHSSPHQSEDEEDPRNGPLEEDGERYDEDEEAAKDRRNIRAPEWPRRASCTSSTSGSKRNSVDTATSSSLSTPSEPLSPTSSLGEERN
uniprot
proteins
disease_involvement
DISEASE: Muscle 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 variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
2.4.1.11
uniprot
proteins
function
FUNCTION: Glycogen synthase participates in the glycogen biosynthetic process along with glycogenin and glycogen branching enzyme. Extends the primer composed of a few glucose units formed by glycogenin by adding new glucose units to it. In this context, glycogen synthase transfers the glycosyl residue from UDP-Glc to the non-reducing end of alpha-1,4-glucan. {ECO:0000269|PubMed:35835870}.
uniprot
proteins
mass_dalton
83786
uniprot
proteins
pathway_participation
PATHWAY: Glycan biosynthesis; glycogen biosynthesis. {ECO:0000269|PubMed:35835870}.
uniprot
proteins
post_translational_modification
PTM: Phosphorylation 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 (PubMed:35835870). {ECO:0000250|UniProtKB:P13834, ECO:0000305|PubMed:35835870}.
uniprot
proteins
protein_family
Glycosyltransferase 3 family
uniprot
proteins
pubmed
15057824
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
17055998
uniprot
proteins
pubmed
17928598
uniprot
proteins
pubmed
18669648
uniprot
proteins
pubmed
18691976
uniprot
proteins
pubmed
19413330
uniprot
proteins
pubmed
19690332
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
21406692
uniprot
proteins
pubmed
21668450
uniprot
proteins
pubmed
23186163
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
2493642
uniprot
proteins
pubmed
35690592
uniprot
proteins
pubmed
35835870
uniprot
proteins
pubmed
7657035
uniprot
proteins
pubmed
9010351
uniprot
proteins
sequence_length
737
uniprot
proteins