Search
glycogen synthase, muscle (GYS1)
Function:
- transfers the glycosyl residue from UDP-Glc to the non- reducing end of alpha-1,4-glucan
- allosteric activation by glucose-6-phosphate
- phosphorylated on 9 Ser by at least 8 different kinases
- 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 (putative)
- glycan biosynthesis; glycogen biosynthesis
UDP-glucose + (1,4-alpha-D-glucosyl)(n)
UDP + (1,4-alpha-D-glucosyl)(n+1)
Structure:
- belongs to the mammalian/fungal glycogen synthase family
Pathology:
- defects in GYS1 are the cause of muscle glycogen storage disease type 0
General
glycogen synthase
oligomerizing protein
phosphoprotein
Properties
SIZE: entity length = 737 aa
MW = 84 kD
MOTIF: Ser phosphorylation site {S8}
Ser phosphorylation site {S11}
binding site
SITE: 39-39
FOR-BINDING-OF: UDP-glucose
Ser phosphorylation site {S641}
Ser phosphorylation site {S645}
Ser phosphorylation site {S649}
Ser phosphorylation site {S653}
Ser phosphorylation site {S657}
Ser phosphorylation site {S698}
Ser phosphorylation site {S710}
MISC-INFO: LIGAND = GLUCOSE_6_PHOSPHATE
Database Correlations
OMIM 138570
MORBIDMAP 138570
UniProt P13807
Pfam PF05693
Kegg hsa:2997
ENZYME 2.4.1.11
References
- UniProt :accession P13807
- Textbook of Biochemistry with Clinical Correlations,
3rd ed., TM Devlin (ed), Wiley-Liss, NY 1992 pg 349