glycogen synthase, muscle (GYS1)
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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
More general terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/P13807.html
- ↑ Textbook of Biochemistry with Clinical Correlations, 3rd ed., TM Devlin (ed), Wiley-Liss, NY 1992 pg 349