Phosphorylated GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1

Stable Identifier
R-HSA-3322041
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
8 UDP-glucose + ((1,4)-alpha-D-glucosyl)4 glycogenin => 8 UDP + ((1,4)-alpha-D-glucosyl)8 glycogenin (GYS1-b)
ReviewStatus
5/5
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Phosphorylated glycogen synthase 1 (GYS1-b) complexed with oligo-D-glucose-GYG1 catalyzes the polyglucosylation of the latter. (Here the addition of four glucose residues is annotated.) This reaction is allosterically activated by glucose-6-phosphate (G6P) (Cameron et al. 2009; Roach et al. 2012). Glucose-6-phosphate (G6P) activates the phosphorylated ("b") form of GYS1 ("muscle") glycogen synthase (Roach et al. 2012).
Literature References
PubMed ID Title Journal Year
19699667 Identification of a novel mutation in GYS1 (muscle-specific glycogen synthase) resulting in sudden cardiac death, that is diagnosable from skin fibroblasts

Utgikar, R, Robinson, BH, Cameron, JM, Chiasson, D, Halliday, W, Ackerley, CA, Raiman, J, Mackay, N, Levandovskiy, V

Mol. Genet. Metab. 2009
22248338 Glycogen and its metabolism: some new developments and old themes

Hurley, TD, Roach, PJ, Tagliabracci, VS, DePaoli-Roach, AA

Biochem. J. 2012
Participants
Participates
Catalyst Activity

glycogen (starch) synthase activity of oligoGlc-GYG1:GYS1-b tetramer [cytosol]

This event is regulated
Orthologous Events
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