Reactome | Phosphorylated GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1

Phosphorylated GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1

Stable Identifier

R-HSA-3322041

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

((1->4)-alpha-D-glucosyl)(n) + UDP-alpha-D-glucose => ((1->4)-alpha-D-glucosyl)(n+1) + UDP + H(+), 8 UDP-glucose + ((1,4)-alpha-D-glucosyl)4 glycogenin => 8 UDP + ((1,4)-alpha-D-glucosyl)8 glycogenin (GYS1-b)

ReviewStatus

4/5

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Phosphorylated GYS1 catalyzes the polyglucosylation of oligoGlc-GYG1

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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 Cameron, JM, Levandovskiy, V, Mackay, N, Utgikar, R, Ackerley, CA, Chiasson, D, Halliday, W, Raiman, J, Robinson, BH	Mol. Genet. Metab.	2009
22248338	Glycogen and its metabolism: some new developments and old themes Roach, PJ, DePaoli-Roach, AA, Hurley, TD, Tagliabracci, VS	Biochem. J.	2012