Reactome | GYS2 catalyzes the incorporation of phosphoglucose into glycogen-GYG2

GYS2 catalyzes the incorporation of phosphoglucose into glycogen-GYG2

Stable Identifier

R-HSA-3780994

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

ReviewStatus

4/5

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GYS2 catalyzes the incorporation of phosphoglucose into glycogen-GYG2

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Glycogen synthase 2 (GYS2) catalyzes the incorporation of phosphoglucose into the glycogen-GYG1 molecules with which it is associated in a cytosolic glycogen granule. This reaction occurs at a low rate, yielding approximately one molecule of glucose phosphorylated at its C2, C3, or C6 positions incorporated into a growing glycogen polymer per ten thousand glucose molecules incorporated (DePaoli-Roach et al. 2015; Irimia et al. 2015; Nitschke et al. 2013; Tagliabracci et al. 2011). The function of these small amounts of phosphoglucose in normal glycogen remains to be established. This reaction has been characterized in muscle cells, where it is catalyzed by the homologous GYS1 enzyme. The occurrence of the reaction in liver, catalyzed by GYS2, can be inferred from the fact that in the absence of the enzyme EMP2A (laforin) that removes these phosphate groups, abnormally phosphorylated glycogen accumulates in both tissues (Worby et al. 2008).

Literature References

PubMed ID	Title	Journal	Year
25416783	Glycogen phosphomonoester distribution in mouse models of the progressive myoclonic epilepsy, Lafora disease DePaoli-Roach, AA, Contreras, CJ, Segvich, DM, Heiss, C, Ishihara, M, Azadi, P, Roach, PJ	J. Biol. Chem.	2015
18070875	Malin decreases glycogen accumulation by promoting the degradation of protein targeting to glycogen (PTG) Worby, CA, Gentry, MS, Dixon, JE	J. Biol. Chem.	2008
23663739	Hyperphosphorylation of glucosyl C6 carbons and altered structure of glycogen in the neurodegenerative epilepsy Lafora disease Nitschke, F, Wang, P, Schmieder, P, Girard, JM, Awrey, DE, Wang, T, Israelian, J, Zhao, X, Turnbull, J, Heydenreich, M, Kleinpeter, E, Steup, M, Minassian, BA	Cell Metab.	2013
21356517	Phosphate incorporation during glycogen synthesis and Lafora disease Tagliabracci, VS, Heiss, C, Karthik, C, Contreras, CJ, Glushka, J, Ishihara, M, Azadi, P, Hurley, TD, DePaoli-Roach, AA, Roach, PJ	Cell Metab.	2011
26216881	Muscle Glycogen Remodeling and Glycogen Phosphate Metabolism following Exhaustive Exercise of Wild Type and Laforin Knockout Mice Irimia, JM, Tagliabracci, VS, Meyer, CM, Segvich, DM, DePaoli-Roach, AA, Roach, PJ	J. Biol. Chem.	2015