Reactome | GBE1 catalyzes branch formation in polyGlc-GYG2 complexed with GYS2-a

GBE1 catalyzes branch formation in polyGlc-GYG2 complexed with GYS2-a

Stable Identifier

R-HSA-3322016

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

poly((1,4)-alpha-D-glucosyl) glycogenin2 => glycogen-glycogenin 2 (GYS2-a)

ReviewStatus

5/5

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GBE1 catalyzes branch formation in polyGlc-GYG2 complexed with GYS2-a

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Cytosolic glycogen branching enzyme (GBE1) associated with glycogen granules transfers terminal alpha(1,4) glucose blocks to form alpha(1,6) branches on growing glycogen molecules formed on glycogenin 2 (GYG2) complexed with unphosphorylated glycogen synthase 2 (GYS2-a) (Bao et al. 1996; Roach et al. 2012).

Literature References

PubMed ID	Title	Journal	Year
8613547	Hepatic and neuromuscular forms of glycogen storage disease type IV caused by mutations in the same glycogen-branching enzyme gene. Bao, Y, Kishnani, P, Wu, JY, Chen, Y-T	J Clin Invest	1996
22248338	Glycogen and its metabolism: some new developments and old themes Roach, PJ, DePaoli-Roach, AA, Hurley, TD, Tagliabracci, VS	Biochem. J.	2012