GBE1 catalyzes branch formation in polyGlc-GYG1 complexed with GYS1-b

Stable Identifier
R-HSA-3322057
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
poly((1,4)-alpha-D-glucosyl) glycogenin1 => glycogen-glycogenin 1 (GYS1-b)
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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 1 (GYG1) complexed with phosphorylated glycogen synthase 1 (GYS1-b) (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.

Wu, JY, Chen, Y-T, Kishnani, P, Bao, Y

J Clin Invest 1996
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

1,4-alpha-glucan branching enzyme activity of GBE1 [cytosol]

Orthologous Events
Reviewed
Created
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