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

Stable Identifier
R-HSA-3322005
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
poly((1,4)-alpha-D-glucosyl) glycogenin1 => glycogen-glycogenin 1 (GYS1-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 1 (GYG1) complexed with unphosphorylated glycogen synthase 1 (GYS1-a) (Bao et al. 1996; Roach et al. 2012).

Literature References
PubMed ID Title Journal Year
22248338 Glycogen and its metabolism: some new developments and old themes

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

Biochem. J. 2012
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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
1,4-alpha-glucan branching enzyme activity of GBE1 [cytosol]
Physical Entity
Activity
Orthologous Events
Reviewed
Created