((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)glycogenin => poly{(1,4)-alpha-glucosyl} glycogenin + alpha-D-glucose

Stable Identifier
R-HSA-71593
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
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At the beginning of this reaction, 1 molecule of '{(1,6)-alpha-glucosyl}poly{(1,4)-alpha-glucosyl}glycogenin-2' is present. At the end of this reaction, 1 molecule of 'alpha-D-glucose', and 1 molecule of 'poly{(1,4)-alpha-glucosyl}glycogenin-2' are present.

This reaction takes place in the 'cytoplasm' and is mediated by the 'amylo-alpha-1,6-glucosidase activity' of 'glycogen debranching enzyme'.

Literature References
PubMed ID Title Journal Year
2961257 Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease.

Chen, Y-T, He, JK, Ding, JH, Brown, BI

Am J Hum Genet 1988
9691087 Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0

Orho, M, Bosshard, NU, Buist, NR, Gitzelmann, R, Aynsley-Green, A, Blumel, P, Gannon, MC, Nuttall, FQ, Groop, LC

J Clin Invest 1998
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
amylo-alpha-1,6-glucosidase activity of AGL [cytosol]
Physical Entity
Activity
Orthologous Events