((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
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
9691087 Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0

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

J Clin Invest 1998
2961257 Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease.

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

Am J Hum Genet 1988
Participants
Participates
Catalyst Activity

amylo-alpha-1,6-glucosidase activity of AGL [cytosol]

Orthologous Events
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