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

((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

cytosol

ReviewStatus

5/5

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

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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 Orho, M, Bosshard, NU, Buist, NR, Gitzelmann, R, Aynsley-Green, A, Blumel, P, Gannon, MC, Nuttall, FQ, Groop, LC	J Clin Invest	1998
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