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Glycogen storage disease

Glycogen storage disease
Classification & external resources
ICD-10 E74.0
ICD-9 271.0
MeSH D006008

Glycogen storage disease (synonyms: glycogenosis, dextrinosis) is any one of several inborn errors of metabolism that result from enzyme defects that affect the processing of glycogen synthesis or breakdown within muscles, liver, and other cell types.


There are nine diseases that are commonly considered to be glycogen storage diseases. (Although glycogen synthase deficiency does not result in storage of extra glycogen in the liver, it is often classified with the GSDs as type 0 because it is another defect of glycogen storage and can cause similar problems.)

Number Enzyme deficiency Eponym
GSD type I glucose-6-phosphatase von Gierke's disease
GSD type II acid maltase Pompe's disease
GSD type III glycogen debrancher Cori's disease or Forbe's disease
GSD type IV glycogen branching enzyme Andersen disease
GSD type V muscle glycogen phosphorylase McArdle disease
GSD type VI liver glycogen phosphorylase Hers' disease
GSD type VII muscle phosphofructokinase Tarui's disease
GSD type IX phosphorylase kinase -
GSD type XI glucose transporter Fanconi-Bickel disease
GSD type 0 glycogen synthase -
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Glycogen_storage_disease". A list of authors is available in Wikipedia.
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