Cori Disease (Type III)
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Glycogen Storage Diseases
Cori Disease (Type III)
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Cori Disease, also called glycogen storage disease type III (GSD-III) or Forbes disease, is an autosomal recessive glycogen storage disease caused by a deficiency of an enzyme called glycogen debranching enzyme, also called alpha-1,6-glucosidase.
The deficiency of debranching enzyme leads to the accumulation of branched glycogen chains (limit dextrins) in the lysosomes of cells of various organs. Symptoms include hepatomegaly and hypoglycemia.
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