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Glycogen storage disease is any one of several metabolic disorders that result from enzyme defects that affect the processing of glycogen within muscles, liver, and other cell types.

There are eight diseases that are commonly considered to be glycogen storage diseases:

GSD type I: glucose-6-phosphatase deficiency , von Gierke's disease
GSD type II: acid maltase deficiency , Pompe's disease
GSD type III: debrancher deficiency
GSD type IV: Andersen disease
GSD type V: McArdle disease
GSD type VI: liver phosphorylase deficiency
GSD type VII: muscle phosphofructokinase deficiency
GSD type IX: phosphorylase kinase deficiency

GSD type 0: glycogen synthase deficiency

Although glycogen synthase deficiency does not result in storage of extra glycogen in the liver, it is often classified with the GSDs because it is another defect of glycogen storage and can cause similar problems.

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Topics: Glycogen Storage Disease, Glycogenolysis...

Glycogen storage diseaseGlycogen storage disease is any one of several metabolic disorders that result from enzyme defects that affect the processing of glycogen within muscles, liver, and other cell types. There are eight diseases that are commonly considered to be glycogen sto

GlycogenGlycogen is the principal storage form of glucose in animal cells. In humans and other vertebrates, most glycogen is found in the liver (10% of the liver mass), giving it a distinctive, "starchy" taste. Muscles contain a relatively low amount of glycogen

GlycogenolysisGlycogenolysis is the catabolism of glycogen (requiring removal of glucose unit from glycogen and addition of phosphate) thus producing glucose-1-phosphate. Glycogenolysis requires three enzymes : Phosphorylase Glucan Transferase Debranching enzyme.

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