Von Gierke disease
Alternate Names : Type I glycogen storage disease
Definition
Von Gierke disease is a condition in which the body cannot break down glycogen for energy. Gycogen is stored in the liver and muscles and is normally broken down into glucose when you do not eat. Von Gierke disease is also called Type I glycogen storage disease (GSD I).
Overview, Causes, & Risk Factors
Von Gierke disease occurs when the body lacks the protein (enzyme) that releases glucose from glycogen. This causes abnormal amounts of glycogen to build up in certain tissues. When glycogen is not broken down properly, it leads to low blood sugar. Von Gierke disease is inherited, which means it is passed down through families. If both parents carry the defective gene related to this condition, each of their children has a 25% chance of developing the disease.
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