Tags: Disease.
Glycogen storage disease type I (GSD I) or von Gierke’s disease is the most common of the glycogen storage diseases. This genetic disease results from deficiency of the enzyme glucose-6-phosphatase and has an incidence in the American population of approximately 1 in 50000 to 100000 births.The deficiency impairs the ability of the liver to produce free glucose from glycogen and from gluconeogenesis.