Glycogen storage disease type 0 fructose results in a marked rise in blood lactate levels. Liver biopsy for microscopic analysis and enzyme assay is required for definitive diagnosis. Diagnosis may include linkage analysis in families with affected members and sequencing of the entire coding region of the GSY2 gene for mutations. Histologic analysis of liver tissue demonstrates moderately decreased amounts of periodic acid-Schiff (PAS)–positive, diastase-sensitive glycogen stores. Evidence of increased fat accumulation in the liver may be observed, as in other glycogen-storage diseases. Electron microscopic analysis of liver sections shows normal glycogen structure. Muscle glycogen stores are normal. There are two types of this\\e condition
Glucose of consumed food. Organisms use glucose as a precursor for the synthesis of several important substances. Starch, cellulose, and glycogen ("animal starch") are common glucose polymers (polysaccharides). Some of these polymers (starch or glycogen) serve as energy stores, while others (cellulose and chitin, which is made from a derivative of glucose) have structural roles. Oligosaccharides of glucose combined with other sugars serve as important energy stores. These include lactose, the predominant sugar in milk, which is a glucose-galactose disaccharide, and sucrose, another disaccharide which is composed of glucose and fructose. Glucose is also added onto certain proteins and lipids in