Understanding Von Gierke's Disease

Von Gierke’s Disease, also called Type I glycogen storage disease (GSDI), is when the body cannot make the enzyme glucose – 6 – phosphatase and as well is deficient in it. Glucose -6- phosphatase is an enzyme that is produced in the liver and is needed for glycogen breakdown. It catalyzes and removes the phosphate group from Glucose-6-phosphate. It functions as a catalyzing enzyme, which means that it helps with production. Without breakdown of glycogen, the body is unable to make energy. It is a disease that occurs in the liver. It is an inherited autosomal recessive disease, which means that it is passed down through families, and two copies of the abnormal gene must be presented for the disease to be developed. In the cells of the body,