Glycogen storage disease type III

Glycogen storage disease type III, abbreviated GSD III, is an autosomal recessive metabolic disorder and an inborn error of carbohydrate metabolism, characterised by a deficiency of the glycogen debranching enzyme. It is also called Cori disease, after the 1947 Nobel laureates Carl and Gerty Cori, Forbes disease, after the American physician Gilbert Burnett Forbes who further described its features, and limit dextrinosis, after the limit dextrin-like structures that accumulate in the cytosol. Because the debrancher enzyme, amylo-1,6-glucosidase, is deficient, abnormal glycogen is deposited in the liver, the muscles and in some cases the heart. This entry follows the lead of the cited English Wikipedia article and is not medical advice.