Forbe&;s disease is due to deficiency of
Glycogen storage disease type III is an autosomal recessive metabolic disorder and inborn error of metabolism characterized by a deficiency in glycogen debranching enzymes. It is also known as Cori&;s disease in honor of the 1947 Nobel laureates Carl Cori and Gey Cori. Other names include Forbes disease in honor of clinician Gilbe Burnett Forbes (1915-2003), an American Physician who fuher described the features of the disorder, or limit dextrinosis, due to the limit dextrin-like structures in cytosol. Limit dextrin is the remaining polymer produced after hydrolysis of glycogen. Without glycogen debranching enzymes to fuher conve these branched glycogen polymers to glucose, limit dextrinosis abnormally accumulates in the cytoplasm.Ref: DM Vasudevan, 7th edition, page no: 129
Branching enzyme
Debranching enzyme
Myophosphorylase
Hepatic phosphorylase
Metabolism of carbohydrate