<prompt> Pompes disease is due to deficiency of:Acid maltaseMuscle phosphorylaseBranching enzymeDebranching enzyme  <response>: Pompes disease is: Type II glycogen storage disease It is the only GSD which is a Lysosomal Storage Disease. Due to deficiency of acid maltase enzyme also known as Acid a 1 - 4Glucosidase. This enzyme is present in lysosomes, catalyzes the minor pathway of glycogen breakdown. Patient has: hyperophic cardiomyopathy progressive skeletal myopathy Death occurs within 2 years due to cardiac failure The muscle biopsy show vacuoles. The treatment for Pompe disease is recombinant enzyme alglucosidase alfa Myozyme. 