Muscle cannot make use of glycogen for energy in the absence of
a. Glucose-6-phosphatase: Normally absent in muscle and present in liver Q and kidney so, organs affected in this are, Liver, Kidney and Intestine.
b. Muscle phosphorylase: 1st enzyme of glycogenolysisQ, Rate limiting step. Harrison -
→Deficiency of muscle phosphorylase is the prototype muscle-energy disorder. Deficiency of this enzyme in muscle limits ATP generation by glycogenolysis and result in glycogen accumulation.
C/f →Diminished muscle exercise tolerance; (= type V, Mc Ardle disease).