MUSCLE PHYSIOLOGY

Detailed explanation-1: -Alpha 1, 6 glucosidase removes the remaining glucose molecule by cleaving the alpa 1, 6 glycosidic bond. In the final step, an enzyme called phosphoglucomutase converts the glucose 1-phosphate into glucose 6-phosphate.

Detailed explanation-2: -Debranching enzyme is a single polypeptide with two catalytic sites, amylo-1, 6-glucosidase and 4-alpha-glucanotransferase. Patients with debrancher deficiency are classified into four types.

Detailed explanation-3: -Debranching enzyme is a single 1532 aminoacid chain weighing about 165 kDa and consisting of two independent catalytic activities: oligo-1, 4-1, 4-glucantransferase [EC 2.4. 1.25] and amylo-1, 6-glucosidase [EC 3.2.

Detailed explanation-4: -In the lysosomes, the breakdown of glycogen is accomplished by the lysosomal enzyme acid -glucosidase or acid maltase (Fig. 4). Glycogen degradation in the cytosol and glycogen storage diseases.