Glycogenosis type VII

Phosphofructokinase is the enzyme deficient in glycolysis leading to glycogenosis type VII (GSD VII, M. Tarui, MIM 232 800). The enzyme in its active form is a tetra-mer, composed of three different subunits M (muscle), L (liver), and P or F (platelets, fibroblasts). The enzyme is found in different compositions in different tissues, as shown in Table 4.6.16. In glycogenosis type VII the enzyme deficiency can only be determined in muscle, because only the deficiency of the M form leads to GSD VII. 

For type VII glycogenosis (Prob. 13.2) explain the likely symptoms due to this inborn error of metabolism, and give the nature of the accumulated glycogen. 

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