Glycogen-storage Diseases

Answer with enzyme, affected organ, and effect on glycogen storage

Type I (Von Gierke)

G-6-Phosphatase or transport; liver and kidney; increased glycogen with normal structure

Type II (Pompe)

alpha-1,4-glucosidase (lysosomal); all organs; massive increase with normal structure

Type III (Cori)

alpha-1,6-glucosidase (debranching); muscle and liver; normal amount with short branches

Type IV (Andersen)

Branching enzyme; liver and spleen; normal amount with long branches

Type V (McArdle)

phosphorylase; muscle; moderate increase with normal structure

Type VI (Hers)

phosphorylase; liver; increased amount

Type VII (Tarui)

phosphofructokinase; muscle; increased amount with normal structure

Type VIII

phosphorylate kinase; liver; increased amount with normal structure