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Glycogen Storage Diseases (GSD): An Overview

Introduction

There are fifteen distinct glycogen storage diseases. Although glycogen synthase deficiency does not result in storage of extra glycogen in the liver, it is usually classified with the GSD Type 0 because it is another defect of glycogen storage and can cause similar problems. The overall frequency of glycogen-storage disease has been established approximately 1:20,000 to 25,000 people worldwide.

TABLE G-3Glycogen Storage Disease (GSD): An Overview
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TABLE G-3 Glycogen Storage Disease (GSD): An Overview
Deficient Enzymatic
GSD no. Activity Eponym
I Glucose-6- phosphatase I Von Gierke disease
II Alpha 1,4-glucosidase (acid maltase) Pompe disease
III Debrancher (amylo-1,6 glucosidase) Cori disease
IV Brancher Andersen disease
V Muscle phosphorylase McArdle disease
VI Liver phosphorylase Hers disease
VII Phosphofructokinase Tarui disease
VIII Phosphorylase kinase of liver  

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