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Gycogen Storage Disease Definition & Overview

Definition

Glucose is a simple sugar. It is a form of carbohydrate. It is the main source of energy for our bodies. Glycogen is the storage form of glucose in our bodies.

Glycogen storage diseases (GSDs) are a group of inherited genetic disorders. They cause glycogen to be improperly formed or released in the body. This results in a build up of abnormal amounts or types of glycogen in tissues.

The main types of GSDs are categorized by number and name and include:

  • Type I (Von Gierke disease, defect in glucose-6-phosphatase)—most common type of GSD; accounts for 90% of all GSD cases
  • Type II (Pompe’s disease, acid maltase deficiency)
  • Type III (Cori’s disease, debrancher enzyme deficiency)
  • Type IV (Andersen’s disease, brancher enzyme deficiency)
  • Type V (McArdle’s disease, muscle glycogen phosphorylase deficiency)
  • Type VI (Hers’ disease, liver phosphorylase deficiency)
  • Type VII (Tarui’s disease, muscle phosphofructokinase deficiency)
  • Type IX (liver glycogen phosphorylase kinase deficiency)

Glycogen is mainly stored in the liver or muscle tissue. As a result, GSDs usually affect functioning of the liver, the muscles, or both.

  • Liver—The GSDs that mainly affect the liver are types I, III, IV, VI, and IX.
  • Muscles—The GSDs that mainly affect muscles are types V and VII.
  • Type II affects nearly all organs including the heart.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition. Copyright © 2024 EBSCO Publishing All rights reserved.

The Latest in Gycogen Storage Disease Definition & Overview

  • Glycogen Storage Diseases

    Encylopedia entry for Glycogen Storage Diseases.

    Glycogen Storage Diseases(Glycogenoses; GSD) by

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Gycogen Storage Disease Guide

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