Glycogen Storage Diseases
Published Online: 22 MAR 2002
Copyright © 2001 John Wiley & Sons, Ltd. All rights reserved.
How to Cite
Cox, T. 2002. Glycogen Storage Diseases. eLS. .
- Published Online: 22 MAR 2002
Glycogen is used as an almost universal energy store in animal tissues; its complex molecular structure requires the interaction of many biosynthetic and degradative enzymes, whose action is finely coordinated and subject to rigorous metabolic regulation. Numerous diseases, each with a particular clinical phenotype, result from inherited defects in the metabolism of glycogen; many of these lead to the storage of abnormal glycogen molecules.