Standard Article

Glycogen Storage Diseases

  1. TM Cox

Published Online: 22 MAR 2002

DOI: 10.1038/npg.els.0002270

eLS

eLS

How to Cite

Cox, T. 2002. Glycogen Storage Diseases. eLS. .

Author Information

  1. University of Cambridge, Cambridge, UK

Publication History

  1. Published Online: 22 MAR 2002

Abstract

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.

Keywords:

  • glycogen;
  • storage;
  • metabolism;
  • muscle;
  • liver;
  • starvation;
  • hypoglycaemia;
  • glycogenolysis