Glycogen Storage Diseases


Book Section

© 2014 Elsevier Inc. All rights reserved. Glycogen is the principal storage form of carbohydrate in animal cells and is present virtually in every tissue of the body. Glycogen storage diseases (GSDs) (glycogenoses) are inborn metabolic disorders characterized by defects in enzymes or transport proteins that affect glycogen metabolism. The glycogen found in these disorders is abnormal in quantity, quality, or both. At present, more than 12 recognized glycogenoses are categorized either numerically in chronological accordance with their discovery or by organ involvement/clinical manifestations (liver or muscle). Hepatic GSDs include types I, III, IV, VI, IX, and XI. Muscle GSDs include types II, III, V, VII, and IX.

Full Text

Duke Authors

Cited Authors

  • Kishnani, PS

Published Date

  • January 1, 2014

Book Title

  • Encyclopedia of the Neurological Sciences

Start / End Page

  • 454 - 459

International Standard Book Number 13 (ISBN-13)

  • 9780123851581

Digital Object Identifier (DOI)

  • 10.1016/B978-0-12-385157-4.00083-X

Citation Source

  • Scopus