Links between autophagy and disorders of glycogen metabolism - Perspectives on pathogenesis and possible treatments.

Journal Article (Journal Article;Review)

The glycogen storage diseases are a group of inherited metabolic disorders that are characterized by specific enzymatic defects involving the synthesis or degradation of glycogen. Each disorder presents with a set of symptoms that are due to the underlying enzyme deficiency and the particular tissues that are affected. Autophagy is a process by which cells degrade and recycle unneeded or damaged intracellular components such as lipids, glycogen, and damaged mitochondria. Recent studies showed that several of the glycogen storage disorders have abnormal autophagy which can disturb normal cellular metabolism and/or mitochondrial function. Here, we provide a clinical overview of the glycogen storage disorders, a brief description of autophagy, and the known links between specific glycogen storage disorders and autophagy.

Full Text

Duke Authors

Cited Authors

  • Farah, BL; Yen, PM; Koeberl, DD

Published Date

  • January 2020

Published In

Volume / Issue

  • 129 / 1

Start / End Page

  • 3 - 12

PubMed ID

  • 31787497

Pubmed Central ID

  • PMC7836271

Electronic International Standard Serial Number (EISSN)

  • 1096-7206

Digital Object Identifier (DOI)

  • 10.1016/j.ymgme.2019.11.005


  • eng

Conference Location

  • United States