Renal endoplasmic reticulum stress is coupled to impaired autophagy in a mouse model of GSD Ia.
Journal Article
GSD Ia (von Gierke Disease, Glycogen Storage Disease Type Ia) is a devastating genetic disorder with long-term sequelae, such as non-alcoholic fatty liver disease and renal failure. Down-regulated autophagy is involved in the development of hepatic metabolic dysfunction in GSD Ia; however, the role of autophagy in the renal pathology is unknown. Here we show that autophagy is impaired and endoplasmic reticulum (ER) stress is increased in the kidneys of a mouse model of GSD Ia. Induction of autophagy by rapamycin also reduces this ER stress. Taken together, these results show an additional role for autophagy down-regulation in the pathogenesis of GSD Ia, and provide further justification for the use of autophagy modulators in GSD Ia.
Full Text
Duke Authors
Cited Authors
- Farah, BL; Landau, DJ; Wu, Y; Sinha, RA; Loh, A; Bay, B-H; Koeberl, DD; Yen, PM
Published Date
- November 2017
Published In
Volume / Issue
- 122 / 3
Start / End Page
- 95 - 98
PubMed ID
- 28888852
Pubmed Central ID
- 28888852
Electronic International Standard Serial Number (EISSN)
- 1096-7206
Digital Object Identifier (DOI)
- 10.1016/j.ymgme.2017.08.013
Language
- eng
Conference Location
- United States