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Title: Renal endoplasmic reticulum stress is coupled to impaired autophagy in a mouse model of GSD Ia
Authors: Farah B.L.
Landau D.J.
Wu Y. 
Sinha R.A. 
Loh A. 
Bay B.-H. 
Koeberl D.D.
Yen P.M. 
Keywords: Autophagy
ER stress
Glycogen storage disease type I
Von Gierke's disease
Issue Date: 2017
Publisher: Academic Press Inc.
Citation: Farah B.L., Landau D.J., Wu Y., Sinha R.A., Loh A., Bay B.-H., Koeberl D.D., Yen P.M. (2017). Renal endoplasmic reticulum stress is coupled to impaired autophagy in a mouse model of GSD Ia. Molecular Genetics and Metabolism 122 (3) : 95-98. ScholarBank@NUS Repository.
Abstract: 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. � 2017 Elsevier Inc.
Source Title: Molecular Genetics and Metabolism
ISSN: 10967192
DOI: 10.1016/j.ymgme.2017.08.013
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