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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.
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. https://doi.org/10.1016/j.ymgme.2017.08.013||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||URI:||https://scholarbank.nus.edu.sg/handle/10635/163988||ISSN:||10967192||DOI:||10.1016/j.ymgme.2017.08.013|
|Appears in Collections:||Staff Publications|
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