کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8343677 | 1541552 | 2017 | 4 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Renal endoplasmic reticulum stress is coupled to impaired autophagy in a mouse model of GSD Ia
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موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: Renal endoplasmic reticulum stress is coupled to impaired autophagy in a mouse model of GSD Ia Renal endoplasmic reticulum stress is coupled to impaired autophagy in a mouse model of GSD Ia](/preview/png/8343677.png)
چکیده انگلیسی
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.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Molecular Genetics and Metabolism - Volume 122, Issue 3, November 2017, Pages 95-98
Journal: Molecular Genetics and Metabolism - Volume 122, Issue 3, November 2017, Pages 95-98
نویسندگان
Benjamin L. Farah, Dustin J. Landau, Yajun Wu, Rohit A. Sinha, Alwin Loh, Boon-Huat Bay, Dwight D. Koeberl, Paul M. Yen,