Substrate Reduction Therapy Reverses Mitochondrial, mTOR, and Autophagy Alterations in a Cell Model of Gaucher Disease

Substrate Reduction Therapy Reverses Mitochondrial, mTOR, and Autophagy Alterations in a Cell Model of Gaucher Disease

Substrate reduction therapy (SRT) in clinic adequately manages the visceral manifestations in Gaucher disease (GD) but has no direct effect on brain disease. To understand the molecular basis of SRT in GD treatment, we evaluated the efficacy and underlying mechanism of SRT in an immortalized neurona...

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Bibliographic Details
Main Authors: Yanyan Peng, Benjamin Liou, Yi Lin, Venette Fannin, Wujuan Zhang, Ricardo A. Feldman, Kenneth D. R. Setchell, Gregory A. Grabowski, Ying Sun
Format: Article
Language:English
Published: MDPI AG 2021-09-01
Series:Cells
Subjects:
lysosomal storage disorders
cell biology
neurons
glucosylceramide
glucosylsphingosine
Online Access:https://www.mdpi.com/2073-4409/10/9/2286

Internet

https://www.mdpi.com/2073-4409/10/9/2286

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