Storage of Mutant Human SOD1 in Non-Neural Cells from the Type-1 Amyotrophic Lateral Sclerosis rat<sup>G93A</sup> Model Correlated with the Lysosomes’ Dysfunction

Storage of Mutant Human SOD1 in Non-Neural Cells from the Type-1 Amyotrophic Lateral Sclerosis rat<sup>G93A</sup> Model Correlated with the Lysosomes’ Dysfunction

Herein, we explored the impact of the lysosome dysfunction during the progression of Amyotrophic Lateral Sclerosis type-1 (ALS1). We conducted the study in non-neural cells, primary fibroblasts (rFFFs), and bone marrow-mesenchymal stem cells (rBM-MSCs), isolated from the animal model rat<sup>G...

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Bibliographic Details
Main Authors: Ilaria Bicchi, Francesco Morena, Chiara Argentati, Laura Rota Nodari, Carla Emiliani, Maurizio Gelati, Angelo L. Vescovi, Sabata Martino
Format: Article
Language:English
Published: MDPI AG 2021-08-01
Series:Biomedicines
Subjects:
Hexosaminidase
GALC
LC3
autophagy
mutant SOD1 lysosomal storage
lysosomal storage disorders
Online Access:https://www.mdpi.com/2227-9059/9/9/1080

Internet

https://www.mdpi.com/2227-9059/9/9/1080

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