Document Type
Article
Publication Date
1-1-2020
Abstract
Motor neuron diseases (MNDs) are fatal diseases characterized by loss of motor neurons in the brain cortex, in the bulbar region, and/or in the anterior horns of the spinal cord. While generally sporadic, inherited forms linked to mutant genes encoding altered RNA/protein products have also been described. Several different mechanisms have been found altered or dysfunctional in MNDs, like the protein quality control (PQC) system. In this review, we will discuss how the PQC system is affected in two MNDs-spinal and bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS)-and how this affects the clearance of aberrantly folded proteins, which accumulate in motor neurons, inducing dysfunctions and their death. In addition, we will discuss how the PQC system can be targeted to restore proper cell function, enhancing the survival of affected cells in MNDs.
Recommended Citation
Cristofani, Riccardo; Crippa, Valeria; Cicardi, Maria Elena; Tedesco, Barbara; Ferrari, Veronica; Chierichetti, Marta; Casarotto, Elena; Piccolella, Margherita; Messi, Elio; Galbiati, Mariarita; Rusmini, Paola; and Poletti, Angelo, "A Crucial Role for the Protein Quality Control System in Motor Neuron Diseases." (2020). Department of Neuroscience Faculty Papers. Paper 47.
https://jdc.jefferson.edu/department_neuroscience/47
Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.
Language
English
Comments
This is the final published version of this article from Frontiers in Aging Neuroscience, 2020, 12: 191.
The full text of the article can also be accessed at http://doi.org/10.3389/fnagi.2020.00191
Copyright Christofani et. al.