A Crucial Role for the Protein Quality Control System in Motor Neuron Diseases.
BAG1
BAG3
CASA complex
HSPB8
motor neuron
protein quality control
Journal
Frontiers in aging neuroscience
ISSN: 1663-4365
Titre abrégé: Front Aging Neurosci
Pays: Switzerland
ID NLM: 101525824
Informations de publication
Date de publication:
2020
2020
Historique:
received:
24
03
2020
accepted:
02
06
2020
entrez:
15
8
2020
pubmed:
15
8
2020
medline:
15
8
2020
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 32792938
doi: 10.3389/fnagi.2020.00191
pmc: PMC7385251
doi:
Types de publication
Journal Article
Langues
eng
Pagination
191Informations de copyright
Copyright © 2020 Cristofani, Crippa, Cicardi, Tedesco, Ferrari, Chierichetti, Casarotto, Piccolella, Messi, Galbiati, Rusmini and Poletti.
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