The role and therapeutic potential of the integrated stress response in amyotrophic lateral sclerosis
Keywords: 
Amyotrophic lateral sclerosis
Integrated stress response
Translation
uORFs-containing mRNAs
ALS experimental models
RNA-binding proteins
ALS clinical trials
Issue Date: 
2022
Publisher: 
MDPI AG
Project: 
info:eu-repo/grantAgreement/AEI/Plan Estatal de Investigación Científica y Técnica y de Innovación 2017-2020/PID2020-120497RB-I00/ES/ESTUDIO DEL MECANISMO Y POTENCIAL TERAPEUTICO DEL PROCESAMIENTO Y TRADUCCION DE MARNS EN RESPUESTA ESTRES EN LA ESCLEROSIS LATERAL AMIOTROFICA
ISSN: 
1422-0067
Note: 
This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ 4.0/)
Citation: 
Marlin, E. (Elías); Viu-Idocin, C. (Cristina); Arrasate, M. (Montserrat); et al. "The role and therapeutic potential of the integrated stress response in amyotrophic lateral sclerosis". Molecular sciences. 23 (14), 2022, 7823
Abstract
In amyotrophic lateral sclerosis (ALS) patients, loss of cellular homeostasis within cortical and spinal cord motor neurons triggers the activation of the integrated stress response (ISR), an intracellular signaling pathway that remodels translation and promotes a gene expression program aimed at coping with stress. Beyond its neuroprotective role, under regimes of chronic or excessive stress, ISR can also promote cell/neuronal death. Given the two-edged sword nature of ISR, many experimental attempts have tried to establish the therapeutic potential of ISR enhancement or inhibition in ALS. This review discusses the complex interplay between ISR and disease progression in different models of ALS, as well as the opportunities and limitations of ISR modulation in the hard quest to find an effective therapy for ALS.

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