This review attempts to reconcile the present dual view of the mechanisms operating in Amyotrophic Lateral Sclerosis (ALS). On one side, oxidative stress, mitochondrial damage and protein aggregation are considered as causative of the disease, as strongly supported by evidence obtained in models based on the expression of ALS-typical mutant SOD1. On the other hand, evidence from models expressing ALS-typical mutations in RNA-binding proteins such as FUS and TDP43 indicate that mRNA (dys)metabolism is a major pathway in this disease. A critical analysis of existing literature suggests that there may be more than one point of intersection.

Bozzo, F., Mirra, A., Carri', M.t. (2017). Oxidative stress and mitochondrial damage in the pathogenesis of ALS: New perspectives. NEUROSCIENCE LETTERS, 636, 3-8 [10.1016/j.neulet.2016.04.065].

Oxidative stress and mitochondrial damage in the pathogenesis of ALS: New perspectives

BOZZO, FRANCESCA;MIRRA, ALESSIA;CARRI', MARIA TERESA
2017-01-01

Abstract

This review attempts to reconcile the present dual view of the mechanisms operating in Amyotrophic Lateral Sclerosis (ALS). On one side, oxidative stress, mitochondrial damage and protein aggregation are considered as causative of the disease, as strongly supported by evidence obtained in models based on the expression of ALS-typical mutant SOD1. On the other hand, evidence from models expressing ALS-typical mutations in RNA-binding proteins such as FUS and TDP43 indicate that mRNA (dys)metabolism is a major pathway in this disease. A critical analysis of existing literature suggests that there may be more than one point of intersection.
2017
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore BIO/10 - BIOCHIMICA
English
ALS; FUS/TLS; Iron; Mitochondria; Oxidative stress; RNA metabolism; SOD1; TDP43
Bozzo, F., Mirra, A., Carri', M.t. (2017). Oxidative stress and mitochondrial damage in the pathogenesis of ALS: New perspectives. NEUROSCIENCE LETTERS, 636, 3-8 [10.1016/j.neulet.2016.04.065].
Bozzo, F; Mirra, A; Carri', Mt
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/174185
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