Mitochondria and ALS: Implications from novel genes and pathways

Evidence from patients with sporadic and familiar amyotrophic lateral sclerosis (ALS) and from models based on the overexpression of mutant SOD1 found in a small subset of patients, clearly point to mitochondrial damage as a relevant facet of this neurodegenerative condition. In this mini-review we...

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Veröffentlicht in:Molecular and cellular neuroscience 2013-07, Vol.55, p.44-49
Hauptverfasser: Cozzolino, Mauro, Ferri, Alberto, Valle, Cristiana, Carrì, Maria Teresa
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container_title Molecular and cellular neuroscience
container_volume 55
creator Cozzolino, Mauro
Ferri, Alberto
Valle, Cristiana
Carrì, Maria Teresa
description Evidence from patients with sporadic and familiar amyotrophic lateral sclerosis (ALS) and from models based on the overexpression of mutant SOD1 found in a small subset of patients, clearly point to mitochondrial damage as a relevant facet of this neurodegenerative condition. In this mini-review we provide a brief update on the subject in the light of newly discovered genes (such as TDP-43 and FUS/TLS) associated to familial ALS and of a deeper knowledge of the mechanisms of derangement of mitochondria. This article is part of a Special Issue entitled 'Mitochondrial function and dysfunction in neurodegeneration'.
doi_str_mv 10.1016/j.mcn.2012.06.001
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subjects Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - genetics
Amyotrophic Lateral Sclerosis - metabolism
Animals
DNA-Binding Proteins - genetics
DNA-Binding Proteins - metabolism
Genes, Mitochondrial
Humans
Mitochondria
Mitochondria - genetics
Mitochondria - metabolism
Oxidative stress
RNA-Binding Protein FUS - genetics
RNA-Binding Protein FUS - metabolism
title Mitochondria and ALS: Implications from novel genes and pathways
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