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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Sprache:eng
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Zusammenfassung: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'.
ISSN:1044-7431
1095-9327
DOI:10.1016/j.mcn.2012.06.001