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 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'.
Institute for Translational Pharmacology, CNR, Rome, Italy.
Mauro
M
Cozzolino
Alberto
A
Ferri
Cristiana
C
Valle
Maria Teresa
MT
Carrì
D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D004268:DNA-Binding Proteins / Q000235:genetics / Q000378:metabolism; D050259:Genes, Mitochondrial; D006801:Humans; D008928:Mitochondria / Q000235:genetics / Q000378:metabolism*; D034702:RNA-Binding Protein FUS / Q000235:genetics / Q000378:metabolism
D004268:DNA-Binding Proteins; D034702:RNA-Binding Protein FUS