Journal
JOURNAL OF BIOENERGETICS AND BIOMEMBRANES
Volume 43, Issue 6, Pages 593-599Publisher
SPRINGER/PLENUM PUBLISHERS
DOI: 10.1007/s10863-011-9394-z
Keywords
Amyotrophic lateral sclerosis; Mitochondria; SOD1; Motor neuron
Categories
Funding
- ALS Association
- Telethon
- MIUR
- IMI-San Paolo
- Fondation Thierry-Latran
- ERA-Net Neuron
- Association Fran aise contre les Myopathies
- AriSLA
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Mutant Cu,Zn superoxide dismutase (mutSOD1) is found in a subset of patients with familial amyotrophic lateral sclerosis (ALS), a fatal progressive paralysis due to loss of motor neurons. In the present article, we review existing evidence linking the expression of mutSOD1 to the many facets of mitochondrial dysfunction in ALS, with a focus on recent studies suggesting that the association and misfolding of the mutant protein (and possibly of the wild type protein as well) within these organelles is causally linked to their functional and structural alterations. Energy deficit, calcium mishandling and oxidative stress are paralleled by alteration in mitochondrial motility, dynamics and turnover and most probably lead to mitochondria-dependent cell death. Thus, the development of new, selective mitochondria-targeted therapies may constitute a promising approach in the treatment of SOD1-linked ALS.
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