期刊
ANTIOXIDANTS & REDOX SIGNALING
卷 17, 期 9, 页码 1277-1330出版社
MARY ANN LIEBERT, INC
DOI: 10.1089/ars.2011.4328
关键词
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资金
- ALS Association
- MIUR (PRIN)
- AISLA
- IMI-San Paolo
- Fondation Thierry-Latran
- ERA-Net Neuron
- Association Francaise contre les Myopathies
- AriSLA
Recent years have witnessed a renewed interest in the pathogenic mechanisms of amyotrophic lateral sclerosis (ALS), a late-onset progressive degeneration of motor neurons. The discovery of new genes associated with the familial form of the disease, along with a deeper insight into pathways already described for this disease, has led scientists to reconsider previous postulates. While protein misfolding, mitochondrial dysfunction, oxidative damage, defective axonal transport, and excitotoxicity have not been dismissed, they need to be re-examined as contributors to the onset or progression of ALS in the light of the current knowledge that the mutations of proteins involved in RNA processing, apparently unrelated to the previous old partners, are causative of the same phenotype. Thus, newly envisaged models and tools may offer unforeseen clues on the etiology of this disease and hopefully provide the key to treatment. Antioxid. Redox Signal. 17, 1277-1330.
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