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Altered autophagy in amyotrophic lateral sclerosis: mechanisms and therapeutic opportunities? (pp.211-242) $100.00
Authors:  (Jean-Paul Decuypere, Kim A. Staats, Kirsten Welkenhuyzen, Ludwig Missiaen, Humbert De Smedt, Ludo Van Den Bosch, Jan B. Parys, Geert Bultynck)
Amyotrophic lateral sclerosis (ALS) is caused by selective degeneration of upper and lower motor neurons, resulting in muscle weakness, paralysis and death of the patient on average 2 to 5 years after diagnosis. In the majority of ALS cases (~90 %) no clear hereditary component is present (sporadic ALS), while the remaining ~10 % of the cases have a familial form (fALS). Most studies have used transgenic mice expressing mutant superoxide dismutase 1 (SOD1) as a model system for the in vivo study of ALS. Clearly, ALS is a multi-factorial disease and one of the best characterized hallmarks is the accumulation of toxic mutant protein aggregates and damaged organelles. These are key features of many neurodegenerative diseases, including ALS. Several of those neurodegenerative diseases are characterized by alterations in autophagic activity, leading
to an impaired clearance of toxic protein aggregates and/or of damaged mitochondria. In mouse models for neurodegeneration, chemical stimulation of autophagic flux has been shown to be beneficial. Stimulation of autophagy in mice resulted in neuroprotective effects, delayed aging and increase in life span. Since there is as yet no effective treatment for ALS, targeting the altered autophagy pathway could be a promising therapeutic strategy. Initial studies in mutant-SOD1-mouse models pointed out that increasing autophagy activity may protect against motor neuron degradation by removing protein aggregates and damaged organelles. Other studies however, reported no therapeutic efficacy or even a faster progression of motor-neuron degeneration and a decrease in life span in presymptomatic mutant-SOD1 mice. Thus, the question arises whether autophagy stimulation is beneficial for the treatment of ALS. In particular, several defects in the autophagic pathway may complicate the outcome and are not fully characterized and understood for each of the different mutant genes associated with ALS. Here, we will critically address the current state-of-the-art knowledge on the role of autophagy in ALS and the modulation of autophagy activity as a potential therapeutic target in ALS. 

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Altered autophagy in amyotrophic lateral sclerosis: mechanisms and therapeutic opportunities? (pp.211-242)