Article ID Journal Published Year Pages File Type
4353256 Progress in Neurobiology 2015 26 Pages PDF
Abstract

•ALS is a neurodegenerative disease affecting upper and lower motoneurons.•Experimental models are instrumental in leading to translational research.•The review covers the diverse aspects of ALS physiopathology.•The most relevant and novel therapeutic strategies are summarized.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive degeneration of upper and lower motoneurons, leading to muscle weakness and paralysis, and finally death. Considerable recent advances have been made in basic research and preclinical therapeutic attempts using experimental models, leading to increasing clinical and translational research in the context of this disease. In this review we aim to summarize the most relevant findings from a variety of aspects about ALS, including evaluation methods, animal models, pathophysiology, and clinical findings, with particular emphasis in understanding the role of every contributing mechanism to the disease for elucidating the causes underlying degeneration of motoneurons and the development of new therapeutic strategies.

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Life Sciences Neuroscience Neuroscience (General)
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