4.7 Review

Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic

Journal

PROGRESS IN NEUROBIOLOGY
Volume 133, Issue -, Pages 1-26

Publisher

PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.pneurobio.2015.07.004

Keywords

Amyotrophic lateral sclerosis; Motoneurons disease; Neurodegeneration; Neuromuscular junction; Neuroprotection

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Funding

  1. CIBERNED
  2. TERCEL
  3. Instituto de Salud Carlos III of Spain
  4. Fundacio La Marato-TV3, Catalunya, Spain [TV3201428-10]
  5. Ministerio de Educacion of Spain

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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. (C) 2015 Elsevier Ltd. All rights reserved.

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