[Experimental models of amyotrophic lateral sclerosis]

Amyotrophic lateral sclerosis (ALS) is a serious neurodegenerative disease that affects almost selectively motor neurons. Its ethiopathogeny is not fully understood, although there are several mechanisms that could play a role. It has no curative treatment and just a drug (riluzole) and mechanical ventilation has demonstrated to improve survival of these patients. In the last decades experimental models have been developed which have led us to better understand this disease and to design possible therapeutic strategies.

We reviewed published articles concerning experimental models for ALS and neurodegeneratives diseases using the PubMed database.

Several experimental models for ALS have been described, from animal models (mainly transgenic animals for human mutations in superoxidedismutase [SOD1]) to cellular models, each of them with advantages and objections.

ALS experimental models have implied a great advance in the knowledge of this disease and the design of new therapeutic strategies.