Motor neuron disease in vitro: the use of cultured motor neurons to study amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease i n which motor neurons in the nervous system die. The cause is unknown, and no effective treatment exists. Mutations in the gene for superoxide dismuta se found in a subpopulation have led to an animal model, but research with these mice has not produced complete insight into the disease mechanism. St udies with isolated motor neurons may produce important information. This r eview discusses approaches to culture motor neurons - single cells, cocultu red with other cells, and in intact preparations, such as the spinal or cor tical slice. Motor neurons in monoculture are suitable for acute but not fo r chronic studies, whereas cocultures and slices survive up to months and a re used for chronic studies. Results with toxic substances believed to play a role in the disease, such as oxidants and glutamate, and of studies wher e the energy status of the cells is manipulated, are presented. (C) 2000 El sevier Science B.V. All rights reserved.