During the past several years, substantial progress has been made in understanding the molecular events that underlie motor neuron disease. Significantly, several genes and a variety of loci have been linked to motor neuron disease. Free radical-mediated chemistries, copper toxicity, neurofilament biology, axonal transport, RNA metabolism, excitotoxicity, protein folding/aggregation, etc. appear to influence the function/ viability of neurons. The mechanisms that account for "selective" neuronal degeneration are uncertain. However, the identification of specific genes/proteins that are mutated/deleted in the inherited forms of the disease has allowed investigators to create in vivo and in vitro model systems. For example, transgenic mice that recapitulate some of the features of human diseases have provided important information about the biology of these diseases. The availability of new models will allow investigators to examine the molecular mechanisms by which mutant proteins cause selective dysfunction/death of motor neurons. Moreover, pathogenic hypotheses can be tested by experimental manipulations and by breeding mice carrying mutant genes to mice that express other transgenes or to gene-targeted mice. The results of these approaches should provide a better understanding of the pathogenic mechanisms of disease. In turn, this new knowledge should lead to the design of novel therapeutic strategies that can be tested in these animal models.

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