Mechanisms of selective motor neuron death in ALS: insights from transgenic mouse models of motor neuron disease

Abstract excerpt

Concerning the mechanism(s) of disease underlying amyotrophic lateral sclerosis (ALS), transgenic mouse models have provided (i) a detailed look at the pathogenic progression of disease, (ii) a tool for testing hypotheses concerning the mechanism of neuronal death, and (iii) a host appropriate for testing therapeutic strategies. Thus far, these efforts have proved that mutation in a neurofilament subunit can...

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