Transgenic-Mouse Model of Amyotrophic Lateral Sclerosis

Abstract

To the Editor: The article by Dr. Brown (Oct. 20 issue)1 highlights the studies that my colleagues and I have performed with a transgenic model of human motor neuron disease.2 Our experiments built on the studies of Dr. Brown and other members of a multicenter group that identified mutations of copper-zinc superoxide dismutase in some patients with a dominantly inherited form of amyotrophic lateral sclerosis.3 In mice expressing mutant forms of human copper-zinc superoxide dismutase a progressive paralytic disease develops that causes death at four to five months of age. Symptoms are accompanied by vacuolar changes, mitochondrial cytopathologic changes, alterations . . .