The use of transgenic mouse models of amyotrophic lateral sclerosis in preclinical drug studies
ME Gurney - Journal of the neurological sciences, 1997 - Elsevier
The discovery of mutations in the human SOD1 gene encoding Cu, Zn superoxide
dismutase (Cu, Zn SOD) in patients with familial amyotrophic lateral sclerosis (ALS) has
made possible the development of etiological models of the disease. Expression of mutant
SOD1 genes in transgenic mice causes a progressive paralytic disease whose general
features resemble ALS in humans. We have used the transgenic model to explore
etiological mechanisms and to screen potential therapeutics. Our results and those of others …
dismutase (Cu, Zn SOD) in patients with familial amyotrophic lateral sclerosis (ALS) has
made possible the development of etiological models of the disease. Expression of mutant
SOD1 genes in transgenic mice causes a progressive paralytic disease whose general
features resemble ALS in humans. We have used the transgenic model to explore
etiological mechanisms and to screen potential therapeutics. Our results and those of others …
