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ALS model

Amyotrophic lateral sclerosis 

ALS is a progressive neurodegenerative disease resulting in generalized muscle weakness and atrophy, and, usually, death within a few years. A transgenic mouse model expressing a mutant form of human SOD-1  (superoxide dismutase 1) exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans, an age-related rapidly progressive decline of motor functions accompanied by degenerative changes of motor neurons within the spinal cord, brain stem and neocortex. We have extensive experience in the use of this transgenic mouse model to test possible new treatments for ALS. This transgenic mouse strain also serves as a general model of neurodegenerative diseases and neurodegenerative treatments. Endpoints are animal survival, behavior, and histology.

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