Article
Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 27 Feb 2008
Jaarsma Dick, Teuling Eva, Haasdijk Elize D, De Zeeuw Chris I, Hoogenraad Casper C
Abstract excerpt
Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), an adult-onset progressive paralytic disease characterized by loss of motor neurons, and cause an ALS-like disease when expressed in mice. Recent data have suggested that motor neuron degeneration results from toxic actions of mutant SOD1 operating in both motor neurons and their neighboring glia, raising the question whether...
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