Article
Onset and progression in inherited ALS determined by motor neurons and microglia.
Science (New York, N.Y.) - 2 Jun 2006
Boillée Séverine, Yamanaka Koji, Lobsiger Christian S, Copeland Neal G, Jenkins Nancy A, Kassiotis George, Kollias George, Cleveland Don W
Abstract excerpt
Dominant mutations in superoxide dismutase cause amyotrophic lateral sclerosis (ALS), a progressive paralytic disease characterized by loss of motor neurons. With the use of mice carrying a deletable mutant gene, expression within motor neurons was shown to be a primary determinant of disease onset and of an early phase of disease progression. Diminishing the mutant levels in microglia had little effect on the...
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