Article
Pathophysiology of neurodegeneration in familial amyotrophic lateral sclerosis.
Current molecular medicine - 1 Apr 2009
Vucic Steve, Kiernan Matthew C
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of the motor neurons in the spinal cord, brainstem, and motor cortex. Ten percent of ALS cases are familial, with both autosomal dominant and recessive modes of inheritance reported. Mutations in the copper/zinc superoxide-dismutase-1 (SOD-1) gene, the first gene linked with ALS, result in the classical ALS phenotype. To date, 135...
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