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C. A. Farah, M. D. Nguyen, J. P. Julien and N. Leclerc, “Altered Levels and Distribution of Microtubule-Associated Proteins before Disease Onset in a Mouse Model of Amyotrophic Lateral Sclerosis,” Journal of Neurochemistry, Vol. 84, No. 1, 2003, pp. 77-86. doi:10.1046/j.1471-4159.2003.01505.x

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• TITLE: Early Exposure to Environmental Toxin Contributes to Neuronal Vulnerability and Axonal Pathology in a Model of Familial ALS

  AUTHORS: Grace Lee, Christopher A. Shaw

  KEYWORDS: SOD1; ALS; Neurotoxin; Axonopathy; NMJ; Gliosis; Toxicity

  JOURNAL NAME: Neuroscience and Medicine, Vol.3 No.4, December 31, 2012

  ABSTRACT: Adult onset amyotrophic lateral sclerosis (ALS) arises due to progressive and irreversible functional deficits to the central nervous system, specifically the loss of motor neurons. Sporadic ALS causality is not well understood, but is almost certainly of multifactorial origin involving a combination of genetic and environmental factors. The discovery of endemic ALS in the native Chamorro population of Guam during the 1950s and the co-occurrence of Parkinsonism and dementia in some patients led to searches for environmental toxins that could be responsible. In the present paper, we report that an environmental neurotoxin enhances mutant superoxide dismutase (SOD)-induced spinal motor neuron death and pathology and induces motor axon abnormalities. These results cumulatively confirm earlier findings that exposure to an environmental toxin is sufficient to produce the disease phenotype and indicate a role for gene-environment interaction in some forms of the disease.