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Neurobiol Dis. 1996 Apr;3(2):137-47.

Electromyographical and motor performance studies in the pmn mouse model of neurodegenerative disease.

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  • 1D√©partement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Universit√© Louis Pasteur, Illkirch, France. kennel@rp.fr

Abstract

The mouse autosomal recessive mutation progressive motor neuronopathy (pmn) results in early onset motor neuron disease with rapidly progressive hindlimb paralysis, severe muscular wasting, and death at around 6 weeks of age. This mutant provides opportunities for testing novel therapeutic strategies, including the administration of trophic factors, to prevent the degeneration of diseased neurons. The construction of a strain expressing the pmn and the Extra-toe (Xt) phenotypes allows the detection, and therefore the treatment, of affected progeny before the onset of the clinical weakness. Electromyography is the most appropriate technique for a longitudinal study in which a given individual is examined repeatedly. We present the results of an electrophysiological and behavioral exploration of the pmn disease and show that electromyography is a powerful tool for following the course of the disease and evaluating potential therapies relevant to motor neuron diseases.

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