ELECTROMYOGRAPHIC AND MOTOR-PERFORMANCE STUDIES IN THE PMN MOUSE MODEL OF NEURODEGENERATIVE DISEASE

The mouse autosomal recessive mutation progressive motor neuronopathy (pmn) results in early onset motor neuron disease with rapidly progres sive hindlimb paralysis, severe muscular wasting, and death at around 6 weeks of age. This mutant provides opportunities for testing novel t herapeutic 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 appr opriate technique for a longitudinal study in which a given individual is examined repeatedly. We present the results of an electrophysiolog ical and behavioral exploration of the pmn disease and show that elect romyography is a powerful tool for following the course of the disease and evaluating potential therapies relevant to motor neuron diseases. (C) 1996 Academic Press, Inc.