| First Author | Kennel PF | Year | 1996 |
| Journal | Neurobiol Dis | Volume | 3 |
| Issue | 2 | Pages | 137-47 |
| PubMed ID | 9173921 | Mgi Jnum | J:43329 |
| Mgi Id | MGI:1097509 | Doi | 10.1006/nbdi.1996.0014 |
| Citation | Kennel PF, et al. (1996) Electromyographical and motor performance studies in the pmn mouse model of neurodegenerative disease. Neurobiol Dis 3(2):137-47 |
| abstractText | 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. (C) 1996 Academic Press, Inc. |
