title: Electromyographical and motor performance studies in the pmn mouse model of neurodegenerative disease.
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.
authors.affiliation: Département d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, Illkirch, France. kennel@rp.fr
authors.forename: P F
authors.initials: PF
authors.lastname: Kennel
authors.forename: P
authors.initials: P
authors.lastname: Fonteneau
authors.forename: E
authors.initials: E
authors.lastname: Martin
authors.forename: J M
authors.initials: JM
authors.lastname: Schmidt
authors.forename: M
authors.initials: M
authors.lastname: Azzouz
authors.forename: J
authors.initials: J
authors.lastname: Borg
authors.forename: J L
authors.initials: JL
authors.lastname: Guenet
authors.forename: H
authors.initials: H
authors.lastname: Schmalbruch
authors.forename: J M
authors.initials: JM
authors.lastname: Warter
authors.forename: P
authors.initials: P
authors.lastname: Poindron
mesh_terms: D000200:Action Potentials; D000818:Animals; D003964:Diaphragm / Q000503:physiopathology; D004195:Disease Models, Animal; D004576:Electromyography; D005154:Facial Nerve / Q000503:physiopathology; D005260:Female; D005532:Foot Deformities, Congenital / Q000235:genetics; D005808:Genes, Recessive; D008040:Genetic Linkage; D015417:Hereditary Sensory and Motor Neuropathy / Q000235:genetics / Q000503:physiopathology*; D008297:Male; D051379:Mice; D008818:Mice, Neurologic Mutants; D009043:Motor Activity; D009046:Motor Neurons / Q000502:physiology; D018482:Muscle, Skeletal / Q000503:physiopathology; D009431:Neural Conduction; D010641:Phenotype; D011930:Reaction Time; D012584:Sciatic Nerve / Q000503:physiopathology; D014034:Toes / Q000002:abnormalities