Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative Disease
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 strategie...
| Main Authors: | , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
1996-04-01
|
| Series: | Neurobiology of Disease |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0969996196900145 |
| _version_ | 1818862613048590336 |
|---|---|
| author | Philippe F. Kennel Paul Fonteneau Elisabeth Martin Jean-Marc Schmidt Mimoun Azzouz Jacques Borg Jean-Louis Guenet Henning Schmalbruch Jean-Marie Warter Philippe Poindron |
| author_facet | Philippe F. Kennel Paul Fonteneau Elisabeth Martin Jean-Marc Schmidt Mimoun Azzouz Jacques Borg Jean-Louis Guenet Henning Schmalbruch Jean-Marie Warter Philippe Poindron |
| author_sort | Philippe F. Kennel |
| collection | DOAJ |
| description | 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 thepmnand theExtra-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 thepmndisease and show that electromyography is a powerful tool for following the course of the disease and evaluating potential therapies relevant to motor neuron diseases. |
| first_indexed | 2024-12-19T10:02:39Z |
| format | Article |
| id | doaj.art-e479a197653d4eb091b69aa009a1136f |
| institution | Directory Open Access Journal |
| issn | 1095-953X |
| language | English |
| last_indexed | 2024-12-19T10:02:39Z |
| publishDate | 1996-04-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Neurobiology of Disease |
| spelling | doaj.art-e479a197653d4eb091b69aa009a1136f2022-12-21T20:26:37ZengElsevierNeurobiology of Disease1095-953X1996-04-0132137147Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative DiseasePhilippe F. Kennel0Paul Fonteneau1Elisabeth Martin2Jean-Marc Schmidt3Mimoun Azzouz4Jacques Borg5Jean-Louis Guenet6Henning Schmalbruch7Jean-Marie Warter8Philippe Poindron9Département d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkDépartement d'Immunologie, Immunopharmacologie et Pathologie, Centre de Recherches Pharmaceutiques, Université Louis Pasteur, BP 24, F-67401, Illkirch, Cedex, France; Cabinet de Rhumatologie et d'Explorations Fonctionnelles Neuromusculaires, 8 Boulevard du Président Roosevelt, F-68100, Mulhouse, France; Unité de Génétique des Mammifères, Institut Pasteur, F-75724, Paris Cedex, France; Institute of Neurophysiology, The Panum Institute, University of Copenhagen, DK-2200, Copenhagen, DenmarkThe 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 thepmnand theExtra-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 thepmndisease and show that electromyography is a powerful tool for following the course of the disease and evaluating potential therapies relevant to motor neuron diseases.http://www.sciencedirect.com/science/article/pii/S0969996196900145 |
| spellingShingle | Philippe F. Kennel Paul Fonteneau Elisabeth Martin Jean-Marc Schmidt Mimoun Azzouz Jacques Borg Jean-Louis Guenet Henning Schmalbruch Jean-Marie Warter Philippe Poindron Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative Disease Neurobiology of Disease |
| title | Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative Disease |
| title_full | Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative Disease |
| title_fullStr | Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative Disease |
| title_full_unstemmed | Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative Disease |
| title_short | Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative Disease |
| title_sort | electromyographical and motor performance studies in thepmnmouse model of neurodegenerative disease |
| url | http://www.sciencedirect.com/science/article/pii/S0969996196900145 |
| work_keys_str_mv | AT philippefkennel electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT paulfonteneau electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT elisabethmartin electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT jeanmarcschmidt electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT mimounazzouz electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT jacquesborg electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT jeanlouisguenet electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT henningschmalbruch electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT jeanmariewarter electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease AT philippepoindron electromyographicalandmotorperformancestudiesinthepmnmousemodelofneurodegenerativedisease |