Modeling Neurodegenerative Diseases in vivo

Evaluation Tools and Animal Models of Peripheral Neuropathies
B. Fricker^{a, b}, A. Muller^a-c, F. René<sup>a, b

a</sup>INSERM U-692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence,
^bUMRS692, Faculté de médecine, Université Louis-Pasteur, et
^cCentre Anti-Douleur, Groupe Hospitalier Universitaire de Strasbourg, Strasbourg, France

Address of Corresponding Author

Neurodegenerative Dis 2008;5:72-108 (DOI: 10.1159/000112835)

  Key Words

• Peripheral neuropathy
• Animal model
• Transgenic mouse behaviour
• Electrophysiology
• Histology
• Schwann cell
• Cyclin D₁
• Sciatic nerve
• Myelin sheath

  Abstract

Peripheral neuropathies are common and frequently debilitating disorders linked to degeneration of peripheral nerves that supply mainly the distal muscles of the extremities. Due to the diverse origin of the pathology (genetic, systemic or environmental), peripheral neuropathies exhibit different clinical forms: acute or chronic, symmetrical or asymmetrical, demyelinating or axonal. In the last 30 years, to gain insight into cellular and molecular mechanisms underlying neuropathies, numerous animal models - either spontaneous or induced by chemical compounds, physical injury or genetic engineering - have been extensively developed and used. In this review, we present (1) the methods used to assess the peripheral neuropathies in the major available animal models and their main pathological characteristics and (2) the main models and their relevance to the human pathology, and, consequently, their usefulness for preclinical studies. Finally, we discuss the emerging, recently developed tools, that should allow to gain a better insight into the mechanisms underlying the peripheral neuropathies.

Copyright © 2008 S. Karger AG, Basel

  Author Contacts

F. René
INSERM U-692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence
FR-67085 Strasbourg (France)
Tel. +33 390 243 091, Fax +33 390 243 065, E-Mail f_rene@neurochem.u-strasbg.fr

  Article Information

Received: April 17, 2007
Accepted after revision: July 12, 2007
Published online: January 4, 2008
Number of Print Pages : 37
Number of Figures : 3, Number of Tables : 5, Number of References : 322