Article

Given the increasing number of transgenic murine models of human disease, functional studies are of great importance to demonstrate how closely these animals resemble patients with these diseases. Electrodiagnostic testing is an excellent functional tool for assessing injury to peripheral nerve in these models. Admittedly, there are variables influencing the results of such testing in mice not seen in humans. These include small segmental distances in nerve conduction, which could influence measurements of conduction velocity, and excessive movement in mice in SSFEMG. However, we have found these tests to be generally reproducible in
the tr-j mouse, providing useful information about the function of peripheral nerve myelin, axon, and neuromuscular transmission in this model for demyelinating CMT.

Langue : ANGLAIS