Nerve Conduction and Neuromuscular Transmission in C57Bl/6 Mice with Genetically Determined Peripheral Neuropathy

Abstract

Charcot–Marie–Tooth disease is one of the most widespread forms of hereditary peripheral neuropathy in humans. C57Bl/6 mice are considered the most appropriate animal model for studies of this disease. We measured the conduction velocity in the sciatic nerve (NCV) and amplitude of the M-wave in mice of strains C57Bl/6 and C57Bl. It was found that the mean conduction velocity in the right and left sciatic nerves of C57Bl/6 mice was about 3.5-fold lower than that in C57Bl animals. In the former mice, the mean amplitude of compound nerve action potentials (CNAPs) and that of the M-wave in the m. gastrocnemius-soleus after stimulation of the sciatic nerve were 5- and 4-fold, respectively, lower, than those in the control (C57B1). Thus, the data obtained show that the genetically determined pathology of the peripheral nervous system caused by a mutation of the PMP22 gene results in dramatic negative shifts of the characteristics of conduction via the peripheral nerves and of neuromuscular trans-mission.