Electron Microscopy Analysis of Sciatic Nerve Fibers in C57BL/6 Transgenic Mice

Hereditary Charcot-Marie-Tooth (CMT) motor/sensory neuropathy is a known disease in the group of polyneuropathies. It is characterized, in particular, by the process of demyelination of peripheral nerve fibers. Variousin vivoandin vitromodels have been used for elucidation of the mechanisms involved in the demyelination processes. We investigated ultrastructural manifestations of peripheral demyelination in C57BL/6 transgenic mice. Electron microscopic examination of fibers of the sciatic nerve in such mice showed that many axons in this nerve were subjected to dys- and demyelination; the so-called onion bulb-like structures were observed. In some fibers, hypertrophy of myelin sheaths was found. In general, ultrastructural modifications in the sciatic nerve of the C57BL transgenic mice were rather similar to the pathomorphological pattern observed in patients suffering from CMT. Thus, the transgenic heterozygous line of C57BL mice can be used as an adequate model for revealing the mechanisms of development of CMT and for the experimental search of treatments for this disease.