ANIMAL MODELS OF PERIPHERAL NEUROPATHYs THE TREMBLER MOUSE J. 0. McLEOD, MB, BS, DPhil and P. A. LOW, MID
Editor's Note: One of the features of the lVth Interna-
tional Congress on Neuromuscular Diseases, held in Montreal September 17-21, 1978, was a series of invited posters. In order to achieve a wider audience for this valuable material, we have made arrangements to Dublish a number of these Dosters in Muscle & Nebe. This is the third poster in this series. TO preserve the original effect of the posters, they are being reproduced almost exactly as they appeared at the Congress, without editing or other alterations. We hope you will find this feature a useful addition to your Journal.
MYELINOGENESIS IN TREMBLER MICE
Abnormalities of myelin are apparent at Seven days (figs. - 7 and 8). Schwann cellcytoplasm appears abundant and active (fig. 9).
months,
At six Seen (fig. lo)-
onion bulb lamellae are
N~,.,,~ graft experiments have conclusively demonstrated that the Trembler neuropathy is due to a primary disorder of Schwann cells (figs. 1 1 and 12). ELECTROPHYSIOLOGIC STUDIES
The Trembler mouse has a dominantly inherited hypertrophic neuropathy. There is a disorder of Schwann cells, which are ineffective in the synthesis, compaction, and maintenance of myelin. HISTOPATHOLOGY OF PERIPHERAL NERVES-ADULT MICE
Myelinated fibers are reduced in density (figs. 1 and 2). Electron microscopy shows that myelin is poorly comDacted (fias. 3 and 4).
Animal models of peripheral neuropathy: the Trembler mouse.
POSTER SESSION
ANIMAL MODELS OF PERIPHERAL NEUROPATHYs THE TREMBLER MOUSE J. 0. McLEOD, MB, BS, DPhil and P. A. LOW, MID
Editor's Note: One of the f...