Ayers M M, Anderson R M
Acta Neuropathol. 1976 Oct 15;36(2):137-52. doi: 10.1007/BF00685276.
The Trembler mouse is a neurological mutant showing dominant inheritance. The periopheral nerves show a delay in myelination, segmental demyelination and the development of an onion bulb neuropathy. Using electron microscopy a comparative morphometric study of nerve fibres in the sciatic nerve in normal and Trembler mice was made. The animals ranged in age from 3 days to adult. The following features were noted: 1. Mean axon diameters of the Trembler mice were smaller than normal at all ages. 2. Myelination was delayed and myelin was thinner than normal in the Trembler mice. 3. The ratio of myelin thickness to axon diameter was reduced in Trembler mice. 4. There were no significant quantitative or qualitative differences observed between middle and distal levels of Trembler nerves. The balance of evidence from these findings and the previous morphological studies is in keeping with the suggestion that the primary lesion resides in the Schwann cell.
颤抖鼠是一种表现出显性遗传的神经学突变体。外周神经显示出髓鞘形成延迟、节段性脱髓鞘以及洋葱球样神经病的发展。利用电子显微镜对正常小鼠和颤抖鼠坐骨神经中的神经纤维进行了比较形态计量学研究。动物年龄从3天到成年不等。观察到以下特征：1. 所有年龄段的颤抖鼠的平均轴突直径均小于正常小鼠。2. 颤抖鼠的髓鞘形成延迟且髓鞘比正常小鼠薄。3. 颤抖鼠的髓鞘厚度与轴突直径之比降低。4. 在颤抖鼠神经的中部和远端水平之间未观察到明显的数量或质量差异。这些发现以及先前形态学研究的证据平衡与原发性病变位于施万细胞的观点一致。
