在遗传性运动感觉神经病（夏科-马里-图斯病）模型颤抖-杰（trembler-J）小鼠中，正常神经条纹发生了改变。
Normal nerve striations are altered in the trembler-J mouse, a model of Charcot-Marie-Tooth disease.
作者信息
Power Bridget J, O'reilly Gavin, Murphy Robert, Murphy Keith J, Pickering Mark, Jones James F X
机构信息
School of Medicine and Medical Science, University College Dublin, Dublin 4, Ireland.
出版信息
Muscle Nerve. 2015 Feb;51(2):246-52. doi: 10.1002/mus.24303.
INTRODUCTION
This study was initiated because it was noted that the peripheral nerves of Trembler-J mice (a model of human Charcot-Marie-Tooth disease) appear to lack normal striations.
METHODS
We performed confocal microscopy of whole sciatic nerves and tested the effect of axial stress on impulse conduction.
RESULTS
We found that the axons of mutant mice were longer than those of the wild-type (1.55 mm of axon/mm length of nerve vs. 1.28 mm/mm respectively). This axonal elongation altered the helical nerve striations (bands of Fontana). As nerves were stretched axially, the conduction distance became correspondingly shorter. The effect on latency was significantly greater in the more coiled nerves of Trembler-J mice (P = 0.038).
CONCLUSIONS
The finding that mice with a mutated peripheral myelin protein 22 (PMP22) possess excessively long axons may be related to the excess Schwann cell numbers found in this disorder.
引言
开展本研究是因为注意到震颤-J小鼠（一种人类夏科-马里-图斯病模型）的外周神经似乎缺乏正常横纹。
方法
我们对完整的坐骨神经进行了共聚焦显微镜检查，并测试了轴向应力对冲动传导的影响。
结果
我们发现突变小鼠的轴突比野生型小鼠的长（分别为每毫米神经长度有1.55毫米轴突和1.28毫米轴突）。这种轴突伸长改变了螺旋状神经横纹（丰塔纳带）。当神经轴向拉伸时，传导距离相应缩短。在震颤-J小鼠盘绕更多的神经中，对潜伏期的影响显著更大（P = 0.038）。
结论
外周髓磷脂蛋白22（PMP22）突变的小鼠具有过长轴突这一发现，可能与该疾病中雪旺细胞数量过多有关。
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