Nonmyelinating Schwann cell involvement with well-preserved unmyelinated Axons in Charcot-Marie-Tooth disease type 1a
Nonmyelinating Schwann cell involvement with well-preserved unmyelinated Axons in Charcot-Marie-Tooth disease type 1a
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DOI：
10.1097/nen.0b013e3181598294
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发表时间：
2007-11-01
影响因子：
3.2
通讯作者：
Sobue, Gen
中科院分区：
文献类型：
--
作者：
Koike, Haruki;Iijima, Masahiro;Sobue, Gen
应用电镜观察了27例伴有髓鞘蛋白22重复的腓骨肌萎缩症1A型（CMT 1A）患者和14例正常对照者的无髓鞘雪旺细胞和无髓鞘轴突的形态学变化。在16例患者中获得了完整的腓肠神经横截面，并估计了每个横截面中轴突和雪旺细胞的总数。在CMT 1A患者中，有髓轴突的数量显著减少，而无髓轴突保存良好，没有显示出任何明显的变化。在CMT 1A患者中，非髓鞘化Schwarm细胞的细胞核、亚基和轮廓的数量均显著增加，而每个含无髓鞘轴突亚基的轴突数量显著减少。在CMT 1A患者中，雪旺细胞亚单位由包裹在无髓轴突周围的扁平细胞质层组成。即使在有髓纤维保存完好的年轻患者中，无髓雪旺氏细胞的数量也增加，每个无髓轴突亚基的轴突数量也减少。总之，有显着的人口和形态的非髓鞘施瓦尔细胞的改变，和轴突-施瓦恩细胞的相互作用似乎受到不同的有髓和无髓纤维之间的CMT 1A。
Electron microscopic examination was performed to compare morphologic changes of nonmyelinating Schwann cells and unmyelinated axons in patients with Charcot-Marie-Tooth disease type 1A (CMT1A) with peripheral myelin protein 22 duplication (n = 27) and normal control individuals (n = 14). Complete transverse sural nerve cross-sections were obtained in 16 patients and the total number of axons and Schwann cells in each cross-section was estimated. In patients with CMT1A, the number of myelinated axons was significantly decreased, whereas unmyelinated axons were well-preserved and did not show any marked changes. The numbers of nuclei, subunits, and profiles of nonmyelinating Schwarm cells were all increased significantly in patients with CMT1A, whereas the numbers of axons per unmyelinated axon-containing subunit were significantly decreased. Schwann cell subunits consisted of layers of flattened cytoplasmic profiles wrapped around unmyelinated axons in the patient with CMT1A. The numbers of nonmyelinating Schwann cell profiles were increased and the numbers of axons per unmyelinated axon-containing subunit were reduced even in young patients with well-preserved myelinated fibers. In conclusion, there is marked alteration of the population and morphology of nonmyelinating Schwarm cells, and axon-Schwann cell interactions seem to be regulated differently between myelinated and unmyelinated fibers in CMT1A.