Gabriel J M, Erne B, Bernasconi L, Tosi C, Probst A, Landmann L, Steck A J
Department of Research, University Hospital Basel, Switzerland.
Acta Neuropathol. 1998 May;95(5):540-6. doi: 10.1007/s004010050835.
We report here on a patient with anti-myelin-associated glycoprotein (MAG) neuropathy in whom examination of a sural nerve biopsy by multichannel confocal microscopy showed a partly overlapping distribution of MAG and IgM deposits in myelinated fibers. Our data demonstrate that MAG in Schmidt-Lanterman incisures and paranodal loops, as well as some additional HNK-1-positive components of the basal lamina, are the major targets of the anti-MAG monoclonal IgM autoantibodies in this neuropathy in vivo. Perforation of the basal lamina can allow the penetration and binding of anti-MAG IgM inside myelinated fibers. Our results support and extend the notion that the production of monoclonal anti-MAG IgM may be antigenically driven by MAG molecules and that this process may occur in the immunologically privileged environment of the nerve prior to the appearance of a genuine gammopathy in serum.
我们在此报告一例抗髓鞘相关糖蛋白（MAG）神经病患者，对其腓肠神经活检标本进行多通道共聚焦显微镜检查显示，MAG和IgM沉积物在有髓纤维中呈部分重叠分布。我们的数据表明，施密特-兰特尔曼切迹和结旁环中的MAG，以及基膜的一些额外HNK-1阳性成分，是该神经病体内抗MAG单克隆IgM自身抗体的主要靶点。基膜穿孔可使抗MAG IgM渗透并结合到有髓纤维内部。我们的结果支持并扩展了这样一种观点，即单克隆抗MAG IgM的产生可能由MAG分子抗原驱动，且这一过程可能发生在血清中出现真正的丙种球蛋白病之前神经的免疫特权环境中。
