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[神经束蛋白:中枢和外周联合脱髓鞘的新靶点]

[Neurofascin: a novel target for combined central and peripheral demyelination].

作者信息

Kawamura Nobutoshi

机构信息

Department of Neurology, Kawamura Hospital.

出版信息

Rinsho Shinkeigaku. 2014;54(12):978-80. doi: 10.5692/clinicalneurol.54.978.

Abstract

Combined central and peripheral demyelination (CCPD) is a rare clinical entity characterized by inflammatory demyelination in both the central and peripheral nervous system. A recently conducted nation-wide survey revealed that clinical features of CCPD are atypical for multiple sclerosis, including an absence of oligoclonal immunoglobulin G bands in most CCPD cases. We found that autoantibody responses of CCPD target the nodes and paranodes of Ranvier in the brain and peripheral nerve tissues. We identified anti-neurofascin antibody in the serum from these CCPD patients. CCPD patients showed a significantly higher positive rate of anti-neurofascin antibody than the other limited form of inflammatory demyelinating diseases. Autoantibody responses targeting neurofascins, which are common proteins to the central and peripheral nervous system may play a pivotal role in combined demyelination in CCPD.

摘要

中枢与周围联合性脱髓鞘病变(CCPD)是一种罕见的临床病症,其特征为中枢神经系统和周围神经系统均出现炎性脱髓鞘。最近开展的一项全国性调查显示,CCPD的临床特征与多发性硬化症不同,包括大多数CCPD病例中缺乏寡克隆免疫球蛋白G带。我们发现,CCPD的自身抗体反应靶向脑和周围神经组织中的郎飞结和结旁区。我们在这些CCPD患者的血清中鉴定出了抗神经束蛋白抗体。CCPD患者抗神经束蛋白抗体的阳性率显著高于其他局限性炎性脱髓鞘疾病。针对神经束蛋白的自身抗体反应,而神经束蛋白是中枢神经系统和周围神经系统共有的蛋白质,可能在CCPD的联合性脱髓鞘中起关键作用。

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