Saida K
Department of Neurology, Utano National Hospital.
Rinsho Shinkeigaku. 1990 Dec;30(12):1361-3.
Myelin in PNS is multi-layered membranes formed by Schwann cells, and surrounds axon. Destruction of myelin sheath results in demyelination and disturbance of nerve conduction. In PNS, Charcot-Marie-Tooth disease, certain lipidoses, Guillain-Barré syndrome, lead poisoning, compression and some metabolic neuropathies can produce demyelination. In these diseases, GBS is thought to be resulted from abnormalities of immune mechanism. Recently, autoantibodies against Gal-C, P2 and GM1, and complement fixing antibodies against PNS myelin are found in some of GBS patient sera. Here, I present studies on effector mechanism of PNS demyelination using models produced by application of Galactocerebroside (Gal-C) antibodies to PNS. Mainly, three types of effector mechanisms are involved in Gal-C antibody-induced demyelination. In abundance of antibodies, complement-mediated demyelination is at work. When complements are absent, antibody dependent macrophage-mediated demyelination can be involved. Thirdly, myelin once damaged by oxidants and etc can be opsonized by antibodies and C3b, and phagocytized by macrophages. These processes may be operating in such diseases like GBS and CIDP.
周围神经系统（PNS）中的髓鞘是由施万细胞形成的多层膜，包裹着轴突。髓鞘的破坏会导致脱髓鞘和神经传导紊乱。在周围神经系统中，夏科-马里-图斯病、某些脂质沉积症、格林-巴利综合征、铅中毒、压迫以及一些代谢性神经病变都可能导致脱髓鞘。在这些疾病中，格林-巴利综合征被认为是由免疫机制异常引起的。最近，在一些格林-巴利综合征患者的血清中发现了针对半乳糖脑苷脂（Gal-C）、P2和GM1的自身抗体，以及针对周围神经系统髓鞘的补体结合抗体。在此，我介绍利用将抗半乳糖脑苷脂（Gal-C）抗体应用于周围神经系统所产生的模型，对周围神经系统脱髓鞘效应机制的研究。主要地，Gal-C抗体诱导的脱髓鞘涉及三种效应机制。在抗体丰富时，补体介导的脱髓鞘起作用。当补体缺乏时，抗体依赖性巨噬细胞介导的脱髓鞘可能参与其中。第三，一旦被氧化剂等损伤的髓鞘可被抗体和C3b调理，并被巨噬细胞吞噬。这些过程可能在格林-巴利综合征和慢性炎性脱髓鞘性多发性神经根神经病等疾病中发挥作用。
