自己抗体による末梢神経疾患の発症機序

鞘糖脂是神经细胞的组成成分，可作为体液和细胞免疫应答中的重要抗原。糖脂抗体已被证明存在于各种免疫介导的神经系统疾病患者中。在本研究中，我们的目的是阐明这些自身抗体的意义和独特的糖脂家族的作用，以及可能在这些疾病的免疫发病机制下的机制。高抗神经节苷脂抗体滴度检测患有这种疾病的患者。我们已经证明，神经节苷脂，它具有一个共同的碳水化合物表位与细菌的前感染是潜在的靶抗原的格林-巴利综合征和费舍尔病。各种次要神经节苷脂也可以是许多其他周围神经病中抗体的靶点。在慢性炎性脱髓鞘性多发性神经病患者中，经常检测到高滴度抗硫酸化葡萄糖醛酸糖脂。这种高滴度的抗体与M蛋白的存在或血清IgM值无关。我们认为，伴有抗MAG/SGPG IgM抗体的多发性神经病是CIDP的一个亚类，梅尼埃病患者血清抗糖脂抗体阳性。通过对听神经鞘瘤样品的化学分析，检测到新乳系列糖脂。这些结果表明抗糖脂抗体在这些疾病的免疫发病机制中的重要作用。

Glycosphingolipids are constituents of nerve cells that may serve as important antigens prticipating in humoral and cellular immune responses. Antibodies to glycolipids have been shown to be present in patients with variety of immune-mediated neurological disorders. In this research, we aimed to elucidate the significance of these autoantibodies and the roles of unique family of glycolipids as well as mechanisms that may under lie the immunopathogenesis of these disorders.High anti-ganglioside antibodies titers are detected in patients suffering this disorders. We have demonstrated that gangliosides, which possess a common carbohydrate epitope with bacteria of the preceding infection are potential target antigens for the Guillain-Barre syndrome and Fisher disease. Various minor gangliosides can also be the target of antibodies in many other peripheral neuropathies. In patients with chronic inflammatory demyelinating polyneuropathy, high titer anti-sulfated glucuronosyl glycolipids were frequently detected. This high titer of antibody was not related with the presence of M-protein nor serum IgM value. We propose that polyneuropathy with IgM anti MAG/SGPG antibody form a subgroup of CIDP.Serum anti-glycolipids antibody activities in patients with Meniere's disease showed positive anti-sialylparagloboside antibody titers. by the chemical analysis of acoustic neurinoma samples, neolacto-series glycolipids were detected. These results indicate the significant roles of the anti-glycolipid antibody for the immunopathogenesis of these disorders.