The molecular pathogenesis of Guillain-Barre syndrome

格林-巴利综合征的分子发病机制

• 批准号: 10557063
• 负责人: YUKI Nobuhiro
• 金额: $ 2.05万
• 依托单位: Dokkyo University School of Medicine
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (B)
• 财政年份: 1998
• 资助国家: 日本
• 起止时间: 1998 至 2001
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-10557063/
• 关键词: Guillain-Barre syndrome; molecular mimicry; Campylobacter jejuni; anti-GM1 IgG antibody; animal model; ガングリオシド; GM1; モデル動物; 抗GM1抗体; ウシ脳ガングリオシド; N-グリコリルノイラミン酸

Some humans develop the axonal form of Guillain-Barre syndrome after receiving bovine brain ganglioside. On sensitization with ganglioside mixture, all of a group of rabbits injected developed high anti-GM1 IgG antibody titers, flaccid limb weakness of acute onset, and a monophasic illness course. Pathological findings for the peripheral nerves showed predominant Wallerian-like degeneration, with neither lymphocytic infiltration nor demyelination. IgG was deposited on the axons of the anterior roots, and GM1 was proved to be present on the axons of peripheral nerves.Sensitization with purified GM1 also induced axonal neuropathy, indicating that GM1 was the immunogen in the mixture. A model of human axonal Guillain-Barre syndrome has been established that uses inoculation with a bovine brain ganglioside mixture or isolated GM1. This model may help to clarify the molecular pathogenesis of the syndrome and to develop new treatments for it.

一些人在接受牛脑神经节苷脂后发展为格林-巴利综合征的轴突形式。用神经节苷脂混合物致敏后，所有注射组家兔均出现高抗GM 1 IgG抗体滴度，急性发作时四肢无力，病程呈双相性。周围神经的病理结果显示主要的沃勒样变性，既没有淋巴细胞浸润，也没有脱髓鞘。免疫组化结果显示，IgG在前根轴突上沉积，而GM 1在周围神经轴突上沉积，纯化的GM 1致敏也可诱发轴突性神经病，表明GM 1是混合物中的免疫原。已经建立了人轴突格林-巴利综合征模型，该模型使用牛脑神经节苷脂混合物或分离的GM 1接种。该模型可能有助于阐明该综合征的分子发病机制，并开发新的治疗方法。

项目成果

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Nobuhiro Yuki（其他 0 名，第 1 名）：“空肠弯曲菌肠炎后的吉兰-巴利综合征和米勒·费希尔综合征的发病机制”Jpn J Infect Dis. 52. 99-105 (1999)