Odaka M, Yuki N, Yoshino H, Kiso M, Ishida H, Hirata K
Department of Neurology, Dokkyo University School of Medicine, Shimotsuga, Tochigi, Japan.
J Neurol Sci. 1999 Jun 1;165(2):126-32. doi: 10.1016/s0022-510x(99)00097-0.
Certain species of anti-ganglioside antibodies are associated with specific clinical features in various neurologic diseases. Serum autoantibodies to these minor gangliosides were investigated in a number of neurological diseases in order to examine the biological functions of GD1alpha and GQ1beta. Eleven patients with Guillain-Barré syndrome had remarkably high IgG anti-GD1alpha antibody titers, but no GD1alpha was detected in human peripheral nerve. An absorption study showed that IgG anti-GD1alpha antibodies from eight of the 11 patients were significantly absorbed by GD1a and GM1b, indicative that the IgG anti-GD1alpha antibodies cross-react with GD1a and GM1b. Both GD1a and GM1b have been reported to be target molecules for serum antibodies in certain patients with Guillain-Barré syndrome. GD1alpha may induce the production of IgG anti-GD1alpha antibody which cross-reacts with GD1a or GM1b, and subsequently functions in the development of Guillain-Barré syndrome. The IgGs from six patients with Fisher's syndrome who had the anti-GQ1beta antibody had anti-GQ1b activity as well. All the patients had external ophthalmoplegia, but no GQ1beta was detected in the human oculomotor nerve, further evidence that GQ1b, not GQ1beta, is the molecule targeted by the autoantibody in Fisher's syndrome.
某些抗神经节苷脂抗体物种与各种神经系统疾病的特定临床特征相关。为了研究GD1α和GQ1β的生物学功能，对多种神经系统疾病患者的血清中针对这些次要神经节苷脂的自身抗体进行了检测。11例吉兰-巴雷综合征患者的IgG抗GD1α抗体滴度显著升高，但在人周围神经中未检测到GD1α。吸收研究表明，11例患者中有8例的IgG抗GD1α抗体可被GD1a和GM1b显著吸收，这表明IgG抗GD1α抗体与GD1a和GM1b发生交叉反应。据报道，在某些吉兰-巴雷综合征患者中，GD1a和GM1b均为血清抗体的靶分子。GD1α可能诱导产生与GD1a或GM1b发生交叉反应的IgG抗GD1α抗体，随后在吉兰-巴雷综合征的发病过程中发挥作用。6例患有Fisher综合征且具有抗GQ1β抗体的患者的IgG也具有抗GQ1b活性。所有患者均有眼外肌麻痹，但在人动眼神经中未检测到GQ1β，这进一步证明GQ1b而非GQ1β是Fisher综合征中自身抗体的靶分子。
