Nakao T, Kon K, Ando S, Miyatake T, Yuki N, Li Y T, Furuya S, Hirabayashi Y
Department of Applied Biology, Taisho Pharmaceutical Co., Ltd., Saitama, Japan.
J Biol Chem. 1993 Oct 5;268(28):21028-34.
A motor neuron disorder resembling that of amyotrophic lateral sclerosis was found in a patient who had received the intramuscular administration of a mixture of bovine brain gangliosides (Yuki, N., Sato, S., Miyatake, T., Sugiyama, K., Katagiri, T., and Sasaki, H. (1991) Lancet 337, 1109-1110). A very high titer of anti-GM2 IgM was detected in the patient's serum and the patient quickly recovered after plasmapheresis. The clinical course of the patient appeared to be different from amyotrophic lateral sclerosis and the anti-GM2 IgM was thought to be the culprit. The IgM reacted with GM2, GM1b-GalNAc, SPG(alpha 2-3)-GalNAc, and GD1a-GalNAc, but not with GA2 or GD2, meaning that the epitope recognized by the IgM was the GM2-like terminal structure, GalNAc beta 1-4(Neu-Ac alpha 2-3)Gal beta 1-. In this study, we found two novel GM2-epitope containing gangliosides, X1 and X2, in bovine brain gangliosides by TLC immunostaining using the patient's IgM. They were characterized as unique lacto-ganglio type gangliosides containing the following branching structures. [formula: see text] Their unusual structures may be immunogenic to humans to induce anti-GM2 antibody.
在一名接受了肌肉注射牛脑神经节苷脂混合物的患者中，发现了一种类似于肌萎缩侧索硬化症的运动神经元疾病（Yuki, N., Sato, S., Miyatake, T., Sugiyama, K., Katagiri, T., and Sasaki, H. (1991) Lancet 337, 1109 - 1110）。在患者血清中检测到非常高滴度的抗GM2 IgM，并且患者在进行血浆置换后迅速康复。该患者的临床病程似乎与肌萎缩侧索硬化症不同，抗GM2 IgM被认为是罪魁祸首。该IgM与GM2、GM1b - GalNAc、SPG(α2 - 3)-GalNAc和GD1a - GalNAc发生反应，但不与GA2或GD2反应，这意味着该IgM识别的表位是GM2样末端结构，即GalNAcβ1 - 4(Neu - Acα2 - 3)Galβ1 - 。在本研究中，我们通过使用患者的IgM进行薄层层析免疫染色，在牛脑神经节苷脂中发现了两种含有GM2表位的新型神经节苷脂，X1和X2。它们被鉴定为具有以下分支结构的独特乳糖 - 神经节型神经节苷脂。[公式：见原文]它们不寻常的结构可能对人类具有免疫原性，从而诱导抗GM2抗体。
