Matsumoto A, Yoshino H, Yuki N, Hara Y, Cashman N R, Handa S, Miyatake T
Department of Biochemistry, Faculty of Medicine, Tokyo Medical and Dental University, Japan.
J Neurol Sci. 1995 Aug;131(2):111-8. doi: 10.1016/0022-510x(95)00101-7.
We have examined ganglioside compositions and the presence of sulfated glucuronyl glycolipids of immortalized motor neuron-like cell lines, neuroblastoma-spinal cord (NSC) hybrid cell lines established by fusing mouse neuroblastoma N18TG2 with motor neuron-enriched embryonic spinal cord cells. Among NSC cell lines, only NSC-34 aggregates acetylcholine receptors on co-cultured myotube and expresses a receptor for S-laminin, a neuromuscular junction specific basal lamina protein. GM2, which is only a minor ganglioside component of CNS, was the major component in NSC-34 occupying almost 75% of total gangliosides, whereas GD1a and GM3 were major species in the parental N18TG2, which had only 8.5% GM2. These results indicated that NSC lines have unique ganglioside pattern that is distinctive from other nervous tissues, and this pattern, especially that of NSC-34 cells, might reflect the characteristics of mouse spinal motor neuron gangliosides. Sulfated glucuronyl paragloboside was demonstrated to be present in N18TG2, however, it could not be detected in either of NSC cell lines. Even though the pathogenesis of amyotrophic lateral sclerosis remains unknown, autoimmunological participation has been suggested. Because high-titered antibody against GM2 has been observed in a patient with amyotrophic lateral sclerosis-like disease, GM2 which is possibly expressed on the surface of motor neurons might serve as a potential target antigen in this disorder.
我们检测了永生化运动神经元样细胞系、通过将小鼠神经母细胞瘤N18TG2与富含运动神经元的胚胎脊髓细胞融合建立的神经母细胞瘤-脊髓（NSC）杂交细胞系的神经节苷脂组成以及硫酸化葡糖醛酸糖脂的存在情况。在NSC细胞系中，只有NSC-34能在共培养的肌管上聚集乙酰胆碱受体，并表达S-层粘连蛋白的受体，S-层粘连蛋白是神经肌肉接头特异性基底膜蛋白。GM2在中枢神经系统中只是一种次要的神经节苷脂成分，但在NSC-34中却是主要成分，几乎占总神经节苷脂的75%，而GD1a和GM3是亲本N18TG2中的主要种类，GM2仅占8.5%。这些结果表明，NSC系具有与其他神经组织不同的独特神经节苷脂模式，这种模式，尤其是NSC-34细胞的模式，可能反映了小鼠脊髓运动神经元神经节苷脂的特征。硫酸化葡糖醛酸副球蛋白在N18TG2中被证实存在，但在任何一种NSC细胞系中都未检测到。尽管肌萎缩侧索硬化症的发病机制尚不清楚，但已有研究表明自身免疫参与其中。因为在一名患有肌萎缩侧索硬化症样疾病的患者中观察到了高滴度的抗GM2抗体，所以可能在运动神经元表面表达的GM2可能是这种疾病的潜在靶抗原。
