Department of Functional Neurobiology, School of Pharmacy, Tokyo University of Pharmacy and Life Sciences.
Department of Biosciences, School of Science, Kitasato University.
Proc Jpn Acad Ser B Phys Biol Sci. 2023;99(2):48-60. doi: 10.2183/pjab.99.004.
Intravenous immunoglobulin (IVIg) has been used to treat inflammatory demyelinating diseases such as chronic inflammatory demyelinating polyneuropathy, Guillain-Barré syndrome, and multifocal motor neuropathy. Despite studies demonstrating the clinical effectiveness of IVIg, the mechanisms underlying its effects remain to be elucidated in detail. Herein, we examined the effects of IVIg on lysolecithin-induced demyelination of the sciatic nerve in a mouse model. Mice -administered with IVIg 1 and 3 days post-injection (dpi) of lysolecithin -exhibited a significantly decreased demyelination area at 7 dpi. Immunoblotting analysis using two different preparations revealed that IVIg reacted with a 36-kDa membrane glycoprotein in the sciatic nerve. Subsequent analyses of peptide absorption identified the protein as a myelin protein in the peripheral nervous system (PNS) known as large myelin protein zero (L-MPZ). Moreover, injected IVIg penetrated the demyelinating lesion, leading to deposition on L-MPZ in the myelin debris. These results indicate that IVIg may modulate PNS demyelination, possibly by binding to L-MPZ on myelin debris.
静脉注射免疫球蛋白 (IVIg) 已被用于治疗炎症性脱髓鞘疾病,如慢性炎症性脱髓鞘性多发性神经病、吉兰-巴雷综合征和多灶性运动神经病。尽管研究表明 IVIg 的临床疗效,但它的作用机制仍需详细阐明。在此,我们研究了 IVIg 对小鼠坐骨神经中溶血磷脂诱导的脱髓鞘的影响。在溶血磷脂注射后 1 天和 3 天给予 IVIg 的小鼠 - 在 7 天 dpi 时表现出明显减少的脱髓鞘区域。使用两种不同制剂进行的免疫印迹分析显示,IVIg 与坐骨神经中的 36 kDa 膜糖蛋白反应。随后的肽吸收分析将该蛋白鉴定为外周神经系统 (PNS) 中的一种髓鞘蛋白,称为大髓鞘蛋白零 (L-MPZ)。此外,注射的 IVIg 穿透脱髓鞘病变,导致在髓鞘碎片中的 L-MPZ 上沉积。这些结果表明,IVIg 可能通过与髓鞘碎片上的 L-MPZ 结合来调节 PNS 脱髓鞘。