3,4-Diaminopyridine improves neuromuscular transmission in a MuSK antibody-induced mouse model of myasthenia gravis

详细信息	查看全文 | 推荐本文 |

• 作者：Shuuichi Mori^a ; Masahiko Kishi^b ; Sachiho Kubo^a ; Takuyu Akiyoshi^a ; Shigeru Yamada^a ; Tsuyoshi Miyazaki^a ; Tetsuro Konishi^c ; Naoki Maruyama^d ; Kazuhiro Shigemoto^a ; ^{kazshige@tmig.or.jp}
• 关键词：Myasthenia gravis ; MuSK ; 3 ; 4-Diaminopyridine
• 刊名：Journal of Neuroimmunology
• 出版年：2012
• 期刊代码：32_01655728
• 类别：neu
• 出版时间：April, 2012
• 卷：245
• 期：1-2
• 页码：75-78
• 文件大小：305 K

摘要

This study investigated the effect of 3,4-diaminopyridine (3,4-DAP), a potent potentiator of transmitter release, on neuromuscular transmission in vivo in a mouse model of myasthenia gravis (MG) caused by antibodies against muscle-specific kinase (MuSK; MuSK-MG) and ex vivo in diaphragm muscle from these mice. 3,4-DAP significantly improved neuromuscular transmission, predominantly by increasing acetylcholine (ACh) release, supporting presynaptic potentiation as an effective treatment strategy for MuSK-MG patients who have defective transmitter release. In MuSK-MG, we suggest that only low-dose acetylcholinesterase (AChE) inhibitors be used to avoid side effects, and we propose that 3,4-DAP may be effective as a symptomatic therapy.