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

作者：Mori Shuuichi; Kishi Masahiko; Kubo Sachiho; Akiyoshi Takuyu; Yamada Shigeru; Miyazaki Tsuyoshi; Konishi Tetsuro; Maruyama Naoki; Shigemoto Kazuhiro^*

来源：Journal of Neuroimmunology, 2012, 245(1-2): 75-78.

DOI：10.1016/j.jneuroim.2012.02.010

• Myasthenia gravis
• MuSK
• 3,4-Diaminopyridine

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.

• 出版日期2012-4