Journal of Neuroimmunology 2012-04-01

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

Shuuichi Mori, Masahiko Kishi, Sachiho Kubo, Takuyu Akiyoshi, Shigeru Yamada, Tsuyoshi Miyazaki, Tetsuro Konishi, Naoki Maruyama, Kazuhiro Shigemoto

Index: J. Neuroimmunol. 245(1-2) , 75-8, (2012)

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Abstract

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.Copyright © 2012 Elsevier B.V. All rights reserved.