| Literature DB >> 22409941 |
Shuuichi Mori1, Masahiko Kishi, Sachiho Kubo, Takuyu Akiyoshi, Shigeru Yamada, Tsuyoshi Miyazaki, Tetsuro Konishi, Naoki Maruyama, Kazuhiro Shigemoto.
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.Entities:
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Year: 2012 PMID： 22409941 DOI： 10.1016/j.jneuroim.2012.02.010
Source DB: PubMed Journal: J Neuroimmunol ISSN： 0165-5728 Impact factor: 3.478