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

被引：34
作者：
Mori, Shuuichi
Kishi, Masahiko [2 ]
Kubo, Sachiho
Akiyoshi, Takuyu
Yamada, Shigeru
Miyazaki, Tsuyoshi
Konishi, Tetsuro [3 ]
Maruyama, Naoki [4 ]
Shigemoto, Kazuhiro [1 ]
机构：
[1] Tokyo Metropolitan Inst Gerontol, Dept Geriatr Med, Itabashi Ku, Tokyo 1730015, Japan
[2] Toho Univ, Sakura Med Ctr, Dept Internal Med, Chiba 2858741, Japan
[3] Natl Utano Hosp, Natl Hosp Org, Dept Neurol, Kyoto 6168255, Japan
[4] Tokyo Metropolitan Inst Gerontol, Dept Mol Regulat Aging, Tokyo 1730015, Japan
来源：
JOURNAL OF NEUROIMMUNOLOGY | 2012年 / 245卷 / 1-2期
关键词：
Myasthenia gravis; MuSK; 3,4-Diaminopyridine; ACETYLCHOLINE-RELEASE; 4-AMINOPYRIDINE; JUNCTION; TRANSMITTER; MG;
D O I：
10.1016/j.jneuroim.2012.02.010
中图分类号：
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号：
100102 ;
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. (C) 2012 Elsevier B.V. All rights reserved.
引用
收藏
页码：75 / 78
页数：4
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