2017年11月
Immunization of mice with LRP4 induces myasthenia similar to MuSK-associated myasthenia gravis
EXPERIMENTAL NEUROLOGY
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- 巻
- 297
- 号
- 開始ページ
- 158
- 終了ページ
- 167
- 記述言語
- 英語
- 掲載種別
- 研究論文(学術雑誌)
- DOI
- 10.1016/j.expneurol.2017.08.006
- 出版者・発行元
- ACADEMIC PRESS INC ELSEVIER SCIENCE
Since the first report of experimental animal models of myasthenia gravis (MG) with autoantibodies against low density lipoprotein receptor-related protein 4 (LRP4), there have not been any major reports replicating the pathogenicity of anti-LRP4 antibodies (Abs). Recent clinical studies have cast doubt on the specificity and pathogenicity of anti-LRP4 antibodies for MG, highlighting the need for further research. In this study, we purified antigens corresponding to the extracellular region of human LRP4 stably expressed with chaperones in 293 cells and used these antigens to immunize female A/J mice. Immunization with LRP4 protein caused mice to develop myasthenia having similar electrophysiological and histological features as are observed in MG patients with circulating Abs against muscle-specific kinase (MuSK). Our results clearly demonstrate that active immunization of mice with LRP4 proteins causes myasthenia similar to the MG induced by anti-MuSK Abs. Further experimental and clinical studies are required to prove the pathogenicity of anti-LRP4 Abs in MG patients.
- リンク情報
- ID情報
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- DOI : 10.1016/j.expneurol.2017.08.006
- ISSN : 0014-4886
- eISSN : 1090-2430
- PubMed ID : 28823823
- Web of Science ID : WOS:000412617300016