重症肌无力动物模型。

International review of neurobiology Pub Date : 2025-01-01 Epub Date: 2025-07-09 DOI:10.1016/bs.irn.2025.04.029
Patricia M Sikorski, Linda L Kusner
{"title":"重症肌无力动物模型。","authors":"Patricia M Sikorski, Linda L Kusner","doi":"10.1016/bs.irn.2025.04.029","DOIUrl":null,"url":null,"abstract":"<p><p>Animal models offer a platform to advance our understanding of myasthenia gravis (MG), an autoimmune disorder characterized by impaired neuromuscular transmission. Experimental autoimmune MG models (EAMG) actively induce autoimmunity through antigen immunization, aiding in understanding the immune response to self. Passive transfer models (PTMG) involve the injection of pathogenic antibodies into animals, providing insights into antibody-mediated mechanisms and complement-driven damage to the neuromuscular junction (NMJ). The pathogenic effect of autoantibodies targeting acetylcholine receptors (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4) results in changes to the NMJ that are mechanistically distinct. These models validate therapeutic interventions preclinically, with methodologies ranging from antibody transfer to genetic modifications. Despite the translational challenges, these models bridge preclinical research and clinical applications, enabling the development of targeted treatments for MG.</p>","PeriodicalId":94058,"journal":{"name":"International review of neurobiology","volume":"182 ","pages":"145-160"},"PeriodicalIF":0.0000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Animal models of myasthenia gravis.\",\"authors\":\"Patricia M Sikorski, Linda L Kusner\",\"doi\":\"10.1016/bs.irn.2025.04.029\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Animal models offer a platform to advance our understanding of myasthenia gravis (MG), an autoimmune disorder characterized by impaired neuromuscular transmission. Experimental autoimmune MG models (EAMG) actively induce autoimmunity through antigen immunization, aiding in understanding the immune response to self. Passive transfer models (PTMG) involve the injection of pathogenic antibodies into animals, providing insights into antibody-mediated mechanisms and complement-driven damage to the neuromuscular junction (NMJ). The pathogenic effect of autoantibodies targeting acetylcholine receptors (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4) results in changes to the NMJ that are mechanistically distinct. These models validate therapeutic interventions preclinically, with methodologies ranging from antibody transfer to genetic modifications. Despite the translational challenges, these models bridge preclinical research and clinical applications, enabling the development of targeted treatments for MG.</p>\",\"PeriodicalId\":94058,\"journal\":{\"name\":\"International review of neurobiology\",\"volume\":\"182 \",\"pages\":\"145-160\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"International review of neurobiology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1016/bs.irn.2025.04.029\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/7/9 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"International review of neurobiology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1016/bs.irn.2025.04.029","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/7/9 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

动物模型为我们理解重症肌无力(MG)提供了一个平台,重症肌无力是一种以神经肌肉传递受损为特征的自身免疫性疾病。实验性自身免疫MG模型(EAMG)通过抗原免疫积极诱导自身免疫,有助于理解对自身的免疫反应。被动转移模型(PTMG)涉及将致病性抗体注射到动物体内,为抗体介导的机制和补体驱动的神经肌肉连接处损伤(NMJ)提供了见解。针对乙酰胆碱受体(AChR)、肌肉特异性激酶(MuSK)和低密度脂蛋白受体相关蛋白4 (Lrp4)的自身抗体的致病作用可导致NMJ发生机制不同的变化。这些模型验证了临床前的治疗干预,方法从抗体转移到基因修饰。尽管存在转化方面的挑战,但这些模型在临床前研究和临床应用之间架起了桥梁,使MG靶向治疗的开发成为可能。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Animal models of myasthenia gravis.

Animal models offer a platform to advance our understanding of myasthenia gravis (MG), an autoimmune disorder characterized by impaired neuromuscular transmission. Experimental autoimmune MG models (EAMG) actively induce autoimmunity through antigen immunization, aiding in understanding the immune response to self. Passive transfer models (PTMG) involve the injection of pathogenic antibodies into animals, providing insights into antibody-mediated mechanisms and complement-driven damage to the neuromuscular junction (NMJ). The pathogenic effect of autoantibodies targeting acetylcholine receptors (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4) results in changes to the NMJ that are mechanistically distinct. These models validate therapeutic interventions preclinically, with methodologies ranging from antibody transfer to genetic modifications. Despite the translational challenges, these models bridge preclinical research and clinical applications, enabling the development of targeted treatments for MG.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信