Alexandra C Bayer, Richard J Nowak, Kevin C O'Connor
{"title":"细胞免疫失调在重症肌无力病理中的作用。","authors":"Alexandra C Bayer, Richard J Nowak, Kevin C O'Connor","doi":"10.1016/bs.irn.2025.04.035","DOIUrl":null,"url":null,"abstract":"<p><p>Myasthenia gravis (MG) is an autoimmune disorder in which autoantibodies attack proteins at the neuromuscular junction, resulting in impaired neuromuscular transmission. Like other autoimmune diseases, MG arises when the immune system fails to distinguish self from non-self, attacking and damaging normal tissues. The pathological response involves not only B cells, responsible for autoantibody production, but also T cells, which provide essential support for B cell pathogenicity. While the precise triggers of this abnormal immune response remain undefined, MG is recognized as a multifactorial disease influenced by immune dysregulation along with genetic and environmental factors. This chapter explores the complex immunopathology of MG, highlighting how these factors collectively contribute to disease development. We examine the physiological development of T and B cell compartments, the tolerance checkpoints designed to prevent autoimmunity, and the consequences of their failure. Finally, we discuss the dysregulation of these cellular compartments in MG, emphasizing their roles in disease progression, the persistence of autoimmunity, and responses to treatment.</p>","PeriodicalId":94058,"journal":{"name":"International review of neurobiology","volume":"182 ","pages":"43-66"},"PeriodicalIF":0.0000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Contribution of cellular immune dysregulation to myasthenia gravis pathology.\",\"authors\":\"Alexandra C Bayer, Richard J Nowak, Kevin C O'Connor\",\"doi\":\"10.1016/bs.irn.2025.04.035\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Myasthenia gravis (MG) is an autoimmune disorder in which autoantibodies attack proteins at the neuromuscular junction, resulting in impaired neuromuscular transmission. Like other autoimmune diseases, MG arises when the immune system fails to distinguish self from non-self, attacking and damaging normal tissues. The pathological response involves not only B cells, responsible for autoantibody production, but also T cells, which provide essential support for B cell pathogenicity. While the precise triggers of this abnormal immune response remain undefined, MG is recognized as a multifactorial disease influenced by immune dysregulation along with genetic and environmental factors. This chapter explores the complex immunopathology of MG, highlighting how these factors collectively contribute to disease development. We examine the physiological development of T and B cell compartments, the tolerance checkpoints designed to prevent autoimmunity, and the consequences of their failure. Finally, we discuss the dysregulation of these cellular compartments in MG, emphasizing their roles in disease progression, the persistence of autoimmunity, and responses to treatment.</p>\",\"PeriodicalId\":94058,\"journal\":{\"name\":\"International review of neurobiology\",\"volume\":\"182 \",\"pages\":\"43-66\"},\"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.2025.04.035\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/5/20 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.2025.04.035","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/5/20 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
Contribution of cellular immune dysregulation to myasthenia gravis pathology.
Myasthenia gravis (MG) is an autoimmune disorder in which autoantibodies attack proteins at the neuromuscular junction, resulting in impaired neuromuscular transmission. Like other autoimmune diseases, MG arises when the immune system fails to distinguish self from non-self, attacking and damaging normal tissues. The pathological response involves not only B cells, responsible for autoantibody production, but also T cells, which provide essential support for B cell pathogenicity. While the precise triggers of this abnormal immune response remain undefined, MG is recognized as a multifactorial disease influenced by immune dysregulation along with genetic and environmental factors. This chapter explores the complex immunopathology of MG, highlighting how these factors collectively contribute to disease development. We examine the physiological development of T and B cell compartments, the tolerance checkpoints designed to prevent autoimmunity, and the consequences of their failure. Finally, we discuss the dysregulation of these cellular compartments in MG, emphasizing their roles in disease progression, the persistence of autoimmunity, and responses to treatment.