{"title":"重症肌无力患者自身抗体介导补体活性的测定、发展和测量","authors":"Abeer H. Obaid","doi":"10.17161/rrnmf.v4i3.19545","DOIUrl":null,"url":null,"abstract":"Autoantibodies against the acetylcholine receptor (AChR) play a critical role in myasthenia gravis, where autoantibody-mediated complement activation has been implicated in neuromuscular junction damage. However, the exact pathogenic role of the autoantibodies in complement activation remains unclear. We developed a cell-based assay that measures AChR autoantibody–mediated complement membrane attack complex (MAC) formation. A modified HEK293T cell line using CRISPR/Cas9 genome editing to disrupt expression of the complement regulator genes (CD46, CD55, and CD59)—was used to measure AChR autoantibody–mediated membrane attack complex (MAC) formation through flow cytometry. We observed a modest correlation between autoantibody-mediated complement mediated activity and disease burden suggesting heterogeneity in autoantibody-mediated activation of complement system. ","PeriodicalId":309700,"journal":{"name":"RRNMF Neuromuscular Journal","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2023-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Assay Development and Measurement of Autoantibody-Mediated Complement Activity in Myasthenia Gravis\",\"authors\":\"Abeer H. Obaid\",\"doi\":\"10.17161/rrnmf.v4i3.19545\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Autoantibodies against the acetylcholine receptor (AChR) play a critical role in myasthenia gravis, where autoantibody-mediated complement activation has been implicated in neuromuscular junction damage. However, the exact pathogenic role of the autoantibodies in complement activation remains unclear. We developed a cell-based assay that measures AChR autoantibody–mediated complement membrane attack complex (MAC) formation. A modified HEK293T cell line using CRISPR/Cas9 genome editing to disrupt expression of the complement regulator genes (CD46, CD55, and CD59)—was used to measure AChR autoantibody–mediated membrane attack complex (MAC) formation through flow cytometry. We observed a modest correlation between autoantibody-mediated complement mediated activity and disease burden suggesting heterogeneity in autoantibody-mediated activation of complement system. \",\"PeriodicalId\":309700,\"journal\":{\"name\":\"RRNMF Neuromuscular Journal\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-08-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"RRNMF Neuromuscular Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.17161/rrnmf.v4i3.19545\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"RRNMF Neuromuscular Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.17161/rrnmf.v4i3.19545","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Assay Development and Measurement of Autoantibody-Mediated Complement Activity in Myasthenia Gravis
Autoantibodies against the acetylcholine receptor (AChR) play a critical role in myasthenia gravis, where autoantibody-mediated complement activation has been implicated in neuromuscular junction damage. However, the exact pathogenic role of the autoantibodies in complement activation remains unclear. We developed a cell-based assay that measures AChR autoantibody–mediated complement membrane attack complex (MAC) formation. A modified HEK293T cell line using CRISPR/Cas9 genome editing to disrupt expression of the complement regulator genes (CD46, CD55, and CD59)—was used to measure AChR autoantibody–mediated membrane attack complex (MAC) formation through flow cytometry. We observed a modest correlation between autoantibody-mediated complement mediated activity and disease burden suggesting heterogeneity in autoantibody-mediated activation of complement system.
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