Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture
Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ). Autoantibodies target key molecules at the NMJ, such as the nicotinic acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4), that lead by a range...
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| Format: | Article |
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MDPI AG
2019-07-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/8/7/671 |
| _version_ | 1797716631331274752 |
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| author | Inga Koneczny Ruth Herbst |
| author_facet | Inga Koneczny Ruth Herbst |
| author_sort | Inga Koneczny |
| collection | DOAJ |
| description | Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ). Autoantibodies target key molecules at the NMJ, such as the nicotinic acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4), that lead by a range of different pathogenic mechanisms to altered tissue architecture and reduced densities or functionality of AChRs, reduced neuromuscular transmission, and therefore a severe fatigable skeletal muscle weakness. In this review, we give an overview of the history and clinical aspects of MG, with a focus on the structure and function of myasthenic autoantigens at the NMJ and how they are affected by the autoantibodies’ pathogenic mechanisms. Furthermore, we give a short overview of the cells that are implicated in the production of the autoantibodies and briefly discuss diagnostic challenges and treatment strategies. |
| first_indexed | 2024-03-12T08:24:19Z |
| format | Article |
| id | doaj.art-07b560b3b4f048d999ae6c4b6a9f39bf |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-12T08:24:19Z |
| publishDate | 2019-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-07b560b3b4f048d999ae6c4b6a9f39bf2023-09-02T18:11:37ZengMDPI AGCells2073-44092019-07-018767110.3390/cells8070671cells8070671Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular ArchitectureInga Koneczny0Ruth Herbst1Institute of Neurology, Medical University of Vienna, 1090 Vienna, AustriaCenter for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, 1090 Vienna, AustriaMyasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ). Autoantibodies target key molecules at the NMJ, such as the nicotinic acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4), that lead by a range of different pathogenic mechanisms to altered tissue architecture and reduced densities or functionality of AChRs, reduced neuromuscular transmission, and therefore a severe fatigable skeletal muscle weakness. In this review, we give an overview of the history and clinical aspects of MG, with a focus on the structure and function of myasthenic autoantigens at the NMJ and how they are affected by the autoantibodies’ pathogenic mechanisms. Furthermore, we give a short overview of the cells that are implicated in the production of the autoantibodies and briefly discuss diagnostic challenges and treatment strategies.https://www.mdpi.com/2073-4409/8/7/671myasthenia gravisneuromuscular junctionAChRMuSKLrp4Agrinautoimmunityautoantibodieshistoryimmunopathogenesis |
| spellingShingle | Inga Koneczny Ruth Herbst Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture Cells myasthenia gravis neuromuscular junction AChR MuSK Lrp4 Agrin autoimmunity autoantibodies history immunopathogenesis |
| title | Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture |
| title_full | Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture |
| title_fullStr | Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture |
| title_full_unstemmed | Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture |
| title_short | Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture |
| title_sort | myasthenia gravis pathogenic effects of autoantibodies on neuromuscular architecture |
| topic | myasthenia gravis neuromuscular junction AChR MuSK Lrp4 Agrin autoimmunity autoantibodies history immunopathogenesis |
| url | https://www.mdpi.com/2073-4409/8/7/671 |
| work_keys_str_mv | AT ingakoneczny myastheniagravispathogeniceffectsofautoantibodiesonneuromusculararchitecture AT ruthherbst myastheniagravispathogeniceffectsofautoantibodiesonneuromusculararchitecture |