Myasthenia Gravis: An Acquired Interferonopathy?
Myasthenia gravis (MG) is a rare autoimmune disease mediated by antibodies against components of the neuromuscular junction, particularly the acetylcholine receptor (AChR). The thymus plays a primary role in AChR-MG patients. In early-onset AChR-MG and thymoma-associated MG, an interferon type I (IF...
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MDPI AG
2022-04-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/11/7/1218 |
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| author | Cloé A. Payet Axel You Odessa-Maud Fayet Nadine Dragin Sonia Berrih-Aknin Rozen Le Panse |
| author_facet | Cloé A. Payet Axel You Odessa-Maud Fayet Nadine Dragin Sonia Berrih-Aknin Rozen Le Panse |
| author_sort | Cloé A. Payet |
| collection | DOAJ |
| description | Myasthenia gravis (MG) is a rare autoimmune disease mediated by antibodies against components of the neuromuscular junction, particularly the acetylcholine receptor (AChR). The thymus plays a primary role in AChR-MG patients. In early-onset AChR-MG and thymoma-associated MG, an interferon type I (IFN-I) signature is clearly detected in the thymus. The origin of this chronic IFN-I expression in the thymus is not yet defined. IFN-I subtypes are normally produced in response to viral infection. However, genetic diseases called interferonopathies are associated with an aberrant chronic production of IFN-I defined as sterile inflammation. Some systemic autoimmune diseases also share common features with interferonopathies. This review aims to analyze the pathogenic role of IFN-I in these diseases as compared to AChR-MG in order to determine if AChR-MG could be an acquired interferonopathy. |
| first_indexed | 2024-03-09T11:59:18Z |
| format | Article |
| id | doaj.art-262d8201662b4e3caf249d0e12043273 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-09T11:59:18Z |
| publishDate | 2022-04-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-262d8201662b4e3caf249d0e120432732023-11-30T23:05:07ZengMDPI AGCells2073-44092022-04-01117121810.3390/cells11071218Myasthenia Gravis: An Acquired Interferonopathy?Cloé A. Payet0Axel You1Odessa-Maud Fayet2Nadine Dragin3Sonia Berrih-Aknin4Rozen Le Panse5Sorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, FranceSorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, FranceSorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, FranceSorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, FranceSorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, FranceSorbonne University, INSERM, Institute of Myology, Center of Research in Myology, F-75013 Paris, FranceMyasthenia gravis (MG) is a rare autoimmune disease mediated by antibodies against components of the neuromuscular junction, particularly the acetylcholine receptor (AChR). The thymus plays a primary role in AChR-MG patients. In early-onset AChR-MG and thymoma-associated MG, an interferon type I (IFN-I) signature is clearly detected in the thymus. The origin of this chronic IFN-I expression in the thymus is not yet defined. IFN-I subtypes are normally produced in response to viral infection. However, genetic diseases called interferonopathies are associated with an aberrant chronic production of IFN-I defined as sterile inflammation. Some systemic autoimmune diseases also share common features with interferonopathies. This review aims to analyze the pathogenic role of IFN-I in these diseases as compared to AChR-MG in order to determine if AChR-MG could be an acquired interferonopathy.https://www.mdpi.com/2073-4409/11/7/1218interferon type Iautoimmunitymyasthenia gravisthymusthymomagerminal center |
| spellingShingle | Cloé A. Payet Axel You Odessa-Maud Fayet Nadine Dragin Sonia Berrih-Aknin Rozen Le Panse Myasthenia Gravis: An Acquired Interferonopathy? Cells interferon type I autoimmunity myasthenia gravis thymus thymoma germinal center |
| title | Myasthenia Gravis: An Acquired Interferonopathy? |
| title_full | Myasthenia Gravis: An Acquired Interferonopathy? |
| title_fullStr | Myasthenia Gravis: An Acquired Interferonopathy? |
| title_full_unstemmed | Myasthenia Gravis: An Acquired Interferonopathy? |
| title_short | Myasthenia Gravis: An Acquired Interferonopathy? |
| title_sort | myasthenia gravis an acquired interferonopathy |
| topic | interferon type I autoimmunity myasthenia gravis thymus thymoma germinal center |
| url | https://www.mdpi.com/2073-4409/11/7/1218 |
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