The autoimmune regulator AIRE in thymoma biology: autoimmunity and beyond
Thymomas are tumors of thymic epithelial cells. They associate more often than any other human tumors with various autoimmune diseases; myasthenia gravis is the commonest, occurring in 10-50% of thymoma patients, depending on the World Health Organization-defined histologic subtype. Most thymomas ge...
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| Hauptverfasser: | , , , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
2010
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| In: |
Journal of thoracic oncology
Year: 2010, Jahrgang: 5, Heft: 10, Pages: S266-S272 |
| ISSN: | 1556-1380 |
| DOI: | 10.1097/JTO.0b013e3181f1f63f |
| Online-Zugang: | Resolving-System, lizenzpflichtig, Volltext: https://doi.org/10.1097/JTO.0b013e3181f1f63f Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S1556086415326149 
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| Verfasserangaben: | Alexander Marx, Peter Hohenberger, Hans Hoffmann, Joachim Pfannschmidt, Philipp Schnabel, Hans-Stefan Hofmann, Karsten Wiebe, Berthold Schalke, Wilfred Nix, Ralf Gold, Nick Willcox, Pärt Peterson, and Philipp Ströbel |
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| 520 | |a Thymomas are tumors of thymic epithelial cells. They associate more often than any other human tumors with various autoimmune diseases; myasthenia gravis is the commonest, occurring in 10-50% of thymoma patients, depending on the World Health Organization-defined histologic subtype. Most thymomas generate many polyclonal maturing T lymphocytes but in disorganized microenvironments Failure to induce self-tolerance may be a key factor leading to the export of potentially autoreactive CD4+ progeny, thus predisposing to autoimmune diseases. Normally, the master Autoimmune Regulator promotes expression of peripheral tissue-restricted antigens such as insulin by medullary thymic epithelial cells and induction of tolerance to them. The failure of ∼95% of thymomas to express autoimmune regulator is another feature potentially contributing to autoimmunity. | ||
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