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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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Main Authors: Marx, Alexander (Author) , Hohenberger, Peter (Author) , Hoffmann, Hans (Author) , Pfannschmidt, Joachim (Author) , Schnabel, Philipp Albert (Author) , Hofmann, Hans-Stefan (Author) , Wiebe, Karsten (Author) , Schalke, Berthold (Author) , Nix, Wilfred (Author) , Gold, Ralf (Author) , Willcox, Nick (Author) , Peterson, Pärt (Author) , Ströbel, Philipp (Author)
Format: Article (Journal)
Language:English
Published: 2010
In: Journal of thoracic oncology
Year: 2010, Volume: 5, Issue: 10, Pages: S266-S272
ISSN:1556-1380
DOI:10.1097/JTO.0b013e3181f1f63f
Online Access: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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Author Notes: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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Summary: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.
Item Description:Online veröffentlicht am 20. November 2015
Gesehen am 23.05.2023
Physical Description:Online Resource
ISSN:1556-1380
DOI:10.1097/JTO.0b013e3181f1f63f

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