The Autoimmune Regulator AIRE in Thymoma Biology: Autoimmunity and Beyond

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 (+1 others)
2010 Journal of Thoracic Oncology  
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 ϩ
more » ... eactive CD4 ϩ 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 ϳ95% of thymomas to express autoimmune regulator is another feature potentially contributing to autoimmunity. S267 FIGURE 3. AIRE expression in the normal thymus. a, Expression of AIRE (blue) in the nucleus of one of the pancytokeratin-expressing (green; antibodiy AE1/AE3) medullary thymic epithelial cells (mTECs). b, "Promiscuous" expression of thyroglobulin (green) in one of the AIRE-expressing mTECs (blue nuclei). Immunofluorescence (ϫ400).
doi:10.1097/jto.0b013e3181f1f63f pmid:20859117 fatcat:do5ju6vchzbcfinywtsara43gm