The autoimmune regulator AIRE in thymoma biology: autoimmunity and beyond - PubMed (original) (raw)

Review

. 2010 Oct;5(10 Suppl 4):S266-72.

doi: 10.1097/JTO.0b013e3181f1f63f.

Peter Hohenberger, Hans Hoffmann, Joachim Pfannschmidt, Philipp Schnabel, Hans-Stefan Hofmann, Karsten Wiebe, Berthold Schalke, Wilfred Nix, Ralf Gold, Nick Willcox, Pärt Peterson, Philipp Ströbel

Affiliations

Free article

Review

The autoimmune regulator AIRE in thymoma biology: autoimmunity and beyond

Alexander Marx et al. J Thorac Oncol. 2010 Oct.

Free article

Abstract

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 approximately 95% of thymomas to express autoimmune regulator is another feature potentially contributing to autoimmunity.

PubMed Disclaimer

Similar articles

Cited by

Publication types

MeSH terms

Substances

LinkOut - more resources