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 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.
Marx
Alexander
A
Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Heidelberg, Germany. Alexander.marx@umm.de
Hohenberger
Peter
P
Hoffmann
Hans
H
Pfannschmidt
Joachim
J
Schnabel
Philipp
P
Hofmann
Hans-Stefan
HS
Wiebe
Karsten
K
Schalke
Berthold
B
Nix
Wilfred
W
Gold
Ralf
R
Willcox
Nick
N
Peterson
Pärt
P
Ströbel
Philipp
P
D001327:Autoimmune Diseases / Q000209:etiology* / Q000473:pathology; D015551:Autoimmunity; D006801:Humans; D013945:Thymoma / Q000150:complications / Q000276:immunology* / Q000378:metabolism*; D013953:Thymus Neoplasms / Q000150:complications / Q000276:immunology* / Q000378:metabolism*; D014157:Transcription Factors / Q000378:metabolism*; D000096927:AIRE Protein
D014157:Transcription Factors; D000096927:AIRE Protein