title: The autoimmune regulator AIRE in thymoma biology: autoimmunity and beyond.
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.
authors.affiliation: Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Heidelberg, Germany. Alexander.marx@umm.de
authors.forename: Alexander
authors.initials: A
authors.lastname: Marx
authors.forename: Peter
authors.initials: P
authors.lastname: Hohenberger
authors.forename: Hans
authors.initials: H
authors.lastname: Hoffmann
authors.forename: Joachim
authors.initials: J
authors.lastname: Pfannschmidt
authors.forename: Philipp
authors.initials: P
authors.lastname: Schnabel
authors.forename: Hans-Stefan
authors.initials: HS
authors.lastname: Hofmann
authors.forename: Karsten
authors.initials: K
authors.lastname: Wiebe
authors.forename: Berthold
authors.initials: B
authors.lastname: Schalke
authors.forename: Wilfred
authors.initials: W
authors.lastname: Nix
authors.forename: Ralf
authors.initials: R
authors.lastname: Gold
authors.forename: Nick
authors.initials: N
authors.lastname: Willcox
authors.forename: Pärt
authors.initials: P
authors.lastname: Peterson
authors.forename: Philipp
authors.initials: P
authors.lastname: Ströbel
mesh_terms: 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
chemical_list: D014157:Transcription Factors; D000096927:AIRE Protein