Thymus and autoimmunity

• Verfasst von: Marx, Alexander [VerfasserIn]
• Verfasst von: Yamada, Yosuke [VerfasserIn]
• Verfasst von: Simon-Keller, Katja [VerfasserIn]
• Verfasst von: Schalke, Berthold [VerfasserIn]
• Verfasst von: Willcox, Nick [VerfasserIn]
• Verfasst von: Ströbel, Philipp [VerfasserIn]
• Verfasst von: Weis, Cleo-Aron Thias [VerfasserIn]
• Titel: Thymus and autoimmunity
• Verf.angabe: Alexander Marx, Yosuke Yamada, Katja Simon-Keller, Berthold Schalke, Nick Willcox, Philipp Ströbel, Cleo-Aron Weis
• E-Jahr: 2021
• Jahr: 3 February 2021
• Umfang: 20 S.
• Fussnoten: Gesehen am 05.05.2021
• Titel Quelle: Enthalten in: Seminars in immunopathology
• Ort Quelle: Berlin : Springer, 2007
• Jahr Quelle: 2021
• Band/Heft Quelle: 43(2021), 1, Seite 45-64
• ISSN Quelle: 1863-2300
• DOI: doi:10.1007/s00281-021-00842-3
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1757137289

Abstract

The thymus prevents autoimmune diseases through mechanisms that operate in the cortex and medulla, comprising positive and negative selection and the generation of regulatory T-cells (Tregs). Egress from the thymus through the perivascular space (PVS) to the blood is another possible checkpoint, as shown by some autoimmune/immunodeficiency syndromes. In polygenic autoimmune diseases, subtle thymic dysfunctions may compound genetic, hormonal and environmental cues. Here, we cover (a) tolerance-inducing cell types, whether thymic epithelial or tuft cells, or dendritic, B- or thymic myoid cells; (b) tolerance-inducing mechanisms and their failure in relation to thymic anatomic compartments, and with special emphasis on human monogenic and polygenic autoimmune diseases and the related thymic pathologies, if known; (c) polymorphisms and mutations of tolerance-related genes with an impact on positive selection (e.g. the gene encoding the thymoproteasome-specific subunit, PSMB11), promiscuous gene expression (e.g. AIRE, PRKDC, FEZF2, CHD4), Treg development (e.g. SATB1, FOXP3), T-cell migration (e.g. TAGAP) and egress from the thymus (e.g. MTS1, CORO1A); (d) myasthenia gravis as the prototypic outcome of an inflamed or disordered neoplastic ‘sick thymus’.