Marx, Alexander and Yamada, Yosuke and Simon-Keller, Katja and Schalke, Berthold and Willcox, Nick and Stroebel, Philipp and Weis, Cleo-Aron (2021) Thymus and autoimmunity. SEMINARS IN IMMUNOPATHOLOGY, 43 (1). pp. 45-64. ISSN 1863-2297, 1863-2300
Full text not available from this repository. (Request a copy)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'.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | MYASTHENIA-GRAVIS; MECHANISMS; THYMOMA; AIRE; Thymus; Myasthenia gravis; Tuft cells; Myoid cells; AIRE; FEZF2 |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurologie |
| Depositing User: | Petra Gürster |
| Date Deposited: | 18 Jan 2023 05:57 |
| Last Modified: | 29 Feb 2024 14:03 |
| URI: | https://pred.uni-regensburg.de/id/eprint/47617 |
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