Immune tolerance and the prevention of autoimmune diseases essentially depend on thymic tissue homeostasis

F Shirafkan, L Hensel, K Rattay - Frontiers in Immunology, 2024 - frontiersin.org
The intricate balance of immune reactions towards invading pathogens and immune
tolerance towards self is pivotal in preventing autoimmune diseases, with the thymus playing …

AIRE expands: new roles in immune tolerance and beyond

MS Anderson, MA Su - Nature Reviews Immunology, 2016 - nature.com
More than 15 years ago, mutations in the autoimmune regulator (AIRE) gene were identified
as the cause of autoimmune polyglandular syndrome type 1 (APS1). It is now clear that this …

Thymic tuft cells promote an IL-4-enriched medulla and shape thymocyte development

CN Miller, I Proekt, J von Moltke, KL Wells… - Nature, 2018 - nature.com
The thymus is responsible for generating a diverse yet self-tolerant pool of T cells. Although
the thymic medulla consists mostly of develo** and mature AIRE+ epithelial cells, recent …

Lessons from primary immunodeficiencies: autoimmune regulator and autoimmune polyendocrinopathy‐candidiasis‐ectodermal dystrophy

GM Constantine, MS Lionakis - Immunological reviews, 2019 - Wiley Online Library
The discovery of the autoimmune regulator (AIRE) protein and the delineation of its critical
contributions in the establishment of central immune tolerance has significantly expanded …

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy

EMN Ferré, MM Schmitt, MS Lionakis - Frontiers in pediatrics, 2021 - frontiersin.org
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), also known
as autoimmune polyglandular syndrome type-1 (APS-1), is a rare monogenic autoimmune …

Twenty years of AIRE

R Perniola - Frontiers in immunology, 2018 - frontiersin.org
About two decades ago, cloning of the autoimmune regulator (AIRE) gene materialized one
of the most important actors on the scene of self-tolerance. Thymic transcription of genes …

Aire in autoimmunity

CN Miller, MR Waterfield, JM Gardner… - Annual Review of …, 2024 - annualreviews.org
The role of the autoimmune regulator (Aire) in central immune tolerance and thymic self-
representation was first described more than 20 years ago, but fascinating new insights into …

Single-cell transcriptome analysis reveals coordinated ectopic gene-expression patterns in medullary thymic epithelial cells

P Brennecke, A Reyes, S Pinto, K Rattay… - Nature …, 2015 - nature.com
Expression of tissue-restricted self antigens (TRAs) in medullary thymic epithelial cells
(mTECs) is essential for the induction of self-tolerance and prevents autoimmunity, with each …

Update on Aire and thymic negative selection

GA Passos, CA Speck‐Hernandez, AF Assis… - …, 2018 - Wiley Online Library
Twenty years ago, the autoimmune regulator (Aire) gene was associated with autoimmune
polyendocrinopathy–candidiasis–ectodermal dystrophy, and was cloned and sequenced. Its …

Insights into immune tolerance from AIRE deficiency

I Proekt, CN Miller, MS Lionakis… - Current opinion in …, 2017 - Elsevier
Highlights•APS-1/APECED patients exhibit marked phenotypic variability despite genotypic
similarities.•Defects in AIRE synergize with defects in peripheral tolerance to promote …