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Immune tolerance and the prevention of autoimmune diseases essentially depend on thymic tissue homeostasis
The intricate balance of immune reactions towards invading pathogens and immune
tolerance towards self is pivotal in preventing autoimmune diseases, with the thymus playing …
tolerance towards self is pivotal in preventing autoimmune diseases, with the thymus playing …
AIRE expands: new roles in immune tolerance and beyond
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 …
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
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 …
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 …
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 …
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 …
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 …
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
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 …
(mTECs) is essential for the induction of self-tolerance and prevents autoimmunity, with each …
Update on Aire and thymic negative selection
Twenty years ago, the autoimmune regulator (Aire) gene was associated with autoimmune
polyendocrinopathy–candidiasis–ectodermal dystrophy, and was cloned and sequenced. Its …
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 …
similarities.•Defects in AIRE synergize with defects in peripheral tolerance to promote …