ArticleJournal of molecular medicine (Berlin, Germany)2008
Critical immunological pathways are downregulated in APECED patient dendritic cells.
Article in Journal of molecular medicine (Berlin, Germany), 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 43 citations in OpenAlex.
- Chronic mucocutaneous candidiasis in APECED or thymoma patients correlates with autoimmunity to Th17-associated cytokines.The Journal of experimental medicine · 2010Trial
- Preliminary Evidence for Autoimmune Regulator Occupancy at Promoter Regions of Known Autoantigens in Human Peripheral Lymphocytes Obtained by Chromatin Immunoprecipitation Assay.International journal of molecular sciences · 2026Article
- Novel homozygous mutations inHeliyon · 2024Article
- Extrathymic AIRE-Expressing Cells: A Historical Perspective.Advances in experimental medicine and biology · 2024Article
- Genes and Microbiota Interaction in Monogenic Autoimmune Disorders.Biomedicines · 2023Review
- The Role of Autoimmune Regulator (AIRE) in Peripheral Tolerance.Journal of immunology research · 2018Review
- Maturing Human CD127+ CCR7+ PDL1+ Dendritic Cells Express AIRE in the Absence of Tissue Restricted Antigens.Frontiers in immunology · 2018Article
- Human APECED; a Sick Thymus Syndrome?Frontiers in immunology · 2013Review
- Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy: insights into genotype-phenotype correlation.International journal of endocrinology · 2012Article
- Expression of the autoimmune regulator gene and its relevance to the mechanisms of central and peripheral tolerance.Clinical & developmental immunology · 2012Review
- Expression and function of the autoimmune regulator (Aire) gene in non-thymic tissue.Clinical and experimental immunology · 2011Review
- Pattern recognition receptor expression is not impaired in patients with chronic mucocutanous candidiasis with or without autoimmune polyendocrinopathy candidiasis ectodermal dystrophy.Clinical and experimental immunology · 2009Article
- Transcriptional regulation by AIRE: molecular mechanisms of central tolerance.Nature reviews. Immunology · 2008Review
Corrections and comments
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Authors and funding
7 authors at 2 institutions in 3 countries.
Funding
Abstract
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a monogenic autoimmune disease caused by mutations in the autoimmune regulator (AIRE) gene. AIRE functions as a transcriptional regulator, and it has a central role in the development of immunological tolerance. AIRE regulates the expression of ectopic antigens in epithelial cells of the thymic medulla and has been shown to participate in the development of peripheral tolerance. However, the mechanism of action of AIRE has remained elusive. To further investigate the role of AIRE in host immune functions, we studied the properties and transcript profiles in in vitro monocyte-differentiated dendritic cells (moDCs) obtained from APECED patients and healthy controls. AIRE-deficient monocytes showed typical DC morphology and expressed DC marker proteins cluster of differentiation 86 and human leukocyte antigen class II. APECED patient-derived moDCs were functionally impaired: the transcriptional response of cytokine genes to pathogens was drastically reduced. Interestingly, some changes were observable already at the immature DC stage. Pathway analyses of transcript profiles revealed that the expression of the components of the host cell signaling pathways involved in cell-cell signalling, innate immune responses, and cytokine activity were reduced in APECED moDCs. Our observations support a role for AIRE in peripheral tolerance and are the first ones to show that AIRE has a critical role in DC responses to microbial stimuli in humans.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.