ArticleScience translational medicine2009
Identification of an autoantigen demonstrates a link between interstitial lung disease and a defect in central tolerance.
Article in Science translational medicine, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 68 citations in OpenAlex.
- Distinct mural cells and fibroblasts promote pathogenic plasma cell accumulation in idiopathic pulmonary fibrosis.The European respiratory journal · 2025Article
- The Interaction Between Vasculogenic Mimicry and the Immune System: Mechanistic Insights and Dual Exploration in Cancer Therapy.Cell proliferation · 2025Review
- Multifunctional acyltransferase HBO1: a key regulatory factor for cellular functions.Cellular & molecular biology letters · 2024Review
- Validation of a murine proteome-wide phage display library for identification of autoantibody specificities.JCI insight · 2023Article
- Genes and Microbiota Interaction in Monogenic Autoimmune Disorders.Biomedicines · 2023Review
- Plasma cell but not CD20-mediated B-cell depletion protects from bleomycin-induced lung fibrosis.The European respiratory journal · 2022Article
- Autoantibodies are present in the bronchoalveolar lavage but not circulation in patients with fibrotic interstitial lung disease.ERJ open research · 2022Article
- Infections in the monogenic autoimmune syndrome APECED.Current opinion in immunology · 2021Review
- B Cells and Autoantibodies in AIRE Deficiency.Biomedicines · 2021Review
- Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy.Frontiers in pediatrics · 2021Review
- GILT in Thymic Epithelial Cells Facilitates Central CD4 T Cell Tolerance to a Tissue-Restricted, Melanoma-Associated Self-Antigen.Journal of immunology (Baltimore, Md. : 1950) · 2020Article
- Article
- A Defect in Thymic Tolerance Causes T Cell-Mediated Autoimmunity in a Murine Model of COPA Syndrome.Journal of immunology (Baltimore, Md. : 1950) · 2020Article
- An AIREless Breath: Pneumonitis Caused by Impaired Central Immune Tolerance.Frontiers in immunology · 2020Review
- Susceptible loci associated with autoimmune disease as potential biomarkers for checkpoint inhibitor-induced immune-related adverse events.ESMO open · 2019Review
- IL-10 Paradoxically Promotes Autoimmune Neuropathy through S1PR1-Dependent CD4Journal of immunology (Baltimore, Md. : 1950) · 2018Article
- Aged polymorphonuclear leukocytes cause fibrotic interstitial lung disease in the absence of regulation by B cells.Nature immunology · 2018Article
- Twenty Years of AIRE.Frontiers in immunology · 2018Review
- Insights into immune tolerance from AIRE deficiency.Current opinion in immunology · 2017Review
- Idiopathic pulmonary fibrosis and a role for autoimmunity.Immunology and cell biology · 2017Review
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 2 countries.
Funding
Abstract
Interstitial lung disease (ILD) is a common manifestation of systemic autoimmunity characterized by progressive inflammation or scarring of the lungs. Patients who develop these complications can exhibit significantly impaired gas exchange that may result in hypoxemia, pulmonary hypertension, and even death. Unfortunately, little is understood about how these diseases arise, including the role of specific defects in immune tolerance. Another key question is whether autoimmune responses targeting the lung parenchyma are critical to ILD pathogenesis, including that of isolated idiopathic forms. We show that a specific defect in central tolerance brought about by mutations in the autoimmune regulator gene (Aire) leads to an autoreactive T cell response to a lung antigen named vomeromodulin and the development of ILD. We found that a human patient and mice with defects in Aire develop similar lung pathology, demonstrating that the AIRE-deficient model of autoimmunity is a suitable translational system in which to unravel fundamental mechanisms of ILD pathogenesis.
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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.