ArticleEuropean journal of immunology2011
Functional type 1 regulatory T cells develop regardless of FOXP3 mutations in patients with IPEX syndrome.
Article in European journal of immunology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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Who cites it
35 citing papers in PubMed, 80 citations in OpenAlex.
- Divergent CD27 expression marks the Treg induction trajectory.Frontiers in immunology · 2026Article
- Development and consequences of red blood cell autoantibodies: warm autoimmune hemolytic anemia.Current opinion in immunology · 2025Review
- Article
- Defining Human Regulatory T Cells beyond FOXP3: The Need to Combine Phenotype with Function.Cells · 2024Review
- A case report of IPEX syndrome in Palestine: detailed family identification and breadth of disorders with the same defect.Frontiers in pediatrics · 2024Article
- The small molecule inhibitor BX-795 uncouples IL-2 production from inhibition of Th2 inflammation and induces CD4Frontiers in immunology · 2023Article
- Type 1 regulatory T cell-mediated tolerance in health and disease.Frontiers in immunology · 2022Review
- Current Strategies to Modulate Regulatory T Cell Activity in Allergic Inflammation.Frontiers in immunology · 2022Review
- Erratum: Type 1 regulatory T cell-mediated tolerance in health and disease.Frontiers in immunology · 2022Article
- IPEX Syndrome: Genetics and Treatment Options.Genes · 2021Review
- The Yin and Yang of Type 1 Regulatory T Cells: From Discovery to Clinical Application.Frontiers in immunology · 2021Review
- Harnessing Mechanisms of Immune Tolerance to Improve Outcomes in Solid Organ Transplantation: A Review.Frontiers in immunology · 2021Review
- IPEX as a Consequence of Alternatively Spliced FOXP3.Frontiers in pediatrics · 2020Review
- Regulatory T Cells in Pregnancy: It Is Not All About FoxP3.Frontiers in immunology · 2020Review
- Review
- High-Dose IL-2 Skews a Glucocorticoid-Driven IL-17Journal of immunology (Baltimore, Md. : 1950) · 2019Article
- The Tolerogenic Function of Regulatory T Cells in Pregnancy and Cancer.Frontiers in immunology · 2019Review
- Polysaccharide-experienced effector T cells induce IL-10 in FoxP3+ regulatory T cells to prevent pulmonary inflammation.Glycobiology · 2018Article
- APVO210: A Bispecific Anti-CD86-IL-10 Fusion Protein (ADAPTIR™) to Induce Antigen-Specific T Regulatory Type 1 Cells.Frontiers in immunology · 2018Article
- Systemic Activation of NRF2 Alleviates Lethal Autoimmune Inflammation in Scurfy Mice.Molecular and cellular biology · 2017Article
Corrections and comments
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Authors and funding
13 authors at 9 institutions in 2 countries.
Funding
Abstract
Mutations of forkhead box p3 (FOXP3), the master gene for naturally occurring regulatory T cells (nTregs), are responsible for the impaired function of nTregs, resulting in an autoimmune disease known as the immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. The relevance of other peripheral tolerance mechanisms, such as the presence and function of type 1 regulatory T (Tr1) cells, the major adaptive IL-10-producing Treg subset, in patients with IPEX syndrome remains to be clarified. FOXP3(mutated) Tr1-polarized cells, differentiated in vitro from CD4(+) T cells of four IPEX patients, were enriched in IL-10(+) IL-4(-) IFN-γ(+) T cells, a cytokine production profile specific for Tr1 cells, and expressed low levels of FOXP3 and high levels of Granzyme-B. IPEX Tr1 cells were hypoproliferative and suppressive, thus indicating that FOXP3 mutations did not impair their function. Furthermore, we isolated Tr1 cell clones from the peripheral blood of one FOXP3(null) patient, demonstrating that Tr1 cells are present in vivo and they can be expanded in vitro in the absence of WT FOXP3. Overall, our results (i) show that functional Tr1 cells differentiate independently of FOXP3, (ii) confirm that human Tr1 and nTregs are distinct T-cell lineages, and (iii) suggest that under favorable conditions Tr1 cells could exert regulatory functions in IPEX patients.
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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.