ArticleClinical and experimental immunology2003
Rescue of the autoimmune scurfy mouse by partial bone marrow transplantation or by injection with T-enriched splenocytes.
Article in Clinical and experimental immunology, 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
25 citing papers in PubMed, 58 citations in OpenAlex.
- Heat shock factor 1 drives regulatory T-cell induction to limit murine intestinal inflammation.Mucosal immunology · 2024Article
- Improving the Efficacy of Regulatory T Cell Therapy.Clinical reviews in allergy & immunology · 2022Review
- Regulatory T cells in acute and chronic kidney diseases.American journal of physiology. Renal physiology · 2018Review
- Regulatory T Cell and Forkhead Box Protein 3 as Modulators of Immune Homeostasis.Frontiers in immunology · 2017Review
- Forkhead-Box-P3 Gene Transfer in Human CD4Frontiers in immunology · 2017Review
- Are Regulatory T Cells Defective in Type 1 Diabetes and Can We Fix Them?Journal of immunology (Baltimore, Md. : 1950) · 2016Review
- Article
- Gene/cell therapy approaches for Immune Dysregulation Polyendocrinopathy Enteropathy X-linked syndrome.Current gene therapy · 2014Review
- A rheostat for immune responses: the unique properties of PD-1 and their advantages for clinical application.Nature immunology · 2013Review
- B cells are critical for autoimmune pathology in Scurfy mice.Proceedings of the National Academy of Sciences of the United States of America · 2013Article
- Severe Developmental B Lymphopoietic Defects in Foxp3-Deficient Mice are Refractory to Adoptive Regulatory T Cell Therapy.Frontiers in immunology · 2012Article
- Genetic control of the inflammatory T-cell response in regulatory T-cell deficient scurfy mice.Clinical immunology (Orlando, Fla.) · 2010Review
- T cell epitope: friend or foe? Immunogenicity of biologics in context.Advanced drug delivery reviews · 2009Review
- Deficiency in regulatory T cells results in development of antimitochondrial antibodies and autoimmune cholangitis.Hepatology (Baltimore, Md.) · 2009Article
- FoxP3: a life beyond regulatory T cells.International journal of clinical and experimental pathology · 2009Article
- Regulatory T cells and their role in rheumatic diseases: a potential target for novel therapeutic development.Pediatric rheumatology online journal · 2008Article
- Large functional repertoire of regulatory T-cell suppressible autoimmune T cells in scurfy mice.Journal of autoimmunity · 2007Article
- Lack of Foxp3 function and expression in the thymic epithelium.The Journal of experimental medicine · 2007Article
- Submyeloablative cord blood transplantation corrects clinical defects seen in IPEX syndrome.Bone marrow transplantation · 2007Article
- Splice variants of human FOXP3 are functional inhibitors of human CD4+ T-cell activation.Immunology · 2006Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
The scurfy mutant mouse is the genetic and phenotypic equivalent of the single-gene human autoimmune disease immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX). The scurfy mutation disrupts the Foxp3 gene, a putative master switch for T regulatory cell development. Bone marrow transplant without conditioning was previously reported to be ineffective in scurfy mice, yet clinical remission occurs in transplanted human IPEX patients despite limited donor engraftment. In view of this contradiction, we sought to validate scurfy as a model for studying the pathogenesis and treatment of human IPEX, in particular the phenomenon of dominant immune regulation. One half of scurfy mice given bone marrow transplants after sublethal irradiation recovered and survived long-term with donor chimerism ranging from 1.7% to 50%. Early transfer of 2 x 107 normal T cell-enriched splenocytes also prevented or limited disease and permitted long-term survival. Donor T cells in rescued mice made up 3-5% of lymphocytes and became highly enriched for CD25+ T cells over time. Transfer of 106 CD4+ CD25+ sorted T cells showed some beneficial effect, while CD4+ CD25- cells did not. Thus, both partial bone marrow transplant and T-enriched splenocyte transfer are effective treatments for scurfy. These results indicate that scurfy results from a lack of cells with dominant immune regulatory capacity, possibly T regulatory cells. The potency of small numbers of normal cells indicates that IPEX may be a feasible target for gene therapy.
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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.