ArticleBlood2015
Fatal autoimmunity in mice reconstituted with human hematopoietic stem cells encoding defective FOXP3.
Article in Blood, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
What it found
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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.
The trial behind it
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
29 citing papers in PubMed, 40 citations in OpenAlex.
- Article
- The Sunrise of Tertiary Lymphoid Structures in Cancer.Immunological reviews · 2025Review
- T Cell Development and Responses in Human Immune System Mice.Annual review of immunology · 2025Review
- Advances and applications of genome-edited animal models for severe combined immunodeficiency.Zoological research · 2025Review
- The oral-gut microbiome axis in health and disease.Nature reviews. Microbiology · 2024Review
- Cellular and molecular basis of proximal small intestine disorders.Nature reviews. Gastroenterology & hepatology · 2024Review
- Article
- Progress in building clinically relevant patient-derived tumor xenograft models for cancer research.Animal models and experimental medicine · 2023Review
- Humanized mouse models for immuno-oncology research.Nature reviews. Clinical oncology · 2023Review
- Human CD4+CD8α+ Tregs induced by Faecalibacterium prausnitzii protect against intestinal inflammation.JCI insight · 2022Article
- Towards gene therapy for IPEX syndrome.European journal of immunology · 2022Review
- Modeling human T1D-associated autoimmune processes.Molecular metabolism · 2022Review
- Current Status, Barriers, and Future Directions for Humanized Mouse Models to Evaluate Stem Cell-Based Islet Cell Transplant.Advances in experimental medicine and biology · 2022Article
- HLA-Restriction of Human Treg Cells Is Not Required for Therapeutic Efficacy of Low-Dose IL-2 in Humanized Mice.Frontiers in immunology · 2021Article
- Humanized mouse models of genetic immune disorders and hematological malignancies.Biochemical pharmacology · 2020Review
- Human-engineered Treg-like cells suppress FOXP3-deficient T cells but preserve adaptive immune responsesClinical & translational immunology · 2020Article
- The development of human immune system mice and their use to study tolerance and autoimmunity.Journal of translational autoimmunity · 2019Review
- Humanized mouse models reveal an immunologic classification of idiopathic CD4 lymphocytopenia subtypes.JCI insight · 2019Article
- Humanized immune system mouse models: progress, challenges and opportunities.Nature immunology · 2019Article
- Humanized Mouse Models of Rheumatoid Arthritis for Studies on Immunopathogenesis and Preclinical Testing of Cell-Based Therapies.Frontiers in immunology · 2019Review
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Authors and funding
24 authors at 9 institutions in 4 countries.
Funding
Abstract
Mice reconstituted with a human immune system provide a tractable in vivo model to assess human immune cell function. To date, reconstitution of murine strains with human hematopoietic stem cells (HSCs) from patients with monogenic immune disorders have not been reported. One obstacle precluding the development of immune-disease specific "humanized" mice is that optimal adaptive immune responses in current strains have required implantation of autologous human thymic tissue. To address this issue, we developed a mouse strain that lacks murine major histocompatibility complex class II (MHC II) and instead expresses human leukocyte antigen DR1 (HLA-DR1). These mice displayed improved adaptive immune responses when reconstituted with human HSCs including enhanced T-cell reconstitution, delayed-type hypersensitivity responses, and class-switch recombination. Following immune reconstitution of this novel strain with HSCs from a patient with immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, associated with aberrant FOXP3 function, mice developed a lethal inflammatory disorder with multiorgan involvement and autoantibody production mimicking the pathology seen in affected humans. This humanized mouse model permits in vivo evaluation of immune responses associated with genetically altered HSCs, including primary immunodeficiencies, and should facilitate the study of human immune pathobiology and the development of targeted therapeutics.
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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.