ArticleCellular & molecular immunology2021
Dysregulated immunity in PID patients with low GARP expression on Tregs due to mutations in LRRC32.
Article in Cellular & molecular immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 21 citations in OpenAlex.
- Pediatric bronchiolitis disease severity is associated with immune checkpoint dysregulation.Pediatric research · 2026Article
- Altered phenotype and gene expression of regulatory T cells (Tregs) in children with Autism, and the relationship with comorbid gastrointestinal symptoms.Journal of neuroinflammation · 2026Article
- Altered Phenotype and Gene Expression of Regulatory T cells (Tregs) in Children with Autism, and the Relationship with Comorbid Gastrointestinal Symptoms.Research square · 2025Article
- Bidirectional anti-tumor and immunological strategies by targeting GARP-TGF-β axis in adult T-cell leukemia/lymphoma.Leukemia · 2025Article
- Comorbidity Between Inflammatory Bowel Disease and Asthma and Allergic Diseases: A Genetically Informed Study.Inflammatory bowel diseases · 2024Article
- Review
- TrematodeFrontiers in immunology · 2024Article
- Analysis of LAPNeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023Article
- Translating non-coding genetic associations into a better understanding of immune-mediated disease.Disease models & mechanisms · 2023Review
- LncRNAs ENST00000499459 and TCONS_00004989 enhance asthma progression in children with house dust mite-induced allergic asthma.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2022Article
- Deep learning explains the biology of branched glycans from single-cell sequencing data.iScience · 2022Article
- Article
- GARP as a Therapeutic Target for the Modulation of Regulatory T Cells in Cancer and Autoimmunity.Frontiers in immunology · 2022Review
- Novel aspects of regulatory T cell dysfunction as a therapeutic target in giant cell arteritis.Annals of the rheumatic diseases · 2022Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Immune dysregulation diseases are characterized by heterogeneous clinical manifestations and may have severe disease courses. The identification of the genetic causes of these diseases therefore has critical clinical implications. We performed whole-exome sequencing of patients with immune dysregulation disorders and identified two patients with previously undescribed mutations in LRRC32, which encodes glycoprotein A repetitions predominant (GARP). These patients were characterized by markedly reduced numbers and frequencies of regulatory T cells (Tregs). Tregs with mutated LRRC32 exhibited strongly diminished cell-surface GARP expression and reduced suppressor function. In a model of conditional Garp deficiency in mice, we confirmed increased susceptibility to inflammatory diseases once GARP expression on Tregs was decreased. Garp deficiency led to an unstable Treg phenotype due to diminished Foxp3 protein acetylation and stability. Our study reinforces the understanding of the immunological mechanisms of immune dysregulation and expands the knowledge on the immunological function of GARP as an important regulator of Treg stability.
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