ArticleImmunology and cell biology2017
Murine LRBA deficiency causes CTLA-4 deficiency in Tregs without progression to immune dysregulation.
Article in Immunology and cell biology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 35 citations in OpenAlex.
- Adult-Onset LRBA Deficiency Presenting with Rheumatoid Arthritis-Like Manifestations: A Case Report.Journal of clinical immunology · 2026Article
- LRBA regulates actin cytoskeleton dynamics through NMIIA during B cell immune responses.EMBO reports · 2026Article
- Case Report: Different faces of LRBA deficiency in five Moroccan families.Frontiers in immunology · 2026Article
- Proteostasis of immune checkpoint receptors.The Biochemical journal · 2025Review
- ZFP36-family RNA-binding proteins in regulatory T cells reinforce immune homeostasis.Nature communications · 2025Article
- Treg Control of CD80/CD86 Expression Mediates Immune System Homeostasis.European journal of immunology · 2025Article
- Tregopathy in focus.Frontiers in immunology · 2025Article
- Single-Cell Analysis Uncovers Striking Cellular Heterogeneity of Lung-Infiltrating Regulatory T Cells during Eosinophilic versus Neutrophilic Allergic Airway Inflammation.Journal of immunology (Baltimore, Md. : 1950) · 2024Article
- LRBA, a BEACH protein mutated in human immune deficiency, is widely expressed in epithelia, exocrine and endocrine glands, and neurons.Scientific reports · 2024Article
- Lrba participates in the differentiation of IgA+ B lymphocytes through TGFβR signaling.Frontiers in immunology · 2024Article
- Genetic characteristics of common variable immunodeficiency patients with autoimmunity.Frontiers in genetics · 2023Article
- LRBA is essential for urinary concentration and body water homeostasis.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
- LRBA Deficiency Can Lead to Lethal Colitis That Is Diminished by SHIP1 Agonism.Frontiers in immunology · 2022Article
- Regulation of CTLA-4 recycling by LRBA and Rab11.Immunology · 2021Article
- Immune checkpoint deficiencies and autoimmune lymphoproliferative syndromes.Biomedical journal · 2021Review
- Inborn Errors of Immunity and Their Phenocopies: CTLA4 and PD-1.Frontiers in immunology · 2021Review
- Case Report: Infantile-Onset Fulminant Type 1 Diabetes Mellitus Caused by Novel Compound HeterozygousFrontiers in immunology · 2021Article
- Conformational diversity facilitates antibody mutation trajectories and discrimination between foreign and self-antigens.Proceedings of the National Academy of Sciences of the United States of America · 2020Article
- Clinical Phenotypes and Immunological Characteristics of 18 Egyptian LRBA Deficiency Patients.Journal of clinical immunology · 2020Article
- The Clinical Immunogenomics Research Consortium Australasia (CIRCA): a Distributed Network Model for Genomic Healthcare Delivery.Journal of clinical immunology · 2020Article
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
Abstract
Inherited mutations in lipopolysaccharide-responsive beige-like anchor (LRBA) cause a recessive human immune dysregulation syndrome with memory B-cell and antibody deficiency (common variable immunodeficiency), inflammatory bowel disease, enlarged spleen and lymph nodes, accumulation of activated T cells and multiple autoimmune diseases. To understand the pathogenesis of the syndrome, C57BL/6 mice carrying a homozygous truncating mutation in Lrba were produced using CRISPR/Cas9-mediated gene targeting. These mice revealed that LRBA has a critical, cell-autonomous role in promoting cytotoxic T-lymphocyte antigen-4 (CTLA-4) accumulation within CD4 effector T cells and FOXP3
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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.