Partial RAG deficiency in humans induces dysregulated peripheral lymphocyte development and humoral tolerance defect with accumulation of T-bet+ B cells View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2022-07-28

AUTHORS

Krisztian Csomos, Boglarka Ujhazi, Peter Blazso, Jose L. Herrera, Christopher M. Tipton, Tomoki Kawai, Sumai Gordon, Maryssa Ellison, Kevin Wu, Matthew Stowell, Lauren Haynes, Rachel Cruz, Bence Zakota, Johnny Nguyen, Michelle Altrich, Christoph B. Geier, Svetlana Sharapova, Joseph F. Dasso, Jennifer W. Leiding, Grace Smith, Waleed Al-Herz, Mayra de Barros Dorna, Olajumoke Fadugba, Eva Fronkova, Veronika Kanderova, Michael Svaton, Sarah E. Henrickson, Joseph D. Hernandez, Taco Kuijpers, Snezhina Mihailova Kandilarova, Elizaveta Naumova, Tomas Milota, Anna Sediva, Despina Moshous, Benedicte Neven, Tara Saco, Ravishankar Sargur, Sinisa Savic, John Sleasman, Gauri Sunkersett, Brant R. Ward, Masanobu Komatsu, Stefania Pittaluga, Attila Kumanovics, Manish J. Butte, Michael P. Cancro, Shiv Pillai, Eric Meffre, Luigi D. Notarangelo, Jolan E. Walter

ABSTRACT

The recombination-activating genes (RAG) 1 and 2 are indispensable for diversifying the primary B cell receptor repertoire and pruning self-reactive clones via receptor editing in the bone marrow; however, the impact of RAG1/RAG2 on peripheral tolerance is unknown. Partial RAG deficiency (pRD) manifesting with late-onset immune dysregulation represents an ‘experiment of nature’ to explore this conundrum. By studying B cell development and subset-specific repertoires in pRD, we demonstrate that reduced RAG activity impinges on peripheral tolerance through the generation of a restricted primary B cell repertoire, persistent antigenic stimulation and an inflammatory milieu with elevated B cell-activating factor. This unique environment gradually provokes profound B cell dysregulation with widespread activation, remarkable extrafollicular maturation and persistence, expansion and somatic diversification of self-reactive clones. Through the model of pRD, we reveal a RAG-dependent ‘domino effect’ that impacts stringency of tolerance and B cell fate in the periphery. More... »

PAGES

1256-1272

Journal

TITLE

Nature Immunology

ISSUE

8

VOLUME

23

Author Affiliations

  • Division of Pediatric Allergy/Immunology, University of South Florida at Johns Hopkins All Children’s Hospital, St. Petersburg, FL, USA
  • Department of Pediatrics, University of Szeged, Szeged, Hungary
  • Department of Orthopaedic Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA
  • Department of Medicine, Division of Rheumatology, Emory University, Atlanta, GA, USA
  • Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD, USA
  • Department of Pathology & Laboratory Medicine, Johns Hopkins All Children’s Hospital, St Petersburg, FL, USA
  • Eurofins Viracor Laboratories, Lee Summit’s, MO, USA
  • Immunology Outpatient Clinic, Vienna, Austria
  • Belarusian Research Center for Pediatric Oncology, Minsk, Belarus
  • Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
  • Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait
  • Department of Pediatrics, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brasil
  • Division of Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, Pennsylvania, PA, USA
  • Childhood Leukemia Investigation Prague, Department of Pediatric Hematology and Oncology, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic
  • Institute for Immunology, the University of Pennsylvania, Philadelphia, PA, USA
  • Department of Pediatrics, Division of Allergy, Immunology and Rheumatology, Stanford University, Stanford, CA, USA
  • Deptartment of Pediatric Immunology, Rheumatology and Infectious Diseases, Emma Children’s Hospital, Academic Medical Center, Amsterdam, Netherlands
  • Department of Clinical Immunology, University Hospital Alexandrovska, Medical University, Sofia, Bulgaria
  • Department of Immunology, Second Faculty of Medicine Charles University and University Hospital Motol, Prague, Czech Republic
  • Laboratory of Genome Dynamics in the Immune System, INSERM UMR1163, Institut Imagine, Paris, France
  • Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR1163, Institut Imagine, Paris, France
  • Windom Allergy, Asthma and Sinus, Sarasota, FL, USA
  • Department of Immunology and Allergy, Sheffield Teaching Hospitals, Sheffield, UK
  • National Institute for Health Research–Leeds Musculoskeletal Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, St James’s University Hospital, Leeds, UK
  • Division of Allergy, Immunology and Pulmonary Medicine, Duke University School of Medicine, Durham, NC, USA
  • Cancer and Blood Disorder Institute, Johns Hopkins All Children’s Hospital, St. Petersburg, FL, USA
  • Division of Allergy and Immunology, Children’s Hospital of Richmond, Virginia Commonwealth University, Richmond, VA, USA
  • Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
  • Division of Immunology, Allergy, and Rheumatology, Department of Pediatrics and Jeffrey Modell Diagnostic and Research Center, University of California, Los Angeles, Los Angeles, CA, USA
  • Department of Pathology, Perelman School of Medicine, University of Pennsylvania, Pennsylvania, PA, USA
  • Ragon Institute of Massachusetts General Hospital, Massachusetts Institute of technology and Harvard University, Cambridge, MA, USA
  • Section of Rheumatology, Allergy and Clinical Immunology, Yale School of Medicine, New Haven, CT, USA
  • Division of Allergy and Immunology, Massachusetts General Hospital for Children, Boston, MA, USA
  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1038/s41590-022-01271-6

    DOI

    http://dx.doi.org/10.1038/s41590-022-01271-6

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1149815773

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/35902638


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