ArticleProceedings of the National Academy of Sciences of the United States of America1998
Defect in IgV gene somatic hypermutation in common variable immuno-deficiency syndrome.
Article in Proceedings of the National Academy of Sciences of the United States of America, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 44 papers.
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Who cites it
44 citing papers in PubMed.
- Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses.The Journal of allergy and clinical immunology · 2019Trial
- Dual variants of uncertain significance in a case of hyper-IgM syndrome: implications for diagnosis and management.Frontiers in immunology · 2025Article
- Genomic crossroads between non-Hodgkin's lymphoma and common variable immunodeficiency.Frontiers in immunology · 2022Article
- Members of the Regulatory Lymphocyte Club in Common Variable Immunodeficiency.Frontiers in immunology · 2022Review
- Serum Free Immunoglobulins Light Chains: A Common Feature of Common Variable Immunodeficiency?Frontiers in immunology · 2020Article
- Selection and Neutral Mutations Drive Pervasive Mutability Losses in Long-Lived Anti-HIV B-Cell Lineages.Molecular biology and evolution · 2018Article
- B-cell receptor repertoire sequencing in patients with primary immunodeficiency: a review.Immunology · 2018Review
- Identification of CVID Patients With Defects in Immune Repertoire Formation or Specification.Frontiers in immunology · 2018Article
- Somatic Hypermutation Defects in Common Variable Immune Deficiency.Current allergy and asthma reports · 2017Review
- IgH sequences in common variable immune deficiency reveal altered B cell development and selection.Science translational medicine · 2015Article
- Levels of somatic hypermutations in B cell receptors increase during childhood.Clinical and experimental immunology · 2014Article
- Exhaustion of bacteria-specific CD4 T cells and microbial translocation in common variable immunodeficiency disorders.The Journal of experimental medicine · 2014Article
- Expression of activation-induced cytidine deaminase gene in B lymphocytes of patients with common variable immunodeficiency.Iranian journal of pediatrics · 2013Article
- Long-term follow-up on affinity maturation and memory B-cell generation in patients with common variable immunodeficiency.Journal of clinical immunology · 2013Article
- TLR-mediated B cell defects and IFN-α in common variable immunodeficiency.Journal of clinical immunology · 2012Article
- Gene Conversion-Like Events in the Diversification of Human Rearranged IGHV3-23*01 Gene Sequences.Frontiers in immunology · 2012Article
- Altered spectrum of somatic hypermutation in common variable immunodeficiency disease characteristic of defective repair of mutations.Immunogenetics · 2011Article
- Pathogenesis and treatment of gastrointestinal disease in antibody deficiency syndromes.The Journal of allergy and clinical immunology · 2009Review
- Role of B cells in common variable immune deficiency.Expert review of clinical immunology · 2009Article
- B-cell activating factor receptor deficiency is associated with an adult-onset antibody deficiency syndrome in humans.Proceedings of the National Academy of Sciences of the United States of America · 2009Article
Corrections and comments
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Authors and funding
7 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Common Variable Immuno-Deficiency (CVID) is the most common symptomatic primary antibody-deficiency syndrome, but the basic immunologic defects underlying this syndrome are not well defined. We report here that among eight patients studied (six CVID and two hypogammaglobulinemic patients with recurrent infections), there is in two CVID patients a dramatic reduction in Ig V gene somatic hypermutation with 40-75% of IgG transcripts totally devoid of mutations in the circulating memory B cell compartment. Functional assays of the T cell compartment point to an intrinsic B cell defect in the process of antibody affinity maturation in these two cases.
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