ArticleClinical and experimental immunology2003
Biased T-cell receptor repertoires in patients with chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome).
Article in Clinical and experimental immunology, 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 65 citations in OpenAlex.
- Follicular Helper T Cells and B Cell Maturation in Patients with 22q11.2 Deletion Syndrome and Recurrent Infections.Journal of clinical immunology · 2026Article
- Primary and secondary defects of the thymus.Immunological reviews · 2024Review
- Clinical Practice Guidelines for the Immunological Management of Chromosome 22q11.2 Deletion Syndrome and Other Defects in Thymic Development.Journal of clinical immunology · 2023Review
- Analyses of thymocyte commitment to regulatory T cell lineage in thymus of healthy subjects and patients with 22q11.2 deletion syndrome.Frontiers in immunology · 2023Article
- COVID-19 Severity, Cardiological Outcome, and Immunogenicity of mRNA Vaccine on Adult Patients With 22q11.2 DS.The journal of allergy and clinical immunology. In practice · 2023Observational
- Accelerated Maturation, Exhaustion, and Senescence of T cells in 22q11.2 Deletion Syndrome.Journal of clinical immunology · 2022Article
- Inborn errors of thymic stromal cell development and function.Seminars in immunopathology · 2021Review
- Relationship Between Severity of T Cell Lymphopenia and Immune Dysregulation in Patients with DiGeorge Syndrome (22q11.2 Deletions and/or Related TBX1 Mutations): a USIDNET Study.Journal of clinical immunology · 2021Article
- B cell development in chromosome 22q11.2 deletion syndrome.Clinical immunology (Orlando, Fla.) · 2016Article
- Eosinophilia Associated with Disorders of Immune Deficiency or Immune Dysregulation.Immunology and allergy clinics of North America · 2015Review
- Neonatal Levels of T-cell Receptor Excision Circles (TREC) in Patients with 22q11.2 Deletion Syndrome and Later Disease Features.Journal of clinical immunology · 2015Article
- Insight into normal thymic activity by assessment of peripheral blood samples.Immunologic research · 2015Article
- The TREC/KREC assay for the diagnosis and monitoring of patients with DiGeorge syndrome.PloS one · 2014Article
- Immunodeficiency in DiGeorge Syndrome and Options for Treating Cases with Complete Athymia.Frontiers in immunology · 2013Review
- DiGeorge Syndrome Presenting as Hypocalcaemia-Induced Seizures in Adulthood.Case reports in medicine · 2013Article
- From murine to human nude/SCID: the thymus, T-cell development and the missing link.Clinical & developmental immunology · 2012Review
- Immunological aspects of 22q11.2 deletion syndrome.Cellular and molecular life sciences : CMLS · 2012Review
- A prospective study of influenza vaccination and a comparison of immunologic parameters in children and adults with chromosome 22q11.2 deletion syndrome (digeorge syndrome/velocardiofacial syndrome).Journal of clinical immunology · 2011Article
- Mycobacterium tuberculosis culture filtrate protein 10-specific effector/memory CD4⁺ and CD8⁺ T cells in tubercular pleural fluid, with biased usage of T cell receptor Vβ chains.Infection and immunity · 2011Article
- Secondary immunologic consequences in chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome).Clinical immunology (Orlando, Fla.) · 2010Article
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Authors and funding
13 authors at 6 institutions in 1 country.
Funding
Abstract
Chromosome 22q11.2 deletion (del22q11.2) syndrome (DiGeorge syndrome/velocardiofacial syndrome) is a common syndrome typically consisting of congenital heart disease, hypoparathyroidism, developmental delay and immunodeficiency. Although a broad range of immunologic defects have been described in these patients, limited information is currently available on the diversity of the T-cell receptor (TCR) variable beta (BV) chain repertoire. The TCRBV repertoires of nine patients with del22q11.2 syndrome were determined by flow cytometry, fragment size analysis of the third complementarity determining region (CDR3 spectratyping) and sequencing of V(D)J regions. The rate of thymic output and the phenotype and function of peripheral T cells were also studied. Expanded TCRBV families were detected by flow cytometry in both CD4+ and CD8+ T cells. A decreased diversity of TCR repertoires was also demonstrated by CDR3 spectratyping, showing altered CDR3 profiles in the majority of TCRBV families investigated. The oligoclonal nature of abnormal peaks detected by CDR3 spectratyping was confirmed by the sequence analysis of the V(D)J regions. Thymic output, evaluated by measuring TCR rearrangement excision circles (TRECs), was significantly decreased in comparison with age-matched controls. Finally, a significant up-regulation in the percentage, but not in the absolute count, of activated CD4+ T cells (CD95+, CCR5+, HLA-DR+), IFN-gamma - and IL-2-expressing T cells was detected. These findings suggest that the diversity of CD4 and CD8 TCRBV repertoires is decreased in patients with del22q11.2 syndrome, possibly as a result of either impaired thymic function and/or increased T-cell activation.
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