Th1/interferon-γ bias in 22q11.2 deletion syndrome is driven by memory T cells and exacerbated by IL-7
Language English Country United States Media print-electronic
Document type Journal Article, Research Support, Non-U.S. Gov't
PubMed
37776967
DOI
10.1016/j.clim.2023.109793
PII: S1521-6616(23)00556-9
Knihovny.cz E-resources
- Keywords
- Exhaustion, IFN-γ, IL-7, Immunodeficiency, RNA-seq, Spectral cytometry, thymus,
- MeSH
- Cytokines MeSH
- DiGeorge Syndrome * MeSH
- Child MeSH
- Interferon-gamma * MeSH
- Interleukin-7 MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Memory T Cells MeSH
- Th1 Cells MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- Cytokines MeSH
- Interferon-gamma * MeSH
- Interleukin-7 MeSH
The aim of this study was to investigate the impact of thymic dysplasia on the phenotypic and functional characteristics of T cells in patients with 22q11.2 deletion syndrome, including T-cell phenotype, transcriptional profile, cytokine production, as well as the possibility of utilizing IL-7 to recover their numbers and function. We found a strong bias towards Th1 response in pediatric and young adult 22q11.2DS patients, expansion of CXCR5+ follicular helper cells and CXCR3+CCR6- Th1 cells, increased production of cytokines IFN-γ, IL-10, IL-2, IL-21 and TNF-α. This Th1 skew was primarily driven by expanded terminally differentiated T cells. IL-7 further reduced naive T cells, increased cytokine production and caused an upregulation of exhaustion markers. Thus, Th1 bias in T cell populations persists from infancy into adolescence and is accompanied by accelerated maturation of T cells into memory stages. This phenotype is exacerbated by IL-7 which causes further decrease in naïve T cells in vitro.
References provided by Crossref.org
Profound T Lymphocyte and DNA Repair Defect Characterizes Schimke Immuno-Osseous Dysplasia
