期刊
CLINICAL IMMUNOLOGY
卷 256, 期 -, 页码 -出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.clim.2023.109793
关键词
Immunodeficiency; thymus; Exhaustion; IL-7; IFN-gamma; RNA-seq; Spectral cytometry
类别
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. The results showed a strong Th1 bias in T cell populations, accompanied by accelerated maturation of T cells into memory stages. In vitro experiments also revealed that IL-7 further reduced naive T cells and increased cytokine production.
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-gamma, IL-10, IL-2, IL-21 and TNF-alpha. 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 na & iuml;ve T cells in vitro.
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