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Gene signature of regulatory T cells isolated from children with Selective IgA Deficiency and Common Variable Immunodeficiency.

ABSTRACT: Gene signature of regulatory T cells isolated from children with Selective IgA Deficiency and Common Variable Immunodeficiency.

PROVIDER: PRJNA1086511 | ENA |

REPOSITORIES: ENA

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Gene signature of regulatory T cells isolated from children with Selective IgA Deficiency and Common Variable Immunodeficiency.

Project description:In our study we aimed to analyze the gene expression profile of Treg cells in CVID and SIgAD patients, compared to age-matched healthy controls. Selective IgA deficiency (SIgAD) is the most common and common variable immunodeficiency (CVID) is the most symptomatic form of predominant antibody deficiency. Despite differences in the clinical picture, a similar genetic background is suggested. A common feature of both disorders is the occurrence of autoimmune conditions. Regulatory T cells (Tregs) are the major immune cell type that maintains autoimmune tolerance. As the different types of abnormalities of Treg cells were associated with autoimmune disorders in primary immunodeficiency (PID) patients, in our study we aimed to analyze the gene expression profile of Treg cells in CVID and SIgAD patients, compared to age-matched healthy controls. The transcriptome-wide gene profiling was performed using microarray technology. As a results, we analyzed and visualized global expression patterns of genes and pathways of isolated population of Treg cells. We showed the differences at gene-level between patients with and without autoimmunizations. Our finding suggest that gene signature of Treg cells isolated from SIgAD and CVID patients differ from age-matched healthy controls and form each other, while the occurrence of autoimmunity in both PID is associated with IFN signaling pathway. In sum, our findings improve our understanding of Treg dysfunctions in patients with common PIDs and associated autoimmunity.

2025-02-23 | GSE261335 | GEO

Duodenal inflammation in Common variable immunodeficiency has altered transcriptional response to viruses

Project description:To elucidate the role of duodenal microenvironment in Common variable immunodeficiency pathogenesis (CVID), we sought to explore the transcriptome regulation in duodenal biopsies.

2022-11-03 | GSE207243 | GEO

Duodenal inflammation in common variable immunodeficiency has altered transcriptional response to viruses

Project description:A substantial proportion of common variable immunodeficiency (CVID) patients has duodenal inflammation of largely unknown etiology. However, because of its histologic similarities with celiac disease, gluten sensitivity has been proposed as a potential mechanism. We aimed to elucidate the role of the duodenal microenvironment in the pathogenesis of duodenal inflammation in CVID by investigating the transcriptional, proteomic, and microbial signatures of duodenal biopsy samples in CVID.

2025-04-29 | PXD063461 | Pride

Whole-exome sequencing of Common Variable Immunodeficiency

Project description:An investigation of rare genetic variants in patients presenting with Common Variable Immunodeficiency

2022-04-11 | PRJEB52220 | EVA

Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable Immunodeficiency

Project description: Not available

| S-EPMC10930802 | biostudies-literature

Gene Expression Profiles of CD21low B cells in Common Variable Immunodeficiency

2009-07-30 | GSE17269 | GEO

Transcriptomic analysis of follicular CD8 T cells in human secondary lymphoid organs in healthy donors and patients with common variable immunodeficiency

Project description:We performed a comparison of transcriptome between follicular CD8 T cells (CXCR5+CD8+CD3+) from 3 lymph nodes of patients with common variable immunodeficiency (CVID) and 3 tonsils of healthy donors, discovering 67 differentially expressed genes that show immunoregulatory potential of CVID follicular CD8 T cells.

2019-09-27 | GSE138076 | GEO

Gene Expression Profiles of CD21low B cells in Common Variable Immunodeficiency

Project description:The homeostasis of circulating B cell subsets in the peripheral blood of healthy adults is well regulated, but in disease it can be severely disturbed. Thus, a subgroup of patients with common variable immunodeficiency (CVID) presents with an extraordinary expansion of an unusual B cell population characterized by the low expression of CD21. Since these circulating atypical B cells in the blood of CVID patients could not be assigned to any certain B cell differentiation stage in the periphery, they were designated as CD21low B cells. Although, CD21low B cells are polyclonal and unmutated IgM+IgD+ B cells like naive B cells in the peripheral blood, they reveal several distinct phenotypic and functional features. In order to uncover the the global programme of gene expression underlying these differences and changes in CD21low B cells we have performed microarray hybridization experiments and identified differentially expressed genes which define a distinct gene expression profile in CD21low B cells compared to naive B cells. Leucocytes were separated by Ficoll gradient from peripheral blood of healthy donors (HD) and CVID patients. CD19+CD27-CD38+CD21+ naive B cells of HD as well as naive and CD19hiCD27-CD38lowCD21low (CD21low) B cells of CVID patients were sorted using a MoFlow cell sorter. RNA was extracted from sorted ex vivo B cell subpopulations and hybridized on Affymetrix microarrays. Gene expression profiles were compared between CD21low B cells and naive B cells of CVID patients as well as HD naive B cells.

2009-07-30 | E-GEOD-17269 | biostudies-arrayexpress

Reexamining the role of TACI coding variants in common variable immunodeficiency and selective IgA deficiency.

Project description: Not available

| S-EPMC2931279 | biostudies-literature

IgM from common variable immunodeficiency patients narrowly recognizes erythrocyte and platelet glycans

Project description:Autoantibody-mediated cytopenias (AICs) regularly occur in profoundly IgG-deficient common variable immunodeficiency (CVID) patients. The isotypes, antigenic targets, and origin(s) of disease-causing autoantibodies in this patient population are unclear. Herein, we report that erythrocytes and platelets from CVID patients with AICs (CVID+AIC) are coated with autoreactive IgM. Glycan array-based analyses of CVID+AIC plasma IgM revealed narrow reactivities to erythrocytic I/i antigens and platelet expressed I/i antigen-related glycans but starkly lower binding to hundreds of other pathogen and tumor-associated carbohydrates. Polyclonal B-cell receptors with corresponding I/i antigen reactivities were highly enriched among CVID+AIC circulating marginal zone (MZ) B cells. Within secondary lymphoid tissues, MZ B cells secreted copious IgM when activated by IL-10 secreting FOXP3-CD25hiTfh cells. In lymph nodes from immunocompetent controls, MZ B cells localized outside of GCs near rare FOXP3-CD25+ cells and plentiful FOXP3+ regulatory T cells. In CVID+AIC lymph nodes, counterpart cells localized to similar anatomic positions but CD25hiTfh cells greatly outnumbered regulatory T cells. In total, our findings indicate glycan-reactive IgM autoantibodies produced outside of GC borders may contribute to the autoimmune pathogenesis of CVID.

2023-06-09 | GSE233753 | GEO

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