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Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents

ABSTRACT: Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents

PROVIDER: PRJNA702154 | ENA |

REPOSITORIES: ENA

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Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents [EBV B-cells]

Project description:We investigated transcriptional responses following in vitro stimulation of EBV-B cells derived from a patient with inherited c-Rel deficiency.

2021-08-31 | GSE164345 | GEO

Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents [primary T and B cells]

Project description:We investigated transcriptional responses following in vitro stimulation of peripheral blood naive CD4+ T cells and naive B cells obtained from a patient with inherited c-Rel deficiency. Samples from unrelated healthy donors and a patient with CD40 deficiency were analyzed for comparison.

2021-08-31 | GSE166872 | GEO

Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents [EBV B-cells]

Project description:Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents [EBV B-cells]

| PRJNA690078 | ENA

Inherited human c-REL deficiency disrupts adaptive immunity

Project description:Inherited human c-REL deficiency disrupts adaptive immunity

| PRJNA703721 | ENA

Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents [primary T and B cells]

Project description:Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents [primary T and B cells]

| PRJNA702159 | ENA

Inherited human c-REL deficiency disrupts adaptive immunity

Project description:Various inborn errors of the canonical NF-kB pathway underlie different forms of human immunodeficiency. However, no patients with autosomal recessive complete deficiencies of RelA, c-REL, or NF-kB1, the three core members of the canonical NF-kB pathway, have yet been identified. We report a child homozygous for a loss-of-expression mutation of REL, encoding c-REL, which is expressed principally in myeloid and lymphoid cells. The distribution of myeloid subsets is normal in this patient, but in vitro-derived monocytes and cDC1s, unlike cDC2s, have impaired IL-12 and IL-23 production. The patient has low frequencies of memory CD4+ T cells, Th1*, Th2, Tregs, and almost no memory B cells. The frequencies of the other lymphoid subsets are normal. The patient’s naïve and memory CD4+ T cells produce only small amounts of IL-2, and the addition of this interleukin rescues their proliferation in response to mitogens, but not to antigens, in vitro. However, we found that HLA-syngeneic control cDCs rescued the proliferation of these cells in response to recall antigens. The production of key effector cytokines, such as IL-4, IL-17A, and IFNg, by memory CD4+ T cells was also highly impaired ex vivo, as was the differentiation of naïve CD4+ T cells into Th1 and Th17 cells in vitro. Both the survival and proliferation of the patient’s naïve B cells were found to be compromised, preventing B-cell differentiation into immunoglobulin (Ig)-secreting plasmablasts in vitro. As a consequence of this pleiotropic effect, this child suffered from severe viral, mycobacterial, fungal, and parasitic infections, while IgG substitution treatment prevented pyogenic infections. The immunological and infectious phenotypes were of hematopoietic origin, as they were cured by hematopoietic stem cell transplantation. Inherited human c-REL deficiency impairs multiple core functions of DCs, T cells, and B cells, thereby disrupting adaptive immunity to multiple infections.

2021-08-31 | GSE167185 | GEO

Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to a variety of infectious agents

Project description:This SuperSeries is composed of the SubSeries listed below.

2021-08-31 | GSE166873 | GEO

Inherited human c-Rel deficiency disrupts myeloid and lymphoid immunity to multiple infectious agents.

Project description: Not available

| S-EPMC8409595 | biostudies-literature

Transcriptional profiling of NF-kB's Rel and RelA proteins

Project description:Rel and RelA proteins were stably expressed in the chicken DT40 pre-B cell line and analyzed by transcription profiling to identify transformation-impacting genes regulated by NF-kB’s oncogenic v-Rel and c-Rel proteins. This analysis uncovered both common and differential gene expression profiles in cells expressing Rel vs. RelA proteins, and revealed that Rel protein expression can lead to gene-specific transcriptional repression, as seen for key B-cell receptor (BCR) components and signaling molecules like B-cell linker (BLNK), the B-cell adaptor for PI3K (BCAP) and Igλ. These were also downregulated in cells expressing a transformation-competent chimeric RelA/v-Rel protein, suggesting a correlation with the capacity of Rel proteins to transform lymphocytes. DNA binding, ChIP and transformation assays indicate that downregulation of BLNK and BCAP is an important contributing factor to the malignant transformation of lymphocytes by Rel and suggest that gene repression may be as important as transcriptional activation for the transforming activity of Rel proteins. Keywords: Comparative transcriptional profiling

2008-02-14 | GSE9544 | GEO

c-Rel orchestrates energy-dependant epithelial and macrophage reprogramming in fibrosis

Project description:To further understand the role of c-Rel in fibrogensis, label-free mass spectrometric analysis was performed on the secretome of cultured WT and Rel-/- M0, M1 and M2 polarised macrophages as well as control and TGFB1-stimulated hepatocytes.

2020-09-28 | PXD017320 | Pride

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