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Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

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Transcriptome analysis in primary fibroblasts from HOIL-1-deficient patients upon TNF-α or IL-1β stimulation

ABSTRACT: HOIL-1 deficient disease is a new early onset fatal autosomal recessive human disorder charaterized by chronic auto-inflammation, recurrent invasive bacterial infections and progressive muscular amylopectinosis. We studied the effect of TNF-α and IL-1β on transcriptional changes of primary fibroblasts from HOIL-1-, MYD88- and NEMO-deficient patients. Primary fibroblasts were obtained from HOIL-1, MYD88- and NEMO-deficient patients and healthy donors and stimulated with TNF-α or IL-1β for 2 and 6 hours. RNA were extracted and globin reduced. Labeled cRNA were hybridized to Illumina Human HT-12 Beadchips.

ORGANISM(S): Homo sapiens

SUBMITTER: Zhaohui Xu

PROVIDER: E-GEOD-40560 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Transcriptome analysis in primary fibroblasts from HOIL-1-deficient patients upon TNF-α or IL-1β stimulation

2012-10-28 | GSE40560 | GEO

Transcriptome analysis in peripheral blood mononuclear cells (PBMC) from HOIL-1-deficient patients upon TNF-α or IL-1β stimulation

Project description:HOIL-1 deficient disease is a new early onset fatal autosomal recessive human disorder charaterized by chronic auto-inflammation, recurrent invasive bacterial infections and progressive muscular amylopectinosis. We studied the effect of TNF-α and IL-1β on transcriptional changes of PBMCs from HOIL-1- and MYD88-deficient patients. PBMCs were obtained from HOIL-1 and MYD88-deficient patients and healthy donors and stimulated with TNF-α or IL-1β for 2 and 6 hours. RNA were extracted. Labeled cRNA were hybridized to Illumina Human HT-12 V4 Beadchips.

2012-10-28 | E-GEOD-40838 | biostudies-arrayexpress

Transcriptome analysis in peripheral blood mononuclear cells (PBMC) from HOIL-1-deficient patients upon TNF-α or IL-1β stimulation

2012-10-28 | GSE40838 | GEO

Transcriptional analysis of whole blood, primary fibroblasts, and PBMCs upon TNF-alpha or IL-1beta stimulation from HOIL-1-deficient patients

Project description:This SuperSeries is composed of the following subset Series: GSE40560: Transcriptome analysis in primary fibroblasts from HOIL-1-deficient patients upon TNF-alpha or IL-1beta stimulation GSE40561: Transcriptional analysis of whole blood in patients with auto-inflammatory disorders GSE40838: Transcriptome analysis in peripheral blood mononuclear cells (PBMC) from HOIL-1-deficient patients upon TNF-alpha or IL-1beta stimulation Refer to individual Series

2012-10-28 | E-GEOD-40752 | biostudies-arrayexpress

In vitro response of fibroblasts isolated from patients with immunodeficiencies

Project description:Children with autosomal recessive deficiency suffer from life-threatening, often recurrent viral or bacterial infections. We have profiled transcriptional responses of fibroblast cell lines derived from patients deficient for key regulators of immune responses such as MYD88, IRAK4, UNC93B, STAT1 or NEMO. This systems immunology approach revealed distinct patterns or responsiveness to stimuli activating the Toll-like/IL1 Receptor pathway in either an MYD88-dependent (IL1B) or independent (poly(I:C)) fashion, or activating the TNF pathway. The prototypic signatures in cell lines derived from patients were as follows: NEMO-deficient cells were unresponsive to all three stimuli, UNC-93B-deficient cells did not respond to poly(I:C), and STAT1-deficient cells responded only weakly to poly(I:C) after eight hours (not shown). MyD88- and IRAK-4-deficient cells had indistinguishable phenotypes, both being unresponsive to IL-1B at both time points. The signatures obtained in response to TNF and poly(I:C) (via TLR3) in these cells were similar to that of control fibroblasts.

2009-07-15 | GSE12124 | GEO

Homo sapiens

Project description:Transcriptome analysis in primary fibroblasts from HOIL-1-deficient patients upon TNF-α or IL-1β stimulation

| PRJNA174575 | ENA

Osteoarthritic chondrocytes undergo a glycolysis-related metabolic switch upon exposure to IL-1β or TNF-α

Project description:Inflammation, which is mainly sustained by pro-inflammatory cytokines such as IL-1β, TNF-α, and IL-6, plays an important role in osteoarthritis progression. However, the therapeutic failures of recent clinical trials evaluating anti-IL-1, anti-TNF, and anti-IL-6 drugs highlight the lack of overall understanding of the effects of these cytokines on chondrocytes. Here, we generated a comprehensive transcriptomic and proteomic dataset of osteoarthritic chondrocytes treated with these cytokines to describe their pro-inflammatory signature and compare it to the transcriptome of non-osteoarthritic chondrocytes. We first identified specific dysregulation of metabolic-related genes in OA chondrocytes. A metabolic shift, toward increased glycolysis at the expense of mitochondrial respiration, was specifically identified and confirmed by Seahorse® assay of osteoarthritic chondrocytes treated with IL-1β or TNF-α. These data show a strong association between inflammation and metabolism, indicating that understanding metabolic dysregulations should be a focus of future investigations for the design of therapies for osteoarthritis.

2023-05-20 | GSE215039 | GEO

Inherited human IRAK-1 deficiency selectively abolishes TLR signaling in fibroblasts

Project description:We describe here a male infant with a 100 kb de novo Xq28 deletion encompassing parts of the TMEM187 and MECP2 protein-coding genes and the IRAK1 protein-coding gene, as well as the MIR3202-1, MIR3202-2, and MIR718 RNA-coding genes. We analyzed the impact of human IRAK-1 deficiency on a genome-wide gene expression in human fibroblasts in response to TLR2/6, TLR4 agonists as well as to IL-1β and TNF-α, using primary fibroblasts from healthy controls and IRAK-4-, MyD88- and MECP2-deficient patients for comparison.

2017-01-29 | GSE92466 | GEO

Homo sapiens

Project description:Transcriptome analysis in peripheral blood mononuclear cells (PBMC) from HOIL-1-deficient patients upon TNF-α or IL-1β stimulation

| PRJNA175062 | ENA

Transcriptional analysis of whole blood in patients with auto-inflammatory disorders

Project description:HOIL1 deficient disease is a new early onset fatal autosomal recessive human disorder charaterized by chronic auto-inflammation, recurrent invasive bacterial infections and progressive muscular amylopectinosis. We studied the transcriptional profiles of whole blood from one HOIL dificient patient and other auto-inflammatory patients, including CINCA, Muckle-Wells syndrome and MVK deficiency. Whole blood was collected in Tempus tubes from 41 healthy children and patients with CINCA (2), MWS (5), MVK deficiency (2) and HOIL deficiency (1) diseases. RNA was extracted and globin reduced. Labeled cRNA was hybridized to Illumina Human HT-12 Beadchips.

2012-10-28 | E-GEOD-40561 | biostudies-arrayexpress

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