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Accession IconGSE40560

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

Organism Icon Homo sapiens
Sample Icon 9 Downloadable Samples
Technology Badge IconIllumina HumanHT-12 V4.0 expression beadchip, Illumina HumanHT-12 V3.0 expression beadchip

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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- and IL-1 on transcriptional changes of primary fibroblasts from HOIL-1-, MYD88- and NEMO-deficient patients.
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