Description | Dataset with samples from Individuals with: A- Defects in Intrinsic and Innate Immunity: 1)- Herpes simplex encephalitis (HSE): UNC93B1 deficiency, Phenotype OMIM number 610551 2)- Predisposition to severe viral infection: IRF7 deficiency, Phenotype OMIM number Not yet assigned see PID classification: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4659841/ - AR : Autosomal Recessive |
Purpose | IRF7 plays a critical role in the production and amplification of the antiviral type I and III interferon response. Autosomal recessive IRF7-deficiency resulted in life-threatening influenza disease in a 3-year-old child. We studied the impact of IRF7-deficiency in non-hematopoietic tissues (fibroblasts and lung epithelial cells) as well in hematopoietic cells (peripheral blood mononuclear cells (PBMCs)). Genome-wide gene expression analysis demonstrated a profound loss of type I and III IFNs in PBMCs infected with influenza virus. |
Experimental Design | PBMCs were isolated from 4 healthy donors and patients with deficiencies for IRF7 and UNC93B. The cells were infected with influenza virus A/CA/4/2009 at a multiplicity of infection (MOI) of 2 for 8 and 16 hours. Uninfected cells were cultured for 16 hours. |
Platform | Illumina HumanHT-12 v4 |
(Uploaded through the Files tab in the Annotation Tool)
sampleset4000034_sampleannotations_Group.csv
sampleset4000034_sampleannotations_Group.csv
|