Dataset with samples from Individuals with:

A- Combined immunodeficiencies with associated or syndromic features:

1)- HOLI1/RBCK1 deficiency, Phenotype OMIM number 615895

PID classification: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4659841/

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 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-? or IL-1? for 2 and 6 hours. RNA were extracted. Labeled cRNA were hybridized to Illumina Human HT-12 V4 Beadchips.