Our results indicate that in contrast to TLR3 stimulation of unprimed skin fibroblasts, TLR3 stimulation of NDAS patient hematopoietic cells with poly(I:C) or by viral infection may lead to enhanced type I IFN production and antiviral responses due to a relatively stabilized NEMO-Δex5-IKKi complex. This evidence concerns the gene IKBKE and viral infectious disease.