Additionally, patients with mutations in genes involved in signal transduction downstream of TLRs, autosomal recessive (AR) interleukin-1 receptor-associated kinase 4 (IRAK4) [23], and AR NF-kappa-B essential modulator (NEMO) deficiencies [24], usually linked to invasive pneumococcal infections, have been described as suffering from IMD. The gene discussed is IKBKG; the disease is pneumococcal infection.