The dysregulation of the pathways that negatively regulate TLR signaling, including T1/ST2, the single immunoglobulin interleukin-1 receptor (IL-1R)-related molecule (SIGIRR), a splicing variant of the myeloid differentiation factor 88 (MyD88s), a suppressor of cytokine signaling 1 (SOCS1), Triad3A, CYLD, and IRAK-M, were well-described in many inflammatory diseases [21,22,23]; however, little is known about if and how these negative regulatory signals of TLRs contribute to CF inflammation. This evidence concerns the gene IL1R1 and cystic fibrosis.