The activation of the canonical NF-κB pathway, especially after triggering TCR or BCR, is controlled by the assembly of a protein complex consisting of CARD11, BCL-10, and MALT1, the so-called CBM signalosome.226 Germline mutations in the genes encoding the components of the CBM signalosome result in a broad spectrum of immunological phenotypes, including combined immunodeficiency, atopy, and autoimmunity, which is more clearly linked with Treg dysfunction. This evidence concerns the gene NFKB1 and Immunodeficiency.