In addition, the germline mutations in HOIP (RNF31), encoding one component of the linear ubiquitin chain assembly complex (LUBAC), which mediated linear poly-ubiquitination is essential for activation of canonical NF-κB signaling pathway, are observed in 7.8% of patients with activated B cell-like (ABC) subtype of diffuse large B-cell lymphoma (DLBCL).105 These two alterations, p.Q584H and p.Q622L, enhance the HOIP-HOIL-1 association, facilitating NF-κB activity in ABC DLBCL. Here, NFKB1 is linked to diffuse large B-cell lymphoma.