Likewise, a number of molecular mechanisms have been implicated in the pathogenesis of TRAPS, including abnormal TNF-receptor 1 (TNFR1) receptor cleavage (3), ligand-independent activation of mutant TNFR1 (4–6), activation of nuclear factor kappa B (NF-κB)/mitogen-activated protein kinase (6–10), generation of mitochondrial reactive oxygen species (7, 11), and TNFR1 misfolding and retention within the endoplasmic reticulum (ER), leading to activation of the unfolded protein response (UPR) (7). The gene discussed is TNFRSF1A; the disease is TNF receptor 1-associated periodic fever syndrome.