IL-1 signaling may contribute to pathophysiology of the autosomal dominant polycystic kidney disease (ADPKD) as suggested by enhanced IL-1α and IL-1β expression in kidneys specimens from ADPKD patients, presence of IL-1β in the cystic fluid, decreased urinary IL-1Ra excretion in ADPKD patients, kidney disease-related polymorphisms within the IL-1 gene cluster, as well as protective effects of genetic IL-1R1 deletion in an ADPKD mouse model (99, 100, 126–128). Here, IL1A is linked to autosomal dominant polycystic kidney disease.