UMOD and autosomal dominant medullary cystic kidney disease with or without hyperuricemia: Thus, mutant uromodulin protein must be present to cause the renal pathology associated with ADTKD-UMOD, and we have shown that histopathological changes characteristic of human ADTKD-UMOD patients, such as interstitial fibrosis and immune cell infiltration, are present in our Umod+/125R and Umod125R/125R knock-in mice.