Umod+/125R and Umod125R/125R mice demonstrate several of the phenotypes of human ADTKD-UMOD patients, including progressive renal failure, urine concentrating defects, decreased uric acid and uromodulin excretion, retention of uromodulin in the ER, and pathological changes in the kidney such as fibrosis, immune cell infiltration and ER stress. The gene discussed is UMOD; the disease is autosomal dominant medullary cystic kidney disease with or without hyperuricemia.