These differences between the germline Umod125R, transgenic TgUmodC147W and ENU A227T and C93F mice with different mutations highlight the advantages of having multiple mouse models that provide the opportunity of elucidating further the relationships between protein, uromodulin mutation, genetic modifiers, phenotype and mechanism in ADTKD-UMOD. This evidence concerns the gene UMOD and autosomal dominant medullary cystic kidney disease with or without hyperuricemia.