The fact that the spectrum of CAKUT phenotypes associated with HNF1β or uromodulin (UMOD) mutations and age of their manifestations differ [33, 55, 85, 86] may be due, in part, to the ability of HNF1β, a developmentally regulated transcription factor, to regulate expression of UMOD or aggravate the phenotype of ADPKD [87, 88]. This evidence concerns the gene UMOD and autosomal dominant polycystic kidney disease.