Then, Ricci et al., have described a similar up-regulation in a group of pediatric patients suffering from ADTKD (Autosomal dominant Tubulointerstitial Kidney Disease) associated with a mutation of HNF1B, a hereditary tubulointerstitial nephritis with cystic dilatation of the renal tubules (49) and in kidney from rat model of ADPKD (50). This evidence concerns the gene HNF1B and autosomal dominant medullary cystic kidney disease with or without hyperuricemia.