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). Here, HNF1B is linked to interstitial nephritis.