Even though the analyzed studies demonstrate that HNF1β defects disturb apical-basolateral cell polarity and tight junction integrity, these mechanisms have never been considered in the pathogenesis of electrolyte disturbances observed in ADTKD-HNF1β patients [25, 103, 105]. This evidence concerns the gene HNF1B and autosomal dominant medullary cystic kidney disease with or without hyperuricemia.