Abstract Background and Aims Autosomal Dominant Tubulointerstitial Kidney Diseases (ADTKD) are caused by mutations in one of at least five genes (UMOD, MUC1, HNF1B, REN SEC61A1) characterized progressive decline kidney function, reaching end-stage renal disease (ESRD) usually mid adulthood. ADTKD-MUC1 is the most frequent subforms, exclusively a characteristic frameshift mutation, leading to de...