The CAKUT phenotypes detected in Tshz3-mutant mice from E16.5 [20, 35, 38] and most frequently in carriers of TSHZ3 missense variants of this study, i.e., hydronephrosis, hydroureter and/or MCDK, may be linked to the expression pattern of TSHZ3. Using RNA in situ hybridization on wild-type mice, Tshz3 expression was observed in the mesenchymal compartment of the ureter from E11.5 and in the medullary stroma of the kidney from E14.5, was diminished at E16.5, and further reduced at E18.5. The gene discussed is TSHZ3; the disease is Hydroureter.