TSHZ3 and congenital anomaly of kidney and urinary tract: Similarly, only 5–27% of heterozygous Tshz3-mutant mice present with a hydronephrosis/proximal hydroureter phenotype [20, 35], and only 5 of 14 (36%) patients with 19q12-q13.11 deletions including the TSHZ3 locus are affected by CAKUT [31–35].