Rare (MAF < 0.002 according to gnomAD v4.1.0, total population) non-silent TSHZ3 variants within this region (amino acids 1–182 of TSHZ3) were significantly more frequent in CAKUT patients compared to gnomAD samples (3.2% versus 0.7%, p = 0.000078447, two-tailed Fisher’s exact test). Here, TSHZ3 is linked to congenital anomaly of kidney and urinary tract.