While their biallelic loss-of-function variants cause Mainzer-Saldino syndrome [16], renal-hepatic-pancreatic dysplasia [17–19], or steroid-resistant nephrotic syndrome [20], respectively, monoallelic variants of IFT140 and NEK8 were found causal in rare forms of ADPKD [21–23] and those of NPHS2 in focal segmental glomerulosclerosis (FSGS) [24]. Here, NPHS2 is linked to focal segmental glomerulosclerosis.