Pkd2 Deficiency in Embryonic Aqp2+ Progenitor Cells Is Sufficient to Cause Severe Polycystic Kidney Disease.
Akaki TsilosaniChao GaoEnuo ChenAndrea R LightleSana ShehzadMadhulika SharmaPamela V TranCarlton Matthew BatesDarren P WallaceWenzheng ZhangPublished in: Journal of the American Society of Nephrology : JASN (2024)
Hence, Pkd2 deletion in embryonic AP, but unlikely in neonate or adult Aqp2+ cells (PC and AP), was sufficient to cause severe PKD with progressive elimination of α-IC, recapitulating a newly identified cellular phenotype of ADPKD patients. We proposed that Pkd2 is critical for balanced AP differentiation into, proliferation and/or maintenance of cystic IC, particularly α-IC.