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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 Zhang
Published in: Journal of the American Society of Nephrology : JASN (2024)
Hence, Pkd2 deletion in embryonic AP, but unlikely in neonate or adult Aqp2 + cells (principal cells and AP), was sufficient to cause severe PKD with progressive elimination of α -intercalated cells, recapitulating a newly identified cellular phenotype of patients with ADPKD. We proposed that Pkd2 is critical for balanced AP differentiation into, proliferation, and/or maintenance of cystic intercalated cells, particularly α -intercalated cells.
Keyphrases
  • induced apoptosis
  • polycystic kidney disease
  • signaling pathway
  • transcription factor
  • endoplasmic reticulum stress
  • oxidative stress
  • multiple sclerosis
  • cell proliferation
  • early onset
  • drug induced