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. Author manuscript; available in PMC: 2022 Mar 4.
Published in final edited form as: J Cell Signal. 2022;3(1):62–78. doi: 10.33696/Signaling.3.068

Figure 1: Cyst initiation in ADPKD requires a secondary insult.

Figure 1:

Although ADPKD is dominantly inherited, cyst initiation is recessive at the cellular level. Cyst initiation occurs when tubule epithelial cells harboring one mutated allele of Pkd1 or Pkd2 receive a secondary insult, either by spontaneous somatic mutation of the healthy allele (two-hit hypothesis) or by any mechanism that reduces the function of the polycystin proteins below a critical threshold (threshold model). Created with BioRender.com