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Review
. 2019 Feb;15(2):70-72.
doi: 10.1038/s41581-018-0108-1.

Progress in the understanding of polycystic kidney disease

Vicente E Torres  1 , Peter C Harris  2
Affiliations
Review

Progress in the understanding of polycystic kidney disease

Vicente E Torres et al. Nat Rev Nephrol. 2019 Feb.

Abstract

The function of polycystin proteins and the pathogenesis of autosomal dominant polycystic kidney disease (ADPKD) are not well understood. Studies published in 2018 made important contributions to the understanding of genetic mechanisms, the structure of the polycystin complex and the roles of G-protein signalling and the immune system in ADPKD.

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Figures

Fig. 1∣
Fig. 1∣. The structure of a PC1-PC2 complex.
a ∣ A polycystin complex composed of three polycystin 2 (PC2) and one polycystin 1 (PC1) subunits. b ∣ The last transmembrane domain of PC1 (S6) has two offset segments (S6a and S6b) and positively charged residues in the pore (+) that may block Ca2+ permeability. NTD, amino-terminal domain; PLAT, polycystin 1, lipoxygenase, and α-toxin region; TOP, TOP domain; VGIC, voltage-gated ion channel. Reproduced with permission from REF., AAAS.
See this image and copyright information in PMC

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