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EMBO Rep January 1, 2019; 20 (11): e48336.
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The ion channel function of polycystin-1 in the polycystin-1/polycystin-2 complex.

Wang Z , Ng C , Liu X , Wang Y , Li B , Kashyap P , Chaudhry HA , Castro A , Kalontar EM , Ilyayev L , Walker R , Alexander RT , Qian F , Chen XZ , Yu Y .

Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 or PKD2 gene, encoding the polycystic kidney disease protein polycystin-1 and the transient receptor potential channel polycystin-2 (also known as TRPP2), respectively. Polycystin-1 and polycystin-2 form a receptor-ion channel complex located in primary cilia. The function of this complex, especially the role of polycystin-1, is largely unknown due to the lack of a reliable functional assay. In this study, we dissect the role of polycystin-1 by directly recording currents mediated by a gain-of-function (GOF) polycystin-1/polycystin-2 channel. Our data show that this channel has distinct properties from that of the homomeric polycystin-2 channel. The polycystin-1 subunit directly contributes to the channel pore, and its eleven transmembrane domains are sufficient for its channel function. We also show that the cleavage of polycystin-1 at the N-terminal G protein-coupled receptor proteolysis site is not required for the activity of the GOF polycystin-1/polycystin-2 channel. These results demonstrate the ion channel function of polycystin-1 in the polycystin-1/polycystin-2 complex, enriching our understanding of this channel and its role in ADPKD.

PubMed ID: 31441214
PMC ID: PMC6832002
Article link: EMBO Rep
Grant support: [+]

Species referenced: Xenopus
Genes referenced: bmp1 clca1.3 pkd1 pkd2

Article Images: [+] show captions
References [+] :
, Polycystic kidney disease: the complete structure of the PKD1 gene and its protein. The International Polycystic Kidney Disease Consortium. 1995, Pubmed