| First Author | Wang Z | Year | 2019 |
| Journal | EMBO Rep | Volume | 20 |
| Issue | 11 | Pages | e48336 |
| PubMed ID | 31441214 | Mgi Jnum | J:282579 |
| Mgi Id | MGI:6379116 | Doi | 10.15252/embr.201948336 |
| Citation | Wang Z, et al. (2019) The ion channel function of polycystin-1 in the polycystin-1/polycystin-2 complex. EMBO Rep 20(11):e48336 |
| abstractText | 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. |
