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Polycystin-2 belongs to the transient receptor potential (TRP) family of non-selective cation channels. To test the hypothesis that altered ion channel properties of polycystin-2 compromise its putative role in a control circuit controlling lumen formation of renal tubular structures, we generated a mouse model in which we exchanged the pore loop of polycystin-2 with that of the closely related cation channel polycystin-2L1 (encoded by PKD2L1), thereby creating the protein polycystin-2poreL1. Functional characterization of this mutant channel in Xenopus laevis oocytes demonstrated that its electrophysiological properties differed from those of polycystin-2 and instead resembled the properties of polycystin-2L1, in particular regarding its permeability for Ca2+ ions. Homology modeling of the ion translocation pathway of polycystin-2poreL1 argues for a wider pore in polycystin-2poreL1 than in polycystin-2. In Pkd2poreL1 knock-in mice in which the endogenous polycystin-2 protein was replaced by polycystin-2poreL1 the diameter of collecting ducts was increased and collecting duct cysts developed in a strain-dependent fashion.<\/jats:p>","DOI":"10.1242\/jcs.259013","type":"journal-article","created":{"date-parts":[[2021,8,4]],"date-time":"2021-08-04T08:59:32Z","timestamp":1628067572000},"source":"Crossref","is-referenced-by-count":7,"title":["A polycystin-2 protein with modified channel properties leads to an increased diameter of renal tubules and to renal cysts"],"prefix":"10.1242","volume":"134","author":[{"given":"Melanie","family":"Grosch","sequence":"first","affiliation":[{"name":"Institute for Molecular and Cellular Anatomy, University of Regensburg, 93053 Regensburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katrin","family":"Brunner","sequence":"additional","affiliation":[{"name":"Institute for Molecular and Cellular Anatomy, University of Regensburg, 93053 Regensburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexandr V.","family":"Ilyaskin","sequence":"additional","affiliation":[{"name":"Institute of Cellular and Molecular Physiology, Friedrich-Alexander University of Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Schober","sequence":"additional","affiliation":[{"name":"Institute for Molecular and Cellular Anatomy, University of Regensburg, 93053 Regensburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tobias","family":"Staudner","sequence":"additional","affiliation":[{"name":"Institute of Cellular and Molecular Physiology, Friedrich-Alexander University of Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Denise","family":"Schmied","sequence":"additional","affiliation":[{"name":"Institute for Molecular and Cellular Anatomy, University of Regensburg, 93053 Regensburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tina","family":"Stumpp","sequence":"additional","affiliation":[{"name":"Institute for Molecular and Cellular Anatomy, University of Regensburg, 93053 Regensburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kerstin N.","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Institute for Molecular and Cellular Anatomy, University of Regensburg, 93053 Regensburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. 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