{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T11:28:15Z","timestamp":1791286095548,"version":"4.1.0"},"reference-count":26,"publisher":"National Academy of Sciences","issue":"3","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2001,1,30]]},"abstract":"<jats:p>\n                    Defects in polycystin-2, a ubiquitous transmembrane glycoprotein of\n unknown function, is a major cause of autosomal dominant polycystic\n kidney disease (ADPKD), whose manifestation entails the development of\n fluid-filled cysts in target organs. Here, we demonstrate that\n polycystin-2 is present in term human syncytiotrophoblast, where it\n behaves as a nonselective cation channel. Lipid bilayer reconstitution\n of polycystin-2-positive human syncytiotrophoblast apical membranes\n displayed a nonselective cation channel with multiple subconductance\n states, and a high perm-selectivity to Ca\n                    <jats:sup>2+<\/jats:sup>\n                    . This channel\n was inhibited by anti-polycystin-2 antibody, Ca\n                    <jats:sup>2+<\/jats:sup>\n                    ,\n La\n                    <jats:sup>3+<\/jats:sup>\n                    , Gd\n                    <jats:sup>3+<\/jats:sup>\n                    , and the diuretic amiloride. Channel\n function by polycystin-2 was confirmed by patch-clamping experiments of\n polycystin-2 heterologously infected Sf9 insect cells. Further,\n purified insect cell-derived recombinant polycystin-2 and\n                    <jats:italic>in\n vitro<\/jats:italic>\n                    translated human polycystin-2 had similar ion channel\n activity. The polycystin-2 channel may be associated with fluid\n accumulation and\/or ion transport regulation in target epithelia,\n including placenta. Dysregulation of this channel provides a mechanism\n for the onset and progression of ADPKD.\n                  <\/jats:p>","DOI":"10.1073\/pnas.98.3.1182","type":"journal-article","created":{"date-parts":[[2012,7,26]],"date-time":"2012-07-26T19:06:14Z","timestamp":1343329574000},"page":"1182-1187","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":454,"title":["Polycystin-2, the protein mutated in autosomal dominant polycystic kidney disease (ADPKD), is a Ca\n                    <sup>2+<\/sup>\n                    -permeable nonselective cation channel"],"prefix":"10.1073","volume":"98","author":[{"given":"Silvia","family":"Gonz\u00e1lez-Perrett","sequence":"first","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keetae","family":"Kim","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Ibarra","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia E.","family":"Damiano","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elsa","family":"Zotta","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisa","family":"Batelli","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter C.","family":"Harris","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignacio L.","family":"Reisin","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Amin","family":"Arnaout","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Horacio F.","family":"Cantiello","sequence":"additional","affiliation":[{"name":"Laboratorio de Canales I\u00f3nicos, Departamento de\r Fisicoqu\u00edmica y Qu\u00edmica Anal\u00edtica, Facultad de\r Farmacia y Bioqu\u00edmica, Buenos Aires, Argentina 1113;\r Renal Unit, Massachusetts General Hospital East,\r Charlestown, MA, 02129 and Department of Medicine, Harvard Medical\r School, Boston, MA 02115; Departamento de\r Fisiolog\u00eda, Facultad de Medicina, Buenos Aires, Argentina 1121;\r and Department of Nephrology, Mayo Clinic, Rochester, MN\r 55905"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2000,12,26]]},"reference":[{"key":"e_1_3_3_1_2","first-page":"1","volume":"328","author":"Dalgaard O Z","year":"1957","unstructured":"O Z Dalgaard Acta Med Scand Suppl 328, 1\u2013255 (1957).","journal-title":"Acta Med Scand Suppl"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.med.52.1.93"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V3121841"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90137-6"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90339-9"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.272.5266.1339"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1038\/43810"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.1998.78.4.1165"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.1997.77.3.805"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)65428-4"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1007\/s002329900218"},{"key":"e_1_3_3_12_2","volume-title":"Ionic Channels of Excitable Membranes","author":"Hille B","year":"1992","unstructured":"B Hille Ionic Channels of Excitable Membranes (Sinauer, Sunderland, MA, 1992)."},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.40.25967"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1988.255.3.C278"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1991.261.5.C882"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1038\/361467a0"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1038\/43907"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.40.28557"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1996.271.5.C1527"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1997.272.1.C27"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.107.6.695"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-2952(94)90474-X"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI112155"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0697-179"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.94.13.6965"},{"key":"e_1_3_3_26_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V115814"}],"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.98.3.1182","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T17:29:42Z","timestamp":1649870982000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.98.3.1182"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2000,12,26]]},"references-count":26,"aliases":["10.1073\/pnas.021456598","10.1073\/pnas.021456598"],"journal-issue":{"issue":"3","published-print":{"date-parts":[[2001,1,30]]}},"alternative-id":["10.1073\/pnas.98.3.1182"],"URL":"https:\/\/doi.org\/10.1073\/pnas.98.3.1182","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[2000,12,26]]},"assertion":[{"value":"2000-09-22","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2000-12-26","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}