{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T21:05:48Z","timestamp":1790888748759,"version":"4.1.0"},"reference-count":43,"publisher":"National Academy of Sciences","issue":"15","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2001,7,17]]},"abstract":"\n The voltage-dependent K\n +<\/jats:sup>\n channel responsible for the slowly activating delayed K\n +<\/jats:sup>\n current\n I<\/jats:italic>\n Ks<\/jats:sub>\n is composed of pore-forming KCNQ1 and regulatory KCNE1 subunits, which are mutated in familial forms of cardiac long QT syndrome. Because\n KCNQ1<\/jats:italic>\n and\n KCNE1<\/jats:italic>\n genes also are expressed in epithelial tissues, such as the kidneys and the intestine, we have investigated the adaptation of KCNE1-deficient mice to different K\n +<\/jats:sup>\n and Na\n +<\/jats:sup>\n intakes. On a normal K\n +<\/jats:sup>\n diet, homozygous\n kcne1<\/jats:italic>\n \u2212\/\u2212<\/jats:sup>\n mice exhibit signs of chronic volume depletion associated with fecal Na\n +<\/jats:sup>\n and K\n +<\/jats:sup>\n wasting and have lower plasma K\n +<\/jats:sup>\n concentration and higher levels of aldosterone than wild-type mice. Although plasma aldosterone can be suppressed by low K\n +<\/jats:sup>\n diets or stimulated by low Na\n +<\/jats:sup>\n diets, a high K\n +<\/jats:sup>\n diet provokes a tremendous increase of plasma aldosterone levels in\n kcne1<\/jats:italic>\n \u2212\/\u2212<\/jats:sup>\n mice as compared with wild-type mice (7.1-fold vs. 1.8-fold) despite lower plasma K\n +<\/jats:sup>\n in\n kcne1<\/jats:italic>\n \u2212\/\u2212<\/jats:sup>\n mice. This exacerbated aldosterone production in\n kcne1<\/jats:italic>\n \u2212\/\u2212<\/jats:sup>\n mice is accompanied by an abnormally high plasma renin concentration, which could partly explain the hyperaldosteronism. In addition, we found that KCNE1 and KCNQ1 mRNAs are expressed in the zona glomerulosa of adrenal glands where\n I<\/jats:italic>\n Ks<\/jats:sub>\n may directly participate in the control of aldosterone production by plasma K\n +<\/jats:sup>\n . These results, which show that KCNE1 and\n I<\/jats:italic>\n Ks<\/jats:sub>\n are involved in K\n +<\/jats:sup>\n homeostasis, might have important implications for patients with\n I<\/jats:italic>\n Ks<\/jats:sub>\n -related long QT syndrome, because hypokalemia is a well known risk factor for the occurrence of torsades de pointes ventricular arrhythmia.\n <\/jats:p>","DOI":"10.1073\/pnas.141233398","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T10:34:10Z","timestamp":1027679650000},"page":"8792-8797","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":54,"title":["Altered potassium balance and aldosterone secretion in a mouse model of human congenital long QT syndrome"],"prefix":"10.1073","volume":"98","author":[{"given":"Isabelle","family":"Arrighi","sequence":"first","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"May","family":"Bloch-Faure","sequence":"additional","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian","family":"Grahammer","sequence":"additional","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Bleich","sequence":"additional","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Warth","sequence":"additional","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymond","family":"Mengual","sequence":"additional","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Milou-Daniel","family":"Drici","sequence":"additional","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacques","family":"Barhanin","sequence":"additional","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Meneton","sequence":"additional","affiliation":[{"name":"Institut de Pharmacologie Mol\u00e9culaire et Cellulaire, Centre National de la Recherche Scientifique, 660, Route des Lucioles, Sophia Antipolis, 06560 Valbonne, France; Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale U367, 17, Rue du Fer \u00e0 Moulin, 75005 Paris, France; Institute of Physiology, University of Freiburg, Hermann-Herder-Strasse 7, D-79104 Freiburg, Germany; and Centre Hospitalo- Universitaire de Nice, 06000 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2001,7,3]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1038\/384078a0"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/384080a0"},{"key":"e_1_3_3_3_2","first-page":"438","volume":"19","author":"Meneton P","year":"1999","unstructured":"P Meneton, F Lesage, J Barhanin Semin Nephrol 19, 438\u2013457 (1999).","journal-title":"Semin Nephrol"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-6147(00)01475-9"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.99.4.518"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.97.24.2420"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0008-6363(99)00411-3"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8703(57)90079-0"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(00)80255-X"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.83.1.95"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.3194754"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0008-6363(98)00043-1"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/clc.4960200318"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI1720"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1096-152"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90250-X"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0696-183"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0196-24"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1038\/969"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1210\/endo-90-2-422"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00704157"},{"key":"e_1_3_3_22_2","first-page":"586","volume":"73","author":"Sibony M","year":"1995","unstructured":"M Sibony, F Commo, P Callard, J M Gasc Lab Invest 73, 586\u2013591 (1995).","journal-title":"Lab Invest"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1991.tb07829.x"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01869604"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-6147(96)01016-4"},{"key":"e_1_3_3_26_2","doi-asserted-by":"publisher","DOI":"10.1038\/35003200"},{"key":"e_1_3_3_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1050-1738(98)00013-9"},{"key":"e_1_3_3_28_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.2001.280.2.C359"},{"key":"e_1_3_3_29_2","first-page":"202","volume":"440","author":"Bleich M","year":"2000","unstructured":"M Bleich, R Warth Pflugers Arch 440, 202\u2013206 (2000).","journal-title":"Pflugers Arch"},{"key":"e_1_3_3_30_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V871179"},{"key":"e_1_3_3_31_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V10122527"},{"key":"e_1_3_3_32_2","doi-asserted-by":"publisher","DOI":"10.1097\/00041552-199801000-00008"},{"key":"e_1_3_3_33_2","first-page":"F817","volume":"43","author":"Giebisch G","year":"1998","unstructured":"G Giebisch Am J Physiol 43, F817\u2013F833 (1998).","journal-title":"Am J Physiol"},{"key":"e_1_3_3_34_2","unstructured":"Vallon V. Grahammer F. Richter K. Bleich M. Lang F. Barhanin J. Voelkl H. & Warth R. (2001) J. Am. Soc. Nephrol. in press."},{"key":"e_1_3_3_35_2","first-page":"2145","volume-title":"Hypertension: Pathophysiology, Diagnosis, and Management","author":"Biglieri E G","year":"1995","unstructured":"E G Biglieri, C E Kater, F Mantero Hypertension: Pathophysiology, Diagnosis, and Management, eds J H Laragh, B M Brenner (Raven, 2nd Ed., New York), pp. 2145\u20132162 (1995).","edition":"2"},{"key":"e_1_3_3_36_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0008-6363(99)00088-7"},{"key":"e_1_3_3_37_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1999.276.1.C182"},{"key":"e_1_3_3_38_2","doi-asserted-by":"publisher","DOI":"10.1210\/mend.14.6.0466"},{"key":"e_1_3_3_39_2","first-page":"199","volume-title":"Current Topics in Membranes","author":"Lesage F","year":"1999","unstructured":"F Lesage, M Lazdunski Current Topics in Membranes, eds Y Kurachi, L Y Jan, M Lazdunski (Academic, San Diego) 46, 199\u2013222 (1999)."},{"key":"e_1_3_3_40_2","doi-asserted-by":"publisher","DOI":"10.1210\/endo.138.10.5463"},{"key":"e_1_3_3_41_2","doi-asserted-by":"publisher","DOI":"10.1007\/s002329900206"},{"key":"e_1_3_3_42_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110262"},{"key":"e_1_3_3_43_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199909023411001"}],"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.141233398","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,12]],"date-time":"2022-04-12T23:55:22Z","timestamp":1649807722000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.141233398"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,7,3]]},"references-count":43,"journal-issue":{"issue":"15","published-print":{"date-parts":[[2001,7,17]]}},"alternative-id":["10.1073\/pnas.141233398"],"URL":"https:\/\/doi.org\/10.1073\/pnas.141233398","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[2001,7,3]]},"assertion":[{"value":"2000-12-21","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2001-05-11","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2001-07-03","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}