{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T14:51:17Z","timestamp":1781103077676,"version":"3.54.1"},"reference-count":63,"publisher":"National Academy of Sciences","issue":"52","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2012,12,26]]},"abstract":"<jats:p>\n                    Polycystic kidney diseases are the most common genetic diseases that affect the kidney. There remains a paucity of information regarding mechanisms by which G proteins are regulated in the context of polycystic kidney disease to promote abnormal epithelial cell expansion and cystogenesis. In this study, we describe a functional role for the accessory protein, G-protein signaling modulator 1 (GPSM1), also known as activator of G-protein signaling 3, to act as a modulator of cyst progression in an orthologous mouse model of autosomal dominant polycystic kidney disease (ADPKD). A complete loss of\n                    <jats:italic>Gpsm1<\/jats:italic>\n                    in the\n                    <jats:italic>Pkd1<\/jats:italic>\n                    <jats:sup>V\/V<\/jats:sup>\n                    mouse model of ADPKD, which displays a hypomorphic phenotype of polycystin-1, demonstrated increased cyst progression and reduced renal function compared with age-matched cystic\n                    <jats:italic>Gpsm1<\/jats:italic>\n                    <jats:sup>+\/+<\/jats:sup>\n                    and\n                    <jats:italic>Gpsm1<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice. Electrophysiological studies identified a role by which GPSM1 increased heteromeric polycystin-1\/polycystin-2 ion channel activity via G\u03b2\u03b3 subunits. In summary, the present study demonstrates an important role for GPSM1 in controlling the dynamics of cyst progression in an orthologous mouse model of ADPKD and presents a therapeutic target for drug development in the treatment of this costly disease.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1216830110","type":"journal-article","created":{"date-parts":[[2012,12,11]],"date-time":"2012-12-11T21:28:16Z","timestamp":1355261296000},"page":"21462-21467","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":38,"title":["G-protein signaling modulator 1 deficiency accelerates cystic disease in an orthologous mouse model of autosomal dominant polycystic kidney disease"],"prefix":"10.1073","volume":"109","author":[{"given":"Michelle","family":"Kwon","sequence":"first","affiliation":[{"name":"Division of Nephrology, Department of Medicine, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tengis S.","family":"Pavlov","sequence":"additional","affiliation":[{"name":"Departments of bPhysiology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kandai","family":"Nozu","sequence":"additional","affiliation":[{"name":"Pediatrics, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shauna A.","family":"Rasmussen","sequence":"additional","affiliation":[{"name":"Departments of bPhysiology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daria V.","family":"Ilatovskaya","sequence":"additional","affiliation":[{"name":"Departments of bPhysiology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Lerch-Gaggl","sequence":"additional","affiliation":[{"name":"Pathology, Medical College of Wisconsin, Milwaukee, WI 53226;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren M.","family":"North","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyunho","family":"Kim","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD 21205; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Feng","family":"Qian","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD 21205; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"William E.","family":"Sweeney","sequence":"additional","affiliation":[{"name":"Pediatrics, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellis D.","family":"Avner","sequence":"additional","affiliation":[{"name":"Departments of bPhysiology,"},{"name":"Pediatrics, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joe B.","family":"Blumer","sequence":"additional","affiliation":[{"name":"Department of Cell and Molecular Pharmacology, Medical University of South Carolina, Charleston, SC 29425"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Staruschenko","sequence":"additional","affiliation":[{"name":"Departments of bPhysiology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Park","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, and"},{"name":"Departments of bPhysiology,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2012,12,10]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90339-9"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0695-151"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.272.5266.1339"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.V571462"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/ki.2009.128"},{"key":"e_1_3_3_6_2","first-page":"F434","article-title":"Epithelial cell polarity and disease","volume":"272","author":"Wilson PD","year":"1997","unstructured":"PD Wilson, Epithelial cell polarity and disease. 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