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Maryland PKD Research and Translation Core Center","award":["U54DK126114"],"award-info":[{"award-number":["U54DK126114"]}]},{"name":"U54 center grant to Maryland PKD Research and Translation Core Center","award":["U54DK126114"],"award-info":[{"award-number":["U54DK126114"]}]},{"name":"Pilot and Feasibility sub-awards from Mayo Clinic Translational PKD Center","award":["P30DK90728"],"award-info":[{"award-number":["P30DK90728"]}]},{"name":"Pilot and Feasibility sub-awards from Mayo Clinic Translational PKD Center","award":["P30DK90728"],"award-info":[{"award-number":["P30DK90728"]}]},{"name":"Pilot and Feasibility sub-awards from Mayo Clinic Translational PKD Center","award":["P30DK90728"],"award-info":[{"award-number":["P30DK90728"]}]},{"DOI":"10.13039\/100014535","name":"Center for Individualized Medicine, Mayo Clinic","doi-asserted-by":"crossref","award":["P30DK90728"],"award-info":[{"award-number":["P30DK90728"]}],"id":[{"id":"10.13039\/100014535","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Pilot and Feasibility sub-awards from Mayo Clinic Translational PKD Center","award":["P30DK90728"],"award-info":[{"award-number":["P30DK90728"]}]}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["JASN"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:sec>\n                    <jats:title>Key Points<\/jats:title>\n                    <jats:p>\n                      <jats:list list-type=\"bullet\">\n                        <jats:list-item>\n                          <jats:p>Inositol polyphosphate-5-phosphatase E (INPP5E) and type Ig phosphatidylinositol-4-phosphate 5-kinase (PIPKI\u03b3) coordinated the homeostasis of phosphatidylinositol-4-phosphate and phosphatidylinositol-4,5-bisphosphate in primary cilia.<\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>Modulating INPP5E or PIPKI\u03b3 activity changed the level of polycystin-1 and polycystin-2 in primary cilia.<\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>\n                            INPP5E inhibition increased the hypomorphic PKD1 variants (PC1-R3277C) in cilia and reduced cystogenesis in the kidney\n                            <jats:italic toggle=\"yes\">in vitro<\/jats:italic>\n                            .\n                          <\/jats:p>\n                        <\/jats:list-item>\n                      <\/jats:list>\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Autosomal dominant polycystic kidney disease is mainly caused by mutations in\n                      <jats:italic toggle=\"yes\">PKD1<\/jats:italic>\n                      and\n                      <jats:italic toggle=\"yes\">PKD2<\/jats:italic>\n                      , which encode polycystin-1 (PC1) and polycystin-2 (PC2), respectively. PC1 and PC2 assemble a cation channel complex enriched in primary cilium, a sensory organelle associated with various developmental diseases, including polycystic kidney disease (PKD). Accumulating evidence supports the necessity of functional polycystin (PC) complex in cilia to prevent cystogenesis in the kidney, indicating that improving their ciliary levels may ameliorate defects underlying PKD pathogenesis. Yet, molecular mechanisms underlying the ciliary targeting and homeostasis of the PC complex are not fully understood.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Indirect immunofluorescence microscopy was used to monitor ciliary levels of PC1 and PC2 in renal epithelial cells. Electrophysiology analysis in oocytes was used to determine the channel activity of the PC complex. Cystogenesis in the kidney was measured using\n                      <jats:italic toggle=\"yes\">in vitro<\/jats:italic>\n                      3D-Matrigel cell models and\n                      <jats:italic toggle=\"yes\">ex vivo<\/jats:italic>\n                      mouse embryonic kidney models.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Suppressing inositol polyphosphate-5-phosphatase E (INPP5E) or activating type Ig phosphatidylinositol-4-phosphate 5-kinase raised ciliary levels of the PC complex in both normal renal epithelial cells and cells carrying autosomal dominant polycystic kidney disease mutations that interrupt the trafficking of PCs into cilia, including\n                      <jats:italic toggle=\"yes\">GANAB<\/jats:italic>\n                      inactivation and the trafficking\n                      <jats:italic toggle=\"yes\">PKD1<\/jats:italic>\n                      mutation p.Arg3277Cys (RC). PC1\n                      <jats:sup>RC<\/jats:sup>\n                      formed a complex with PC2 and exhibited normal channel activity\n                      <jats:italic toggle=\"yes\">in vitro<\/jats:italic>\n                      . An INPP5E that increases PC1 and PC2 in cilia, suppressed\n                      <jats:italic toggle=\"yes\">in vitro<\/jats:italic>\n                      forskolin-induced cystogenesis of inner medullary collecting duct epithelial cell line 3 cells in 3D Matrigel and\n                      <jats:italic toggle=\"yes\">ex vivo<\/jats:italic>\n                      cyst formation in embryonic\n                      <jats:italic toggle=\"yes\">Pkd1<\/jats:italic>\n                      <jats:sup>RC\/RC<\/jats:sup>\n                      mouse kidneys.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Our results demonstrated that increasing the ciliary level of PCs, by manipulating a ciliary phosphoinositide signaling axis, enhanced the functionality of PCs and suppressed cystogenesis of renal epithelial cells\n                      <jats:italic toggle=\"yes\">in vitro<\/jats:italic>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1681\/asn.0000000942","type":"journal-article","created":{"date-parts":[[2025,11,25]],"date-time":"2025-11-25T17:00:17Z","timestamp":1764090017000},"page":"944-958","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":2,"title":["A Ciliary Phosphoinositide Pathway Regulates the Dosage of Polycystins in Primary Cilia"],"prefix":"10.1681","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0694-0867","authenticated-orcid":false,"given":"Chuan","family":"Chen","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, Minnesota"},{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, 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