{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T14:24:09Z","timestamp":1793111049768,"version":"4.5.0"},"reference-count":67,"publisher":"National Academy of Sciences","issue":"18","license":[{"start":{"date-parts":[[2022,4,28]],"date-time":"2022-04-28T00:00:00Z","timestamp":1651104000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2022,5,3]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Juvenile nephronophthisis (NPH) is a renal ciliopathy due to a dysfunction of primary cilia for which no curative treatment is available. This paper describes the identification of agonists of prostaglandin E\n                    <jats:sub>2<\/jats:sub>\n                    receptors as a potential therapeutic approach for the most common\n                    <jats:italic>NPHP1<\/jats:italic>\n                    -associated ciliopathies. We demonstrated that prostaglandin E\n                    <jats:sub>1<\/jats:sub>\n                    rescues defective ciliogenesis and ciliary composition in\n                    <jats:italic>NPHP1<\/jats:italic>\n                    patient urine-derived renal tubular cells and improves ciliary and kidney phenotypes in our NPH zebrafish and\n                    <jats:italic>\n                      Nphp1\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    <\/jats:italic>\n                    mouse models. In addition, Taprenepag alleviates the severe retinopathy observed in\n                    <jats:italic>\n                      Nphp1\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    <\/jats:italic>\n                    mice. Finally, transcriptomic analyses pointed out several pathways downstream the prostaglandin receptors as cell cycle progression, extracellular matrix, or actin cytoskeleton organization. Altogether, our findings provide an alternative for treatment of NPH.\n                  <\/jats:p>","DOI":"10.1073\/pnas.2115960119","type":"journal-article","created":{"date-parts":[[2022,4,28]],"date-time":"2022-04-28T14:31:31Z","timestamp":1651156291000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":49,"title":["Agonists of prostaglandin E\n                    <sub>2<\/sub>\n                    receptors as potential first in class treatment for nephronophthisis and related ciliopathies"],"prefix":"10.1073","volume":"119","author":[{"given":"Hugo","family":"Garcia","sequence":"first","affiliation":[{"name":"Laboratory of Hereditary Kidney Disease, Imagine Institute, Universit\u00e9 Paris Cit\u00e9, INSERM UMR 1163, 75015 Paris, France"},{"name":"Department of Nephrology, Groupe Hospitalier Sorbonne Universit\u00e9, 75013 Paris, 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