{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T21:37:33Z","timestamp":1790199453787,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,3,13]],"date-time":"2014-03-13T00:00:00Z","timestamp":1394668800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Genesis"],"published-print":{"date-parts":[[2014,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Sphingomyelin phosphodiesterase 3 (SMPD3) is a pleiotropic lipid metabolizing enzyme involved in multiple physiological processes. A deletion mutation in the murine <jats:italic>Smpd3<\/jats:italic> gene called <jats:italic>fragilitas ossium (fro<\/jats:italic>) leads to severe skeletal abnormalities in the developing <jats:italic>fro\/fro<\/jats:italic> embryos. Although <jats:italic>fro\/fro<\/jats:italic> mice can be useful to study many different aspects of SMPD3 functions, their perinatal lethality makes it difficult to generate a sufficient number of mice for controlled studies. In fact, on the C57BL\/6 genetic background, none of the <jats:italic>fro\/fro<\/jats:italic> mice survive beyond the perinatal stage. In this study, we used the \u201cTet\u2010On\u201d inducible gene expression system to express <jats:italic>Smpd3<\/jats:italic> transiently in <jats:italic>fro\/fro;ROSA\u2010rtTA;TRE\u2010Smpd3<\/jats:italic> embryos on the C57BL\/6 background. This induced <jats:italic>Smpd3<\/jats:italic> expression corrected all the skeletal abnormalities in these embryos and prevented their early death. However, induction of <jats:italic>Smpd3<\/jats:italic> expression in the adolescent <jats:italic>fro\/fro;ROSA\u2010rtTA;TRE\u2010Smpd3<\/jats:italic> mice was not sufficient to correct the defects in trabecular bone mineralization and the impaired growth of the long bones. This novel mouse model will be a useful tool to study SMPD3 biology in vivo. genesis 52:408\u2013416, 2014. \u00a9 2014 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/dvg.22765","type":"journal-article","created":{"date-parts":[[2014,3,3]],"date-time":"2014-03-03T09:37:10Z","timestamp":1393839430000},"page":"408-416","source":"Crossref","is-referenced-by-count":6,"title":["Inducible transient expression of <i>Smpd3<\/i> prevents early lethality in <i>fro\/fro<\/i> mice"],"prefix":"10.1002","volume":"52","author":[{"given":"Sharifa","family":"Alebrahim","sequence":"first","affiliation":[{"name":"Faculty of Dentistry McGill University Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zohreh","family":"Khavandgar","sequence":"additional","affiliation":[{"name":"Faculty of Dentistry McGill University Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliana","family":"Marulanda","sequence":"additional","affiliation":[{"name":"Faculty of Dentistry McGill University Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monzur","family":"Murshed","sequence":"additional","affiliation":[{"name":"Faculty of Dentistry McGill University Montreal Quebec Canada"},{"name":"Department of Medicine McGill University Montreal Quebec Canada"},{"name":"Genetics Unit, Shriners Hospital for Children, McGill University Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,3,13]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-7091-1368-4_3"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1603"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gni051"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12033-011-9461-z"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0009-3084(99)00077-8"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01221856"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.5.9.1513"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1177\/154405910808700103"},{"key":"e_1_2_5_10_1","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1093\/oxfordjournals.jhered.a109554","article-title":"Fragilitas ossium: A new autosomal recessive mutation in the mouse","volume":"72","author":"Guenet JL","year":"1981","journal-title":"J Hered"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrm2329"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1134\/S0006297906070030"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.18-09-03470.1998"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/0022034513478429"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201102051"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00335-012-9419-x"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2007.061285"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.51.30701"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/ilar.47.2.94"}],"container-title":["genesis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fdvg.22765","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/dvg.22765","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,6]],"date-time":"2023-10-06T11:22:50Z","timestamp":1696591370000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/dvg.22765"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,3,13]]},"references-count":18,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2014,5]]}},"alternative-id":["10.1002\/dvg.22765"],"URL":"https:\/\/doi.org\/10.1002\/dvg.22765","archive":["Portico"],"relation":{},"ISSN":["1526-954X","1526-968X"],"issn-type":[{"value":"1526-954X","type":"print"},{"value":"1526-968X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,3,13]]}}}