{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T13:09:22Z","timestamp":1792069762323,"version":"4.4.0"},"reference-count":22,"publisher":"Springer Science and Business Media LLC","issue":"6","license":[{"start":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T00:00:00Z","timestamp":1598918400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T00:00:00Z","timestamp":1598918400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"University of Pennsylvania Orphan Disease Center in partnership with the Fibrous Dysplasia Foundation","award":["MDBR-18-114-FD\/MAS"],"award-info":[{"award-number":["MDBR-18-114-FD\/MAS"]}]},{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"crossref","award":["GGP15198"],"award-info":[{"award-number":["GGP15198"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Sapienza University","award":["RM11916B839074A8"],"award-info":[{"award-number":["RM11916B839074A8"]}]},{"name":"Sapienza University","award":["RP11715C7C4DC57A"],"award-info":[{"award-number":["RP11715C7C4DC57A"]}]},{"name":"Sapienza University","award":["RM118164289636F0"],"award-info":[{"award-number":["RM118164289636F0"]}]},{"name":"Universit\u00e0 degli Studi di Roma La Sapienza"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Calcif Tissue Int"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    We compared the effects of a nitrogen-containing bisphosphonate (N-BP), zoledronic acid (ZA), and an anti-mouse RANKL antibody (anti-mRANKL Ab) on the bone tissue pathology of a transgenic mouse model of human fibrous dysplasia (FD). For comparison, we also reviewed the histological samples of a child with McCune\u2013Albright syndrome (MAS) treated with Pamidronate for 3\u00a0years. EF1\u03b1-Gs\u03b1\n                    <jats:sup>R201C<\/jats:sup>\n                    mice with FD-like lesions in the tail vertebrae were treated with either 0.2\u00a0mg\/kg of ZA at day 0, 7, and 14 or with 300\u00a0\u03bcg\/mouse of anti-mRANKL Ab at day 0 and 21. All mice were monitored by Faxitron and histological analysis was performed at day 42. ZA did not affect the progression of the radiographic phenotype in EF1\u03b1-Gs\u03b1\n                    <jats:sup>R201C<\/jats:sup>\n                    mice. FD-like lesions in the ZA group showed the persistence of osteoclasts, easily detectable osteoclast apoptotic activity and numerous \u201cgiant osteoclasts\u201d. In contrast, in the anti-mRANKL Ab-treated mice, osteoclasts were markedly reduced\/absent, the radiographic phenotype reverted and the FD-like lesions were extensively replaced by newly formed bone. Numerous \u201cgiant osteoclasts\u201d were also detected in the samples of the child with MAS. This study supports the hypothesis that osteoclasts per se, independently of their resorptive activity, are essential for development and expansion of FD lesions.\n                  <\/jats:p>","DOI":"10.1007\/s00223-020-00752-w","type":"journal-article","created":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T14:45:46Z","timestamp":1598971546000},"page":"603-610","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Zoledronic Acid in a Mouse Model of Human Fibrous Dysplasia: Ineffectiveness on Tissue Pathology, Formation of \u201cGiant Osteoclasts\u201d and Pathogenetic Implications"],"prefix":"10.1007","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6896-4509","authenticated-orcid":false,"given":"Alessandro","family":"Corsi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biagio","family":"Palmisano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Spica","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annamaria","family":"Di Filippo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilenia","family":"Coletta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Dello Spedale Venti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rossella","family":"Labella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Fabretti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Donsante","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Remoli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Serafini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8643-9211","authenticated-orcid":false,"given":"Mara","family":"Riminucci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,9,1]]},"reference":[{"key":"752_CR1","doi-asserted-by":"publisher","first-page":"1511","DOI":"10.2106\/JBJS.F.00726","volume":"89","author":"M Al Muderis","year":"2007","unstructured":"Al Muderis M, Azzopardi T, Cundy P (2007) Zebra lines of pamidronate therapy in children. 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All procedures performed on the human participant were in accordance with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Human and Animal Rights"}},{"value":"The human patient reported in this study has been already published (reference number 18 of the manuscript).","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed Consent"}}]}}