{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T20:31:16Z","timestamp":1791664276063,"version":"4.3.4"},"reference-count":45,"publisher":"National Academy of Sciences","issue":"3","license":[{"start":{"date-parts":[[2017,12,27]],"date-time":"2017-12-27T00:00:00Z","timestamp":1514332800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000072","name":"HHS | NIH | National Institute of Dental and Craniofacial Research","doi-asserted-by":"publisher","award":["Z01DE00551"],"award-info":[{"award-number":["Z01DE00551"]}],"id":[{"id":"10.13039\/100000072","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000057","name":"HHS | NIH | National Institute of General Medical Sciences","doi-asserted-by":"publisher","award":["GM007752"],"award-info":[{"award-number":["GM007752"]}],"id":[{"id":"10.13039\/100000057","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2018,1,16]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    We generated a novel conditional inducible mouse model of fibrous dysplasia (FD) by expressing mutant\n                    <jats:italic>GNAS<\/jats:italic>\n                    in skeletal stem cells (SSCs) in a temporally controlled and tissue-specific fashion. Typical FD bone lesions developed rapidly in mutant embryos and postnatal mice.\n                    <jats:italic>GNAS<\/jats:italic>\n                    promoted PKA activation and proliferation of SSCs along the osteogenic lineage but impaired their differentiation to mature osteoblasts and triggered increased osteoclastogenesis and bone resorption. FD lesions reverted on cessation of\n                    <jats:italic>GNAS<\/jats:italic>\n                    expression. Thus,\n                    <jats:italic>GNAS<\/jats:italic>\n                    mutation is sufficient and necessary for FD initiation and maintenance. This model provides a valuable opportunity to identify the molecular mechanism underlying FD progression and accelerate the development of more effective treatment options.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1713710115","type":"journal-article","created":{"date-parts":[[2017,12,27]],"date-time":"2017-12-27T14:36:52Z","timestamp":1514385412000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":50,"title":["Expression of an active G\u03b1\n                    <sub>s<\/sub>\n                    mutant in skeletal stem cells is sufficient and necessary for fibrous dysplasia initiation and maintenance"],"prefix":"10.1073","volume":"115","author":[{"given":"Xuefeng","family":"Zhao","sequence":"first","affiliation":[{"name":"State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu, 610041, China;"},{"name":"Moores Cancer Center, University of California, San Diego, La Jolla, CA 92093;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peng","family":"Deng","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu, 610041, China;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramiro","family":"Iglesias-Bartolome","sequence":"additional","affiliation":[{"name":"National Cancer Institute, National Institutes of Health, Bethesda, MD 20892;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Panomwat","family":"Amornphimoltham","sequence":"additional","affiliation":[{"name":"Moores Cancer Center, University of California, San Diego, La Jolla, CA 92093;"},{"name":"International College of Dentistry, Walailak University, Nakhon Si Thammarat, 80161, Thailand;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dana J.","family":"Steffen","sequence":"additional","affiliation":[{"name":"Moores Cancer Center, University of California, San Diego, La Jolla, CA 92093;"},{"name":"Department of Pharmacology, University of California, San Diego, La Jolla, CA 92093;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yunyun","family":"Jin","sequence":"additional","affiliation":[{"name":"Department of Developmental Biology, Harvard School of Dental Medicine, Boston, MA 02115;"},{"name":"Institute of Biomedical Sciences and School of Life Sciences, East China Normal University, Shanghai, 200241, China;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfredo A.","family":"Molinolo","sequence":"additional","affiliation":[{"name":"Moores Cancer Center, University of California, San Diego, La Jolla, CA 92093;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis Fernandez","family":"de Castro","sequence":"additional","affiliation":[{"name":"Section on Skeletal Disorders and Mineral Homeostasis, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Ovejero","sequence":"additional","affiliation":[{"name":"Section on Skeletal Disorders and Mineral Homeostasis, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2836-1081","authenticated-orcid":false,"given":"Quan","family":"Yuan","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu, 610041, China;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qianming","family":"Chen","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu, 610041, China;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xianglong","family":"Han","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu, 610041, China;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ding","family":"Bai","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu, 610041, China;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan S.","family":"Taylor","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, University of California, San Diego, La Jolla, CA 92093;"},{"name":"Department of Chemistry and Biochemistry, University of California, San Diego, La Jolla, CA 92093"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3933-887X","authenticated-orcid":false,"given":"Yingzi","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Developmental Biology, Harvard School of Dental Medicine, Boston, MA 02115;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael T.","family":"Collins","sequence":"additional","affiliation":[{"name":"Section on Skeletal Disorders and Mineral Homeostasis, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Silvio","family":"Gutkind","sequence":"additional","affiliation":[{"name":"Moores Cancer Center, University of California, San Diego, La Jolla, CA 92093;"},{"name":"Department of Pharmacology, University of California, San Diego, La Jolla, CA 92093;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2017,12,27]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199112123252403"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11832-007-0006-8"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11914-015-0268-x"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI2361"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.1999.14.3.336"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.06s224"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1677\/JME-10-0097"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)80943-6"},{"key":"e_1_3_3_9_2","first-page":"179","article-title":"Albright\u2019s syndrome-A group of cases characterized by osteitis fibrosa disseminata, areas of pigmentation and a gonadal dysfunction","volume":"57","author":"Gorham LW","year":"1941","unstructured":"LW Gorham, EH Campbell, WC Howard, JL Donhauser, NH Rust, Albright\u2019s syndrome-A group of cases characterized by osteitis fibrosa disseminata, areas of pigmentation and a gonadal dysfunction. Trans Am Clin Climatol Assoc 57, 179\u2013187 (1941).","journal-title":"Trans Am Clin Climatol Assoc"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1210\/en.2004-0865"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.3389\/fnins.2015.00129"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-7-S1-S4"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrc3521"},{"key":"e_1_3_3_14_2","first-page":"1848","article-title":"Fibrous dysplasia. Pathophysiology, evaluation, and treatment","volume":"87","author":"DiCaprio MR","year":"2005","unstructured":"MR DiCaprio, WF Enneking, Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 87, 1848\u20131864 (2005).","journal-title":"J Bone Joint Surg Am"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000144"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.2267"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1038\/ncb3164"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1002\/gene.10092"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1038\/nm.3314"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2009.06.059"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.stemcr.2017.03.002"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gni051"},{"key":"e_1_3_3_23_2","first-page":"1587","article-title":"Fibrous dysplasia of bone in the McCune-Albright syndrome: Abnormalities in bone formation","volume":"151","author":"Riminucci M","year":"1997","unstructured":"M Riminucci, , Fibrous dysplasia of bone in the McCune-Albright syndrome: Abnormalities in bone formation. Am J Pathol 151, 1587\u20131600 (1997).","journal-title":"Am J Pathol"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3800753"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI18399"},{"key":"e_1_3_3_26_2","first-page":"155","article-title":"Natural history and treatment of fibrous dysplasia of bone: A multicenter clinicopathologic study promoted by the European Pediatric Orthopaedic Society","volume":"12","author":"Ippolito E","year":"2003","unstructured":"E Ippolito, , Natural history and treatment of fibrous dysplasia of bone: A multicenter clinicopathologic study promoted by the European Pediatric Orthopaedic Society. J Pediatr Orthop B; European Pediatric Orthopaedic Society 12, 155\u2013177 (2003).","journal-title":"J Pediatr Orthop B"},{"key":"e_1_3_3_27_2","first-page":"750","article-title":"An activating Gs alpha mutation is present in fibrous dysplasia of bone in the McCune-Albright syndrome","volume":"79","author":"Shenker A","year":"1994","unstructured":"A Shenker, LS Weinstein, DE Sweet, AM Spiegel, An activating Gs alpha mutation is present in fibrous dysplasia of bone in the McCune-Albright syndrome. J Clin Endocrinol Metab 79, 750\u2013755 (1994).","journal-title":"J Clin Endocrinol Metab"},{"key":"e_1_3_3_28_2","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.1359\/jbmr.091036","article-title":"Transfer, analysis, and reversion of the fibrous dysplasia cellular phenotype in human skeletal progenitors","volume":"25","author":"Piersanti S","year":"2010","unstructured":"S Piersanti, , Transfer, analysis, and reversion of the fibrous dysplasia cellular phenotype in human skeletal progenitors. J Bone Miner Res 25, 1103\u20131116 (2010).","journal-title":"J Bone Miner Res"},{"key":"e_1_3_3_29_2","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.2002.17.9.1667"},{"key":"e_1_3_3_30_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1114656108"},{"key":"e_1_3_3_31_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI46406"},{"key":"e_1_3_3_32_2","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.06s222"},{"key":"e_1_3_3_33_2","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.1603"},{"key":"e_1_3_3_34_2","doi-asserted-by":"publisher","DOI":"10.4158\/EP10136.CR"},{"key":"e_1_3_3_35_2","doi-asserted-by":"publisher","DOI":"10.1038\/bonekey.2012.166"},{"key":"e_1_3_3_36_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2009.08.050"},{"key":"e_1_3_3_37_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.stem.2012.02.022"},{"key":"e_1_3_3_38_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.aad3680"},{"key":"e_1_3_3_39_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0090766"},{"key":"e_1_3_3_40_2","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.2079"},{"key":"e_1_3_3_41_2","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.141"},{"key":"e_1_3_3_42_2","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.1805"},{"key":"e_1_3_3_43_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-62703-989-5_17"},{"key":"e_1_3_3_44_2","doi-asserted-by":"publisher","DOI":"10.1002\/0471142735.im22f12s102"},{"key":"e_1_3_3_45_2","doi-asserted-by":"publisher","DOI":"10.1038\/srep19296"}],"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.pnas.org\/syndication\/doi\/10.1073\/pnas.1713710115","content-type":"unspecified","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.1713710115","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,6,29]],"date-time":"2024-06-29T23:57:48Z","timestamp":1719705468000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.1713710115"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12,27]]},"references-count":45,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2018,1,16]]}},"alternative-id":["10.1073\/pnas.1713710115"],"URL":"https:\/\/doi.org\/10.1073\/pnas.1713710115","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,12,27]]},"assertion":[{"value":"2017-12-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}