{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T19:30:44Z","timestamp":1793302244346,"version":"4.5.0"},"reference-count":37,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2013,2,14]],"date-time":"2013-02-14T00:00:00Z","timestamp":1360800000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2013,7,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>\u2003<\/jats:title>\n                  <jats:p>Osteogenesis imperfecta type VI (OI type VI) has recently be linked to a mutation in the SERPINF1 gene, which encodes pigment epithelium-derived factor (PEDF), a ubiquitously expressed protein originally described for its neurotrophic and antiangiogenic properties. In this study, we characterized the skeletal phenotype of a mouse with targeted disruption of Pedf. In normal mouse bone, Pedf was localized to osteoblasts and osteocytes. Micro\u2013computed tomography (\u00b5CT) and quantitative bone histomorphometry in femurs of mature Pedf null mutants revealed reduced trabecular bone volume and the accumulation of unmineralized bone matrix. Fourier transform infrared microscopy (FTIR) indicated an increased mineral:matrix ratio in mutant bones, which were more brittle than controls. In vitro, osteoblasts from Pedf null mice exhibited enhanced mineral deposition as assessed by Alizarin Red staining and an increased mineral:matrix determined by FTIR analysis of calcified nodules. The findings in this mouse model mimic the principal structural and biochemical features of bone observed in humans with OI type VI and consequently provide a useful model with which to further investigate the role of PEDF in this bone disorder.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1002\/jbmr.1892","type":"journal-article","created":{"date-parts":[[2013,2,14]],"date-time":"2013-02-14T16:29:29Z","timestamp":1360859369000},"page":"1531-1536","source":"Crossref","is-referenced-by-count":57,"title":["A Mouse Model for Human Osteogenesis Imperfecta Type VI"],"prefix":"10.1093","volume":"28","author":[{"given":"Rosalind","family":"Bogan","sequence":"first","affiliation":[{"name":"Department of Orthopaedic SurgeryJohns Hopkins University School of MedicineBaltimore, MDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan C","family":"Riddle","sequence":"additional","affiliation":[{"name":"Baltimore Veterans Administration Medical CenterBaltimore, MDUSA"},{"name":"Department of Orthopaedic SurgeryJohns Hopkins University School of MedicineBaltimore, MDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhu","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic SurgeryJohns Hopkins University School of MedicineBaltimore, MDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarvesh","family":"Kumar","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic SurgeryJohns Hopkins University School of MedicineBaltimore, MDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anjali","family":"Nandal","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic SurgeryJohns Hopkins University School of MedicineBaltimore, MDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Claude","family":"Faugere","sequence":"additional","affiliation":[{"name":"Department of MedicineUniversity of KentuckyLexington, KYUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adele","family":"Boskey","sequence":"additional","affiliation":[{"name":"Mineralized Tissue Research LaboratoryHospital for Special SurgeryNew YorkNYUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan E","family":"Crawford","sequence":"additional","affiliation":[{"name":"Department of PathologySt. Louis UniversitySt. Louis, MOUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas L","family":"Clemens","sequence":"additional","affiliation":[{"name":"Baltimore Veterans Administration Medical CenterBaltimore, MDUSA"},{"name":"Department of Orthopaedic SurgeryJohns Hopkins University School of MedicineBaltimore, MDUSA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2013,2,14]]},"reference":[{"key":"2024020318372524800_jbmr1892-bib-0001","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1002\/humu.20429","article-title":"Consortium for osteogenesis imperfecta mutations in the helical domain of type I collagen: regions rich in lethal mutations align with collagen binding sites for integrins and proteoglycans","volume":"28","author":"Marini","year":"2007","journal-title":"Hum Mutat."},{"key":"2024020318372524800_jbmr1892-bib-0002","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1146\/annurev.bi.64.070195.002155","article-title":"Collagens: molecular biology, diseases, and potentials for therapy","volume":"64","author":"Prockop","year":"1995","journal-title":"Annu Rev 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