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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2021,6,22]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Osteogenesis imperfecta (OI), also known as brittle bone disease, commonly features low bone mass and frequent fractures. We and others have previously identified mutations that cause joint deformities in OI patients. However, mechanisms responsible for joint pathologies in OI are poorly understood. Here, we have established a mouse model of OI joint dysfunction that is caused by mutations in\n                    <jats:italic>Fkbp10<\/jats:italic>\n                    . Our mouse model recapitulates key features of\n                    <jats:italic>FKBP10<\/jats:italic>\n                    OI patients, including reduced collagen cross-linking and joint deformities. In addition, we found that inflammation and altered matrix signaling is associated with joint abnormalities in\n                    <jats:italic>Fkbp10<\/jats:italic>\n                    mutant animals. Overall, our work provides a valuable model that can be used to identify novel therapies to treat joint dysfunction in OI and OI-related diseases.\n                  <\/jats:p>","DOI":"10.1073\/pnas.2100690118","type":"journal-article","created":{"date-parts":[[2021,6,14]],"date-time":"2021-06-14T16:47:22Z","timestamp":1623689242000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":18,"title":["Localized chondro-ossification underlies joint dysfunction and motor deficits in the\n                    <i>Fkbp10<\/i>\n                    mouse model of osteogenesis imperfecta"],"prefix":"10.1073","volume":"118","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9670-806X","authenticated-orcid":false,"given":"Joohyun","family":"Lim","sequence":"first","affiliation":[{"name":"Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 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