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Dental and craniofacial defects in the Crtap−/− mouse model of osteogenesis imperfecta type VII
dc.contributor.authorXu, He
dc.contributor.authorLenhart, Sydney A.
dc.contributor.authorChu, Emily Y.
dc.contributor.authorChavez, Michael B.
dc.contributor.authorWimer, Helen F.
dc.contributor.authorDimori, Milena
dc.contributor.authorSomerman, Martha J.
dc.contributor.authorMorello, Roy
dc.contributor.authorFoster, Brian L.
dc.contributor.authorHatch, Nan E.
dc.date.accessioned2020-07-02T20:32:10Z
dc.date.availableWITHHELD_13_MONTHS
dc.date.available2020-07-02T20:32:10Z
dc.date.issued2020-07
dc.identifier.citationXu, He; Lenhart, Sydney A.; Chu, Emily Y.; Chavez, Michael B.; Wimer, Helen F.; Dimori, Milena; Somerman, Martha J.; Morello, Roy; Foster, Brian L.; Hatch, Nan E. (2020). "Dental and craniofacial defects in the Crtap−/− mouse model of osteogenesis imperfecta type VII." Developmental Dynamics 249(7): 884-897.
dc.identifier.issn1058-8388
dc.identifier.issn1097-0177
dc.identifier.urihttps://hdl.handle.net/2027.42/155878
dc.description.abstractBackgroundInactivating mutations in the gene for cartilage‐associated protein (CRTAP) cause osteogenesis imperfecta type VII in humans, with a phenotype that can include craniofacial defects. Dental and craniofacial manifestations have not been a focus of case reports to date. We analyzed the craniofacial and dental phenotype of Crtap−/− mice by skull measurements, micro‐computed tomography (micro‐CT), histology, and immunohistochemistry.ResultsCrtap−/− mice exhibited a brachycephalic skull shape with fusion of the nasofrontal suture and facial bones, resulting in mid‐face retrusion and a class III dental malocclusion. Loss of CRTAP also resulted in decreased dentin volume and decreased cellular cementum volume, though acellular cementum thickness was increased. Periodontal dysfunction was revealed by decreased alveolar bone volume and mineral density, increased periodontal ligament (PDL) space, ectopic calcification within the PDL, bone‐tooth ankylosis, altered immunostaining of extracellular matrix proteins in bone and PDL, increased pSMAD5, and more numerous osteoclasts on alveolar bone surfaces.ConclusionsCrtap−/− mice serve as a useful model of the dental and craniofacial abnormalities seen in individuals with osteogenesis imperfecta type VII.
dc.publisherJohn Wiley & Sons, Inc.
dc.subject.otherphenotype
dc.subject.otherskull
dc.subject.otherbone
dc.subject.otherdental
dc.subject.othermicro computed tomography
dc.subject.otherosteogenesis imperfecta
dc.subject.othercraniofacial
dc.titleDental and craniofacial defects in the Crtap−/− mouse model of osteogenesis imperfecta type VII
dc.typeArticle
dc.rights.robotsIndexNoFollow
dc.subject.hlbsecondlevelPediatrics
dc.subject.hlbtoplevelHealth Sciences
dc.description.peerreviewedPeer Reviewed
dc.description.bitstreamurlhttps://deepblue.lib.umich.edu/bitstream/2027.42/155878/1/dvdy166.pdf
dc.description.bitstreamurlhttps://deepblue.lib.umich.edu/bitstream/2027.42/155878/2/dvdy166_am.pdf
dc.identifier.doi10.1002/dvdy.166
dc.identifier.sourceDevelopmental Dynamics
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dc.owningcollnameInterdisciplinary and Peer-Reviewed


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