牙本质形成不全
成骨不全
颅面
牙周纤维
咀嚼力
医学
下颌骨(节肢动物口器)
头盖骨
上颌骨
错牙合
解剖
牙本质
闭塞
牙科
病理
生物
内科学
体外
生物化学
植物
属
精神科
作者
Hazem Eimar,Faleh Tamimi,Jean-Marc Retrouvey,Frank Rauch,Jane E. Aubin,Marc D. McKee
标识
DOI:10.1177/0022034516637045
摘要
Certain mutations in the COL1A1 and COL1A2 genes produce clinical symptoms of both osteogenesis imperfecta (OI) and Ehlers-Danlos syndrome (EDS) that include abnormal craniofacial growth, dental malocclusion, and dentinogenesis imperfecta. A mouse model ( Col1a1 Jrt /+) was recently developed that had a skeletal phenotype and other features consistent with moderate-to-severe OI and also with EDS. The craniofacial phenotype of 4- and 20-wk-old Col1a1 Jrt /+ mice and wild-type littermates was assessed by micro–computed tomography (µCT) and morphometry. Teeth and the periodontal ligament compartment were analyzed by µCT, light microscopy/histomorphometry, and electron microscopy. Over time, at 20 wk, Col1a1 Jrt /+ mice developed smaller heads, a shortened anterior cranial base, class III occlusion, and a mandibular side shift with shorter morphology in the masticatory region (maxilla and mandible). Col1a1 Jrt /+ mice also had changes in the periodontal compartment and abnormalities in the dentin matrix and mineralization. These findings validate Col1a1 Jrt /+ mice as a model for OI and EDS in humans.
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