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Craniofacial characteristics of osteogenesis imperfecta mouse and effect of cathepsin K knockout : preliminary craniometry observations

Fosséprez, Julie
(2020)

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fosseprez_77641300_2020.pdf
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Details

Supervisors
Behets Wydemans, Catherine
Faculty
Faculté de médecine et médecine dentaire
Degree label
Master [180] en médecine
Abstract
Besides bone fragility, patients with osteogenesis imperfecta (OI) type III have typical craniofacial abnormalities. However, in the osteogenesis imperfecta mouse (oim), a validated model of OI type III, few descriptions exist about craniofacial phenotype. Treatment of OI mostly consists in bisphosphonate administration. Cathepsin-K inhibition has been shown a promising therapeutic approach for osteoporosis. Positive results were also observed in long bones of cathepsin K knocked out oim (oim/CatK-/-). In this study, we wanted to highlight by cephalometry the craniofacial characteristics of oim and Cathepsin K KO mouse, which are not detailed in the literature. Then we analyzed the craniofacial skeleton of oim/CatK-/- in order to identify the effects of Cathepsin K knockout on the oim mouse. We analyzed heads of 4 mice genotypes (Wt, oim, CatK-/-, oim/CatK-/-) euthanized at 13 weeks, using densitometric (pQCT), X-ray cephalometric and histomorphometric methods. The data showed that the craniofacial skeleton of oim mouse is frailer than the Wt one and presents dysmorphism, similar as the one observed in human with OI type III. Those abnormalities were not improved in the oim/CatK-/- group. These results suggest that oim mouse could serve as a complete model of the human OI type III, including the craniofacial skeleton. They also suggest that invalidation of Cathepsin-K has no impact on the craniofacial abnormalities of the oim model.