Effect of C-molecule treatment on the craniofacial characteristics in a murine model of severe osteogenesis imperfecta

Details

Supervisors

Chretien, Antoine ; Behets Wydemans, Catherine

Faculty

Faculté des sciences de la motricité

Degree label

Master [120] en sciences de la motricité, orientation générale, à finalité approfondie

Abstract

Osteogenesis imperfecta is a rare genetic disease of connective tissue caused by a mutation of the collagen-type I gene. Its main manifestations include bone fragility, reduction of bone mass, deformities, bluish sclera, dentinogenesis imperfecta, ligament and skin hyperlaxity, or hearing disorders. This study aims to analyze the craniofacial characteristics and the effect of Substance C on the skull bone of homozygous Oim mice, a validated model of severe OI. Mice received Substance C in their drinking water from week 5 to week 17 at a 10g/L dose. The head were analyzed via computed tomography. Results show a significant reduction on cephalometry measurements and bone parameters in the OIC group, as compared to the WT one. This research also highlighted the importance of scaled measurements. In conclusion, many craniofacial features are affected by OI, and molecule C improved only some of them. In the future, it would be interesting to continue the analyze on 3D images.