Craniofacial allometry in the OIM−/− mouse model of osteogenesis imperfecta. Issue 8 (27th June 2020)

• Record Type: Journal Article
• Authors: Menegaz, Rachel A.; Ladd, Summer H.; Organ, Jason M.
• Is Part Of: FASEB journal. Volume 34:Issue 8(2020)
• Page Start: 10850
• Page End: 10859
• Publication Date: 2020-06-27
• Subjects: 3D geometric morphometrics -- brittle bone disease -- malocclusions -- midface hypoplasia -- scaling -- type I collagen; Biology -- Periodicals; Biology, Experimental -- Periodicals; 570
• DOI: 10.1096/fj.202000715R
• Languages: English
• ISSNs: 0892-6638

Abstract

Abstract: Osteogenesis imperfecta (OI) is a skeletal disorder characterized by the impaired synthesis of type I collagen (Col1). This study tests the hypothesis that the craniofacial phenotype of severe OI is linked to an overall reduction in body size. 3D landmark data were collected from µCT scans of adult OIM −/− and wild‐type (WT) mice and used to calculate centroid sizes (CS) and interlandmark distances (ILDs). To remove the effect of body size, ILDs were scaled against craniomandibular lengths and CS. Mann‐Whitney U tests were used to compare CS and absolute/scaled ILDs between genotypes. OIM −/− mice are smaller than their WT littermates in body mass, craniomandibular CS, and absolute ILDs including skull, basicranial, palatal, mandibular, and toothrow lengths. When linear distances are scaled to CS, OIM −/− mice have a relatively short midface, short nasal bones, tall mandibular corpora, and long mandibular toothrows. Results underscore the importance of size and scaling in morphometric analyses. The deleterious effect of Col1 mutations on global skeletal dimensions combined with localized morphometric changes may underlie the facial phenotype seen in human patients with severe OI. Attempts to identify these localized changes should first account for the restricted growth and small body sizes present in individuals with OI.