Heterozygous oim mice exhibit a mild form of osteogenesis imperfecta.
The oim strain of mice is one of several rodent models that exhibit an osteogenesis imperfecta (OI) phenotype. These mice have a mutation in the gene encoding alpha-2 chain of type I procollagen that prevents proper assembly of this propeptide with alpha-1 propeptides. Homozygous oim mice experience multiple bone fractures under standard laboratory animal housing conditions and are representative of moderate to severe forms of OI. Because fractures are not typically experienced by heterozygous oim mice, they have not been studied extensively. The present studies show that the organization of cortical bone is deficient in heterozygotes, exhibiting a morphology intermediate to specimens from homozygotes and wild-type mice. The biomechanical properties of femurs isolated from heterozygous oim mice are also intermediate to homozygotes and wild-type mice when tested in four-point bending. Although it is not possible to distinguish visually between heterozygous oim and wild-type mice, the quality and biomechanical properties of bone in heterozygotes is significantly reduced by twelve weeks of age. Heterozygous oim mice are useful as a model for a mild form of OI.
Saban
J
J
Department of Pharmacology and Molecular Biology, Finch University of Health Sciences/The Chicago Medical School, North Chicago, IL 60064-3095, USA.
Zussman
M A
MA
Havey
R
R
Patwardhan
A G
AG
Schneider
G B
GB
King
D
D
D000818:Animals; D001696:Biomechanical Phenomena; D001842:Bone and Bones / Q000293:injuries* / Q000503:physiopathology; D018483:Diaphyses / Q000473:pathology; D004195:Disease Models, Animal; D005260:Female; D005264:Femoral Fractures / Q000235:genetics / Q000503:physiopathology; D006579:Heterozygote; D008297:Male; D051379:Mice; D008817:Mice, Mutant Strains; D010013:Osteogenesis Imperfecta / Q000209:etiology* / Q000235:genetics; D010641:Phenotype; D013977:Tibia / Q000473:pathology