Heterozygous oim mice exhibit a mild form of osteogenesis imperfecta

• Published in: Bone (New York, N.Y.) Vol. 19; no. 6; pp. 575 - 579
• Main Authors: Saban, J., Zussman, M.A., Havey, R., Patwardhan, A.G., Schneider, G.B., King, D.
• Format: Journal Article Conference Proceeding
• Language: English
• Published: New York, NY Elsevier Inc 01.12.1996; Elsevier Science
• Subjects: Animals; Biological and medical sciences; Biomechanical Phenomena; Biomechanics; Bone and Bones - injuries; Bone and Bones - physiopathology; Bones; Diaphyses - pathology; Disease Models, Animal; Diseases of the osteoarticular system; Female; Femoral Fractures - genetics; Femoral Fractures - physiopathology; Heterozygote; Male; Malformations and congenital and or hereditary diseases involving bones. Joint deformations; Medical sciences; Mice; Mice, Mutant Strains; oim mice; Osteogenesis imperfecta; Osteogenesis Imperfecta - etiology; Osteogenesis Imperfecta - genetics; Phenotype; Tibia - pathology; Biomechanics; Osteogenesis imperfecta; Bones; oim mice; Heterozygozity; Animal model; Procollagen; Pathophysiology; Rodentia; Diseases of the osteoarticular system; Mechanical properties; Histology; Genetic disease; Pathology; Vertebrata; Phenotype; Mammalia; Gene; Mouse; Collagen; Mutation; Bone; Osteochondrodysplasia
• ISSN: 8756-3282; 1873-2763
• DOI: 10.1016/S8756-3282(96)00305-5

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 α-2 chain of type I procollagen that prevents proper assembly of this propeptide with α-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.