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Animal Models Of Osteogenesis Imperfecta: Applications In Clinical Research, Tanya A. Enderli, Stephanie R. Burtch, Jara N. Templet, Alessandra Carriero

Biomedical Engineering and Sciences Faculty Publications

Osteogenesis imperfecta (OI), commonly known as brittle bone disease, is a genetic disease characterized by extreme bone fragility and consequent skeletal deformities. This connective tissue disorder is caused by mutations in the quality and quantity of the collagen that in turn affect the overall mechanical integrity of the bone, increasing its vulnerability to fracture. Animal models of the disease have played a critical role in the understanding of the pathology and causes of OI and in the investigation of a broad range of clinical therapies for the disease. Currently, at least 20 animal models have been officially recognized to represent …