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Animal models of osteogenesis imperfecta: applications in clinical research

Authors Enderli T, Burtch S, Templet J, Carriero A

Received 24 February 2016

Accepted for publication 30 April 2016

Published 27 September 2016 Volume 2016:8 Pages 41—55

DOI https://doi.org/10.2147/ORR.S85198

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 2

Editor who approved publication: Professor Clark Hung


Tanya A Enderli, Stephanie R Burtch, Jara N Templet, Alessandra Carriero

Department of Biomedical Engineering, Florida Institute of Technology, Melbourne, FL, USA

Abstract: 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 the phenotype and biochemistry of the 17 different types of OI in humans. These include mice, dogs, and fish. Here, we describe each of the animal models and the type of OI they represent, and present their application in clinical research for treatments of OI, such as drug therapies (ie, bisphosphonates and sclerostin) and mechanical (ie, vibrational) loading. In the future, different dosages and lengths of treatment need to be further investigated on different animal models of OI using potentially promising treatments, such as cellular and chaperone therapies. A combination of therapies may also offer a viable treatment regime to improve bone quality and reduce fragility in animals before being introduced into clinical trials for OI patients.

Keywords: OI, brittle bone, clinical research, mouse, dog, zebrafish

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