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dc.contributor.authorEnderli, Tanya A.
dc.contributor.authorBurtch, Stephanie R.
dc.contributor.authorTemplet, Jara N.
dc.contributor.authorCarriero, Alessandra
dc.date.accessioned2018-01-05T20:44:04Z
dc.date.available2018-01-05T20:44:04Z
dc.date.issued2016
dc.identifier.citationEnderli, T.A., Burtch, S.R., Templet, J.N., Carriero, A. Animal models of osteogenesis imperfecta: Applications in clinical research (2016) Orthopedic Research and Reviews, 8, pp. 41-55.en_US
dc.identifier.urihttp://hdl.handle.net/11141/2250
dc.descriptionOI, brittle bone, clinical research, mouse, dog, zebrafishen_US
dc.description.abstractOsteogenesis 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. © 2016 Enderli et al.en_US
dc.language.isoen_USen_US
dc.rights© 2016 Enderli et al. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php).en_US
dc.rights.urihttp://creativecommons.org/licenses/by-nc/3.0/en_US
dc.titleAnimal models of osteogenesis imperfecta: Applications in clinical researchen_US
dc.typeOtheren_US
dc.identifier.doi10.2147/ORR.S85198


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© 2016 Enderli et al. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php).
Except where otherwise noted, this item's license is described as © 2016 Enderli et al. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php).