Mouse models for muscular dystrophies: an overview

Muscular dystrophies (MDs) encompass a wide variety of inherited disorders that are characterized by loss of muscle tissue associated with a progressive reduction in muscle function. With a cure lacking for MDs, preclinical developments of therapeutic approaches depend on well-characterized animal m...

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Bibliographic Details
Main Authors: Maaike van Putten (Author), Erin M. Lloyd (Author), Jessica C. de Greef (Author), Vered Raz (Author), Raffaella Willmann (Author), Miranda D. Grounds (Author), Annemieke Aartsma-Rus (Author), James Dowling (Author)
Format: Book
Published: The Company of Biologists, 2020-02-01T00:00:00Z.
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Summary:Muscular dystrophies (MDs) encompass a wide variety of inherited disorders that are characterized by loss of muscle tissue associated with a progressive reduction in muscle function. With a cure lacking for MDs, preclinical developments of therapeutic approaches depend on well-characterized animal models that recapitulate the specific pathology in patients. The mouse is the most widely and extensively used model for MDs, and it has played a key role in our understanding of the molecular mechanisms underlying MD pathogenesis. This has enabled the development of therapeutic strategies. Owing to advancements in genetic engineering, a wide variety of mouse models are available for the majority of MDs. Here, we summarize the characteristics of the most commonly used mouse models for a subset of highly studied MDs, collated into a table. Together with references to key publications describing these models, this brief but detailed overview would be useful for those interested in, or working with, mouse models of MD.
Item Description:1754-8403
1754-8411
10.1242/dmm.043562