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.
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SUNY Buffalo, Jacobs Sch Med & Biomed Sci, Dept Neurol, 1010 Main St, Buffalo, NY 14202 USASUNY Buffalo, Jacobs Sch Med & Biomed Sci, Dept Neurol, 1010 Main St, Buffalo, NY 14202 USA
Silvestri, Nicholas J.
Ismail, Haisam
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Harvard Med Sch, Dept Cardiol, Beth Israel Deaconess Med Ctr, Boston, MA USASUNY Buffalo, Jacobs Sch Med & Biomed Sci, Dept Neurol, 1010 Main St, Buffalo, NY 14202 USA
Ismail, Haisam
Zimetbaum, Peter
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Harvard Med Sch, Dept Cardiol, Beth Israel Deaconess Med Ctr, Boston, MA USASUNY Buffalo, Jacobs Sch Med & Biomed Sci, Dept Neurol, 1010 Main St, Buffalo, NY 14202 USA
Zimetbaum, Peter
Raynor, Elizabeth M.
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Harvard Med Sch, Dept Neurol, Beth Israel Deaconess Med Ctr, Boston, MA USASUNY Buffalo, Jacobs Sch Med & Biomed Sci, Dept Neurol, 1010 Main St, Buffalo, NY 14202 USA
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Univ Iowa, Coll Med, Howard Hughes Med Inst, Dept Physiol & Biophys & Neurol, Iowa City, IA 52242 USAUniv Iowa, Coll Med, Howard Hughes Med Inst, Dept Physiol & Biophys & Neurol, Iowa City, IA 52242 USA
Cohn, RD
Campbell, KP
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Univ Iowa, Coll Med, Howard Hughes Med Inst, Dept Physiol & Biophys & Neurol, Iowa City, IA 52242 USAUniv Iowa, Coll Med, Howard Hughes Med Inst, Dept Physiol & Biophys & Neurol, Iowa City, IA 52242 USA