Mouse models for muscular dystrophies: an overview

被引:32
|
作者
van Putten, Maaike [1 ]
Lloyd, Erin M. [2 ]
de Greef, Jessica C. [1 ]
Raz, Vered [1 ]
Willmann, Raffaella [3 ]
Grounds, Miranda D. [2 ]
机构
[1] Leiden Univ, Med Ctr, Dept Human Genet, NL-2333 ZA Leiden, Netherlands
[2] Univ Western Australia, Sch Human Sci, Perth, WA 6009, Australia
[3] Univ Basel, Biozentrum, CH-4056 Basel, Switzerland
关键词
Disease pathology; Mouse models; Muscular dystrophy; LAMININ ALPHA-2; ANIMAL-MODELS; TARGETED DISRUPTION; MUSCLE MEMBRANE; GENE; MICE; MDX; PATHOLOGY; IDENTIFICATION; DEGENERATION;
D O I
10.1242/dmm.043562
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
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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页数:11
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