Open AccessMouse models for muscular dystrophies: an overview
Author(s) -
Maaike van Putten,
Erin M. Lloyd,
Jessica C. de Greef,
Vered Raz,
Raffaella Willmann,
Miranda D. Grounds
Publication year - 2020
Publication title -
disease models and mechanisms
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.327
H-Index - 83
eISSN - 1754-8411
pISSN - 1754-8403
DOI - 10.1242/dmm.043562
Subject(s) - variety (cybernetics) , biology , computational biology , function (biology) , neuroscience , animal model , bioinformatics , computer science , genetics , artificial intelligence , endocrinology
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.