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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...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Company of Biologists Ltd
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7044454/ https://www.ncbi.nlm.nih.gov/pubmed/32224495 http://dx.doi.org/10.1242/dmm.043562 |
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author | van Putten, Maaike Lloyd, Erin M. de Greef, Jessica C. Raz, Vered Willmann, Raffaella Grounds, Miranda D. |
author_facet | van Putten, Maaike Lloyd, Erin M. de Greef, Jessica C. Raz, Vered Willmann, Raffaella Grounds, Miranda D. |
author_sort | van Putten, Maaike |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-7044454 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | The Company of Biologists Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-70444542020-02-27 Mouse models for muscular dystrophies: an overview van Putten, Maaike Lloyd, Erin M. de Greef, Jessica C. Raz, Vered Willmann, Raffaella Grounds, Miranda D. Dis Model Mech Review 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. The Company of Biologists Ltd 2020-02-21 /pmc/articles/PMC7044454/ /pubmed/32224495 http://dx.doi.org/10.1242/dmm.043562 Text en © 2020. Published by The Company of Biologists Ltd http://creativecommons.org/licenses/by/4.0This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed. |
spellingShingle | Review van Putten, Maaike Lloyd, Erin M. de Greef, Jessica C. Raz, Vered Willmann, Raffaella Grounds, Miranda D. Mouse models for muscular dystrophies: an overview |
title | Mouse models for muscular dystrophies: an overview |
title_full | Mouse models for muscular dystrophies: an overview |
title_fullStr | Mouse models for muscular dystrophies: an overview |
title_full_unstemmed | Mouse models for muscular dystrophies: an overview |
title_short | Mouse models for muscular dystrophies: an overview |
title_sort | mouse models for muscular dystrophies: an overview |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7044454/ https://www.ncbi.nlm.nih.gov/pubmed/32224495 http://dx.doi.org/10.1242/dmm.043562 |
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