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Disease Models & Mechanisms
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Disease Models & Mechanisms
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Disease Models & Mechanisms
Other literature type . 2018
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Disease Models & Mechanisms
Article . 2018
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Tracking progress: an update on animal models for Duchenne muscular dystrophy

Authors: Dominic J. Wells;

Tracking progress: an update on animal models for Duchenne muscular dystrophy

Abstract

ABSTRACT Duchenne muscular dystrophy (DMD) is a progressive, fatal, X-linked monogenic muscle disorder caused by mutations in the DMD gene. In order to test treatments for DMD, a range of natural and engineered animal models have been developed, including mice, rats, dogs and pigs. Sui and colleagues have now added a dystrophic rabbit model to this range using CRISPR/Cas9 to disrupt exon 51 of DMD. Rabbits have the advantage of being easier to breed and less costly than dog or pig models, but having clear clinical signs, in contrast to many mouse models. There appears to be an effect of body size in models of DMD, as the severity of the clinical signs increases with increasing body size across species. All DMD models have advantages and disadvantages, and it is crucial that investigators understand the limitations of each model when testing novel therapies for DMD in pre-clinical studies.

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Keywords

Duchenne muscular dystrophy, Dog models, R, Pig models, Mouse models, Muscular Dystrophy, Duchenne, Translational Research, Biomedical, Disease Models, Animal, Editorial, Mutation, Pathology, Medicine, RB1-214, Animals, Rabbit models, CRISPR-Cas Systems

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
44
Top 10%
Top 10%
Top 1%
Green
gold