Why the study?
Does normalization of Naxos plakoglobin levels restore cardiac function in mouse models of Naxos disease?
Population
2 knockin mouse models in which endogenous Jup was engineered to express the Naxos-associated form of…
Design
Preclinical
Authors
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Plakoglobin restoration may inform therapeutic strategies in Naxos models; leaves open translation to human arrhythmogenic cardiomyopathy.
Does normalization of Naxos plakoglobin levels restore cardiac function in mouse models of Naxos disease?
Insufficiency of truncated Naxos plakoglobin, rather than a gain of function, accounts for Naxos disease manifestation, suggesting that increasing plakoglobin levels could be a therapeutic approach.
Zhang et al. (2015) studied this question.
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