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An Inducible Facioscapulohumeral muscular dystrophy (FSHD) Mouse Model Expressing DUX4


Detailed Technology Description

FSHD is the thirdmost common muscular dystrophy, affecting 1 in 20,000 individuals. There is nocurrent treatment for FSHD. Researchers at Nationwide Children's Hospital havedeveloped a mouse model that recapitulates FSHD phenotypes and developsmyopathy. This is the first FSHD mouse model that stably expresses the DUX4gene from the mouse genome using the human DUX4 promoter. This model alsocircumvents lethality, leakiness problems and transient expression inherent inthe standard mouse model.


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Country/Region

USA

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