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Functional Characterization of the Intact Diaphragm in a Nebulin-Based Nemaline Myopathy (NM) Model-Effects of the Fast Skeletal Muscle Troponin Activator tirasemtiv

Respiratory failure due to diaphragm dysfunction is considered a main cause of death in nemaline myopathy (NM) and we studied both isometric force and isotonic shortening of diaphragm muscle in a mouse model of nebulin-based NM (Neb cKO). A large contractile deficit was found in nebulin-deficient in...

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Detalles Bibliográficos
Autores principales:	Lee, Eun-Jeong, Kolb, Justin, Hwee, Darren T., Malik, Fady I., Granzier, Henk L.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2019
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6829460/ https://www.ncbi.nlm.nih.gov/pubmed/31658633 http://dx.doi.org/10.3390/ijms20205008

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