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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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Detalhes bibliográficos
Publicado no:Int J Mol Sci
Main Authors: Lee, Eun-Jeong, Kolb, Justin, Hwee, Darren T., Malik, Fady I., Granzier, Henk L.
Formato: Artigo
Idioma:Inglês
Publicado em: MDPI 2019
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6829460/
https://ncbi.nlm.nih.gov/pubmed/31658633
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms20205008
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