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

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

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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Bibliographic Details
Main Authors: Eun-Jeong Lee, Justin Kolb, Darren T. Hwee, Fady I. Malik, Henk L. Granzier
Format: Article
Language:English
Published: MDPI AG 2019-10-01
Series:International Journal of Molecular Sciences
Subjects:
muscle contraction
isometric force
shortening velocity
fast skeletal muscle troponin activators
diaphragm muscle
nebulin
nemaline myopathy
Online Access:https://www.mdpi.com/1422-0067/20/20/5008

Internet

https://www.mdpi.com/1422-0067/20/20/5008

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