Myofibrillar Lattice Remodeling Is a Structural Cytoskeletal Predictor of Diaphragm Muscle Weakness in a Fibrotic <i>mdx</i> (<i>mdx Cmah<sup>−/−</sup></i>) Model

Myofibrillar Lattice Remodeling Is a Structural Cytoskeletal Predictor of Diaphragm Muscle Weakness in a Fibrotic <i>mdx</i> (<i>mdx Cmah<sup>−/−</sup></i>) Model

Duchenne muscular dystrophy (DMD) is a degenerative genetic myopathy characterized by complete absence of dystrophin. Although the <i>mdx</i> mouse lacks dystrophin, its phenotype is milder compared to DMD patients. The incorporation of a null mutation in the <i>Cmah</i> gene...

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Bibliographic Details
Main Authors: Paul Ritter, Stefanie Nübler, Andreas Buttgereit, Lucas R. Smith, Alexander Mühlberg, Julian Bauer, Mena Michael, Lucas Kreiß, Michael Haug, Elisabeth Barton, Oliver Friedrich
Format: Article
Language:English
Published: MDPI AG 2022-09-01
Series:International Journal of Molecular Sciences
Subjects:
muscular dystrophy
skeletal muscle
multiphoton microscopy
verniers density
cosine angle sum
Online Access:https://www.mdpi.com/1422-0067/23/18/10841

Internet

https://www.mdpi.com/1422-0067/23/18/10841

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