Dystrophic cardiomyopathy: role of the cardiac myofilaments.
Duchenne muscular dystrophy
cardiac myocytes
length dependent activation
mavacamten
myofibrillar power
Journal
Frontiers in physiology
ISSN: 1664-042X
Titre abrégé: Front Physiol
Pays: Switzerland
ID NLM: 101549006
Informations de publication
Date de publication:
2023
2023
Historique:
received:
17
04
2023
accepted:
31
05
2023
medline:
26
6
2023
pubmed:
26
6
2023
entrez:
26
6
2023
Statut:
epublish
Résumé
Dystrophic cardiomyopathy arises from mutations in the dystrophin gene. Dystrophin forms part of the dystrophin glycoprotein complex and is postulated to act as a membrane stabilizer, protecting the sarcolemma from contraction-induced damage. Duchenne muscular dystrophy (DMD) is the most severe dystrophinopathy, caused by a total absence of dystrophin. Patients with DMD present with progressive skeletal muscle weakness and, because of treatment advances, a cardiac component of the disease (i.e., dystrophic cardiomyopathy) has been unmasked later in disease progression. The role that myofilaments play in dystrophic cardiomyopathy is largely unknown and, as such, this study aimed to address cardiac myofilament function in a mouse model of muscular dystrophy. To assess the effects of DMD on myofilament function, isolated permeabilized cardiomyocytes of wild-type (WT) littermates and Dmd
Identifiants
pubmed: 37362434
doi: 10.3389/fphys.2023.1207658
pii: 1207658
pmc: PMC10288979
doi:
Types de publication
Journal Article
Langues
eng
Pagination
1207658Informations de copyright
Copyright © 2023 George, Hanft, Krenz, Domeier and McDonald.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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