Ryanodine receptor dysfunction causes senescence and fibrosis in Duchenne dilated cardiomyopathy.
Monia SouidiJessica RestaHaikel DridiYvonne SleimanSteve ReikenKarina FormosoSarah ColombaniPascal AmédroPierre MeyerAzzouz CharrabiMarie VincentiYang LiuRajesh Kumar SoniFrank Lezoualc'hD V M Stéphane BlotFrançois RivierOlivier CazorlaAngelo PariniAndrew R MarksJeanne Mialet-PerezAlain LacampagneAlbano C MeliPublished in: Journal of cachexia, sarcopenia and muscle (2024)
leak in DMD cardiomyocytes. We revealed that RyR2 is an early biomarker of DMD-associated cardiac damages in DMD patients. The progressive and later DCM onset could be linked with the RyR2-mediated increased fibrosis and premature senescence, eventually causing cell death and further cardiac fibrosis in a vicious cycle leading to further hypocontractility as a major feature of DCM. The present study provides a novel understanding of the pathophysiological mechanisms of the DMD-induced DCM. By targeting RyR2 channels, it provides a potential pharmacological treatment.
Keyphrases
- duchenne muscular dystrophy
- muscular dystrophy
- cell death
- end stage renal disease
- endothelial cells
- high glucose
- left ventricular
- chronic kidney disease
- dna damage
- newly diagnosed
- multiple sclerosis
- oxidative stress
- machine learning
- peritoneal dialysis
- stress induced
- liver fibrosis
- heart failure
- single cell
- patient reported outcomes
- climate change
- signaling pathway
- human health
- neural network