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L’homotransplantation de muscles rapides et lents est bien tolérée dans les deux souches de souris, les muscles transplantés présentant une régénération fonctionnelle.
Les différences mécaniques et biochimiques entre souris mdx et c57, à savoir que le muscle mdx est plus lent que le muscle c57, sont conservées pour les muscles transplantés.
La transplantation modifie le muscle de façon durable. Nos muscles transplantés pouvant être considérées comme stabilisées après 60 jours de régénération. Le muscle transplanté est nettement plus rapide que le muscle normal et contient un mélange de myosine lente et rapide.
Une hypothèse fréquemment avancée pour interpréter l’évolution de la DMD, est l’apparition de cycles de dégénération-régénération qui seraient responsables de l’évolution fatale de la maladie.
or, si les fibres apparaissant dans le muscle mdx sont des fibres résultant d’un processus de régénération normal, on peut s’attendre à ce que ces fibres présentent les mêmes caractéristiques que les fibres des muscles transplantés. On constate cependant que la transplantation provoque une augmentation de vitesse du muscle alors que le muscle mdx reste toujours plus lent que le muscle normal. On ne peut dons assimiler le muscle DMD purement et simplement à un muscle en dégénération-régénération.
Le muscle dystrophique est morphologiquement semblable au muscle normal et développe une force équivalente : l’absence de dystrophine ne peut donc pas être par elle-même responsable de l’asthénie musculaire caractéristique de la DMD

Mice --- Muscular Distrophy, Duchenne

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Electromyography --- Electromyographie --- ELECTROMYOGRAPHY --- MUSCLE, SKELETAL --- NEUROMUSCULAR JUNCTION --- NEUROMUSCULAR DISEASES --- MUSCULAR DISTROPHY --- MYOTONIA --- MUSCULAR DISEASES --- METHODS --- PHYSIOLOGY

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Dilated cardiomyopathy (DCM) is a particular phenotype of non-ischemic systolic heart failure, frequently recognizing a genetic background and affecting relatively young patients with few comorbidities. Nowadays, long-term survival of DCM patients has been markedly improved due to an early diagnosis and uninterrupted and tailored follow-up under constant optimal medical and non-pharmacological evidence-based treatments. Nevertheless, DCM is still one of the most common causes of heart transplantation in the western world. Clinical management requires an integrated and systematic use of diagnostic tools and a deeper investigation of the basic mechanisms underlying the disease. However, several emerging issues remain debated. Specifically, the genotype–phenotype correlation, the role of advanced imaging techniques and genetic testing, the lack of appropriate risk stratification models, the need for a multiparametric and multidisciplinary approach for device implantation, and a continuous reclassification of the disease during follow-up remain challenging issues in clinical practice. Therefore, the aim of this Special Issue is to shed the light on the most recent advancements in characterization and clinical management of DCM in order to unveil the conundrum of this particular disease.

Medicine --- SCN5A --- cardiac sodium channel --- cardiac channelopathy --- dilated cardiomyopathy --- precision medicine --- arrhythmias --- atrial fibrillation --- cardiomyopathy --- heart failure --- supraventricular arrhythmia --- systolic dysfunction --- tachycardiomyopathy --- ventricular arrhythmia --- left atrial strain --- cardiac resynchronization therapy --- muscular dystrophy --- calcium --- heart --- gene therapy --- phospholamban --- Serca2a --- mdx --- oxidative stress --- membrane stabilization --- left ventricular noncompaction --- congenital heart disease --- congestive heart failure --- non-ischemic cardiomyopathy --- genetics --- desmin --- mitochondrial dysfunction --- myopathy --- whole exome sequencing --- laminopathy --- LMNA --- biomarkers --- troponin T --- NT-proBNP --- malignant ventricular arrhythmia --- arrhythmic risk stratification --- DNA methylation --- alternative splicing --- epigenetics --- nonischemic dilated cardiomyopathy --- cardiac magnetic resonance imaging --- late gadolinium enhancement --- long axis strain --- left ventricle sphericity index --- major adverse cardiovascular events --- sex differences --- left ventricular reverse remodelling --- long-term outcomes --- left ventricle non-compaction cardiomyopathy --- cardiac magnetic resonance --- titin --- RNA binding motif protein 20 (RBM20) --- sarcomere --- diastolic dysfunction --- phosphorylation --- non-sense mRNA decay --- mammalian target of rapamycin (mTOR) complex-1 --- duchenne muscular distrophy --- SCN5A --- cardiac sodium channel --- cardiac channelopathy --- dilated cardiomyopathy --- precision medicine --- arrhythmias --- atrial fibrillation --- cardiomyopathy --- heart failure --- supraventricular arrhythmia --- systolic dysfunction --- tachycardiomyopathy --- ventricular arrhythmia --- left atrial strain --- cardiac resynchronization therapy --- muscular dystrophy --- calcium --- heart --- gene therapy --- phospholamban --- Serca2a --- mdx --- oxidative stress --- membrane stabilization --- left ventricular noncompaction --- congenital heart disease --- congestive heart failure --- non-ischemic cardiomyopathy --- genetics --- desmin --- mitochondrial dysfunction --- myopathy --- whole exome sequencing --- laminopathy --- LMNA --- biomarkers --- troponin T --- NT-proBNP --- malignant ventricular arrhythmia --- arrhythmic risk stratification --- DNA methylation --- alternative splicing --- epigenetics --- nonischemic dilated cardiomyopathy --- cardiac magnetic resonance imaging --- late gadolinium enhancement --- long axis strain --- left ventricle sphericity index --- major adverse cardiovascular events --- sex differences --- left ventricular reverse remodelling --- long-term outcomes --- left ventricle non-compaction cardiomyopathy --- cardiac magnetic resonance --- titin --- RNA binding motif protein 20 (RBM20) --- sarcomere --- diastolic dysfunction --- phosphorylation --- non-sense mRNA decay --- mammalian target of rapamycin (mTOR) complex-1 --- duchenne muscular distrophy