A 12-week strength training improves mitochondrial respiration, H2O2 emission and skeletal muscle integrity in women with myotonic dystrophy type 1

Marcangeli, V., Girard-Côté, L., Di Leo, V., Roussel, M.-P., Lawless, C., Charest, O., Argaw, A., Dulac, M., Hajj-Boutros, G., Morais, J. A., Vincent, A., Gouspillou, G., Leduc-Gaudet, J.-P. et Duchesne, E. (2025). A 12-week strength training improves mitochondrial respiration, H2O2 emission and skeletal muscle integrity in women with myotonic dystrophy type 1. Acta Physiologica, 241 (12). Article e70135. ISSN 1748-1708 1748-1716 DOI 10.1111/apha.70135

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Résumé

ABSTRACT

Background
Myotonic dystrophy type 1 (DM1) is caused by expanded CTG repeats in the DMPK gene, causing the accumulation of toxic RNA that sequesters RNA-binding proteins. Clinically, DM1 is characterized by progressive muscle weakness and atrophy, resulting in reduced physical capacity and quality of life. Recent evidence implicates mitochondrial dysfunction in DM1 pathophysiology. While aerobic exercise has been shown to improve skeletal muscle and mitochondrial health in individuals with DM1, the benefits of strength training remain unexplored.

Objectives
We investigated the effects of a 12-week strength training program on mitochondrial respiration, reactive oxygen species (ROS) production and muscle integrity in women with DM1.

Methods
Vastus lateralis muscle biopsies were collected pre- and post-training in participants with DM1 and once in unaffected/untrained individuals. Mitochondrial respiration and hydrogen peroxide emission (marker of ROS production) were assessed in permeabilized myofibers, while OXPHOS protein contents were quantified by immunoblotting and immunofluorescence. Markers of myofiber denervation (NCAM+) and integrity (centrally located myonuclei, damaged laminin, nuclear clumps) were assessed on histological sections.

Results
At baseline, DM1 participants exhibited lower mitochondrial respiration compared to unaffected individuals. Strength training significantly improved mitochondrial respiration and content in DM1 participants. At baseline, absolute ROS production was lower, while ROS production normalized to oxygen consumption (free radical leak) was higher, in DM1. Histological signs of denervation and altered muscle integrity were observed. Strength training partially normalized mitochondrial free radical leak and restored some markers of myofiber integrity.

Conclusion
Collectively, our results indicate that strength training enhances mitochondrial health and improves myofiber integrity in women with DM1.

Type de document:	Article
Mots-clés libres:	Exercise Mitochondria Mitochondrial function Mitochondrial respiration Myotonic dystrophy Neuromuscular disease Oxidative Phosphorylation defects Resistance training ROS
Date de dépôt:	29 mars 2026 14:34
Dernière modification:	29 mars 2026 14:34
Version du document déposé:	Version officielle de l'éditeur
URI:	https://depot-e.uqtr.ca/id/eprint/12762

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