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Lookup NU author(s): Dr Valeria Di Leo, Dr Conor LawlessORCiD, Dr Amy VincentORCiD
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© 2025 The Author(s). Acta Physiologica published by John Wiley & Sons Ltd on behalf of Scandinavian Physiological Society.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.
Author(s): Marcangeli V, Girard-Cote L, Di Leo V, Roussel M-P, Lawless C, Charest O, Argaw A, Dulac M, Hajj-Boutros G, Morais JA, Vincent A, Gouspillou G, Leduc-Gaudet J-P, Duchesne E
Publication type: Article
Publication status: Published
Journal: Acta Physiologica
Year: 2025
Volume: 241
Issue: 12
Online publication date: 16/11/2025
Acceptance date: 10/10/2025
Date deposited: 01/12/2025
ISSN (print): 1748-1708
ISSN (electronic): 1748-1716
Publisher: John Wiley and Sons Inc
URL: https://doi.org/10.1111/apha.70135
DOI: 10.1111/apha.70135
Data Access Statement: The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.
PubMed id: 41241935
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