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Interactions of mitochondrial and skeletal muscle biology in mitochondrial myopathy

Lookup NU author(s): Dr Valeria Di Leo, Dr Tiago Gomes Bernardino Gomes, Dr Amy VincentORCiD



This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


© 2023 The Author(s). Mitochondrial dysfunction in skeletal muscle fibres occurs with both healthy aging and a range of neuromuscular diseases. The impact of mitochondrial dysfunction in skeletal muscle and the way muscle fibres adapt to this dysfunction is important to understand disease mechanisms and to develop therapeutic interventions. Furthermore, interactions between mitochondrial dysfunction and skeletal muscle biology, in mitochondrial myopathy, likely have important implications for normal muscle function and physiology. In this review, we will try to give an overview of what is known to date about these interactions including metabolic remodelling, mitochondrial morphology, mitochondrial turnover, cellular processes and muscle cell structure and function. Each of these topics is at a different stage of understanding, with some being well researched and understood, and others in their infancy. Furthermore, some of what we know comes from disease models. Whilst some findings are confirmed in humans, where this is not yet the case, we must be cautious in interpreting findings in the context of human muscle and disease. Here, our goal is to discuss what is known, highlight what is unknown and give a perspective on the future direction of research in this area.

Publication metadata

Author(s): Di Leo V, Bernardino Gomes TM, Vincent AE

Publication type: Review

Publication status: Published

Journal: Biochemical Journal

Year: 2023

Volume: 480

Issue: 21

Pages: 1767-1789

Online publication date: 15/11/2023

Acceptance date: 26/10/2023

ISSN (print): 0264-6021

ISSN (electronic): 1470-8728


DOI: 10.1042/BCJ20220233

PubMed id: 37965929