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.

中文翻译：

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线粒体肌病中线粒体和骨骼肌生物学的相互作用

骨骼肌纤维的线粒体功能障碍与健康衰老和一系列神经肌肉疾病有关。骨骼肌线粒体功能障碍的影响以及肌纤维适应这种功能障碍的方式对于了解疾病机制和制定治疗干预措施非常重要。此外，在线粒体肌病中，线粒体功能障碍和骨骼肌生物学之间的相互作用可能对正常肌肉功能和生理学具有重要影响。在这篇综述中，我们将尝试概述迄今为止已知的这些相互作用，包括代谢重塑、线粒体形态、线粒体周转、细胞过程以及肌肉细胞结构和功能。每个主题都处于不同的理解阶段，其中一些已得到充分研究和理解，而另一些则处于起步阶段。此外，我们所知道的一些知识来自疾病模型。虽然一些发现在人类身上得到了证实，但情况尚未如此，但我们在解释人类肌肉和疾病背景下的发现时必须谨慎。在这里，我们的目标是讨论已知的内容，强调未知的内容，并对该领域的未来研究方向提出展望。