UMR CNRS 6015-INSERM U1083, MitoVasc Institute, University of Angers, Angers, France.
Department of Biochemistry and Genetics, University Hospital of Angers, Angers, France.
Methods Mol Biol. 2021;2276:1-29. doi: 10.1007/978-1-0716-1266-8_1.
Until recently restricted to hereditary mitochondrial diseases, mitochondrial dysfunction is now recognized as a key player and strategic factor in the pathophysiological of many human diseases, ranging from the metabolism, vascular, cardiac, and neurodegenerative diseases to cancer. Because of their participation in a myriad of cellular functions and signaling pathways, precisely identifying the cause of mitochondrial "dysfunctions" can be challenging and requires robust and controlled techniques. Initially limited to the analysis of the respiratory chain functioning, these analytical techniques now enlarge to the analyses of mitochondrial and cellular metabolism, based on metabolomic approaches.Here, we address the methods used to assay mitochondrial dysfunction, with a highlight on the techniques used in diagnosis on tissues and cells derived from patients, the information they provide, and their strength and weakness.Targeting mitochondrial dysfunction by various strategies is a huge challenge, requires robust methods of evaluation, and should be able to take into consideration the mitochondria dynamics and localization. The future of mitochondrial medicine is strongly related to a perfect comprehension of its dysfunction.
直到最近，线粒体功能障碍还仅限于遗传性线粒体疾病，但现在已被认为是许多人类疾病（从代谢、血管、心脏和神经退行性疾病到癌症）病理生理学的关键参与者和战略因素。由于它们参与了无数的细胞功能和信号通路，因此准确识别线粒体“功能障碍”的原因可能具有挑战性，并且需要强大且受控的技术。这些分析技术最初仅限于呼吸链功能的分析，现在根据代谢组学方法扩展到线粒体和细胞代谢的分析。在这里，我们介绍了用于测定线粒体功能障碍的方法，重点介绍了用于分析来自患者的组织和细胞的技术、它们提供的信息以及它们的优缺点。通过各种策略靶向线粒体功能障碍是一个巨大的挑战，需要强大的评估方法，并能够考虑到线粒体的动态和定位。线粒体医学的未来与对其功能障碍的完美理解密切相关。
