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Mitochondrial Dysfunction in Mitochondrial Medicine: Current Limitations, Pitfalls, and Tomorrow

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Mitochondrial Medicine

Part of the book series: Methods in Molecular Biology ((MIMB,volume 2276))

Abstract

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.

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Acknowledgments

The authors thank the following institutions and patient associations: Université d’Angers and CHU d’Angers, Fondation Maladies Rares and UNADEV.

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Authors and Affiliations

  1. UMR CNRS 6015-INSERM U1083, MitoVasc Institute, University of Angers, Angers, France

    Naig Gueguen, Guy Lenaers & Pascal Reynier

  2. Department of Biochemistry and Genetics, University Hospital of Angers, Angers, France

    Naig Gueguen & Pascal Reynier

  3. Department of Pharmaceutical Sciences and Nanocenter of Excellence, Midwestern University College of Pharmacy at Glendale, Glendale, AZ, USA

    Volkmar Weissig

  4. Université de Paris, INSERM U1016, Institut Cochin, CNRS UMR8104, Paris, France

    Marvin Edeas

  5. Laboratory of Excellence GR-Ex, Paris, France

    Marvin Edeas

Authors
  1. Naig Gueguen
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  2. Guy Lenaers
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  3. Pascal Reynier
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  4. Volkmar Weissig
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  5. Marvin Edeas
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Corresponding author

Correspondence to Marvin Edeas .

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Editors and Affiliations

  1. Department of Pharmaceutical Sciences, Midwestern University, Glendale, AZ, USA

    Volkmar Weissig

  2. Cochin Hospital, Cochin Institute, INSERM U1016, PARIS, France

    Marvin Edeas

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Gueguen, N., Lenaers, G., Reynier, P., Weissig, V., Edeas, M. (2021). Mitochondrial Dysfunction in Mitochondrial Medicine: Current Limitations, Pitfalls, and Tomorrow. In: Weissig, V., Edeas, M. (eds) Mitochondrial Medicine . Methods in Molecular Biology, vol 2276. Springer, New York, NY. https://doi.org/10.1007/978-1-0716-1266-8_1

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  • DOI: https://doi.org/10.1007/978-1-0716-1266-8_1

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  • Online ISBN: 978-1-0716-1266-8

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