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Development of Treatments and Therapies to Target Mitochondrial Dysfunction

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Mitochondrial Dysfunction in Neurodegenerative Disorders

Abstract

Mitochondrial diseases affect 1:10,000 people worldwide, with a further 1:6,000 at risk of developing symptoms, and can be caused by mutations in the nuclear genome or by mutations within/deletion of mitochondrial DNA (mtDNA). There is currently no therapy for patients with these diseases. In addition, mitochondrial dysfunction can be considered contributory or causal for other neurological diseases, such that therapies that improve mitochondrial function may have wide-reaching benefits. This chapter summarizes cell- and animal-based attempts to find a therapy by modulating major mitochondrial pathways including mitochondrial biogenesis, fission, fusion and altering metabolic intermediates, mitophagy and the mammalian target of rapamycin (mTOR) pathway. Clinical trials currently in progress include drugs that are direct or indirect antioxidants, a mitogenesis activator, an antiapoptotic compound and a compound that improves electron transport chain efficiency.

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Acknowledgments

I would like to thank Nathalie Vancampenhout for her help with clinical trial database searches.

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  1. Development and Molecular Pathways, Novartis Institutes for BioMedical Research, Basel, Switzerland

    Stephen B. Helliwell BSc, PhD

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  1. Stephen B. Helliwell BSc, PhD
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Correspondence to Stephen B. Helliwell BSc, PhD .

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Editors and Affiliations

  1. Newcastle University Medical School, Well. Trust Ctr. for Mitochondrial Res., Newcastle upon Tyne, United Kingdom

    Amy K. Reeve

  2. Newcastle University Medical School, Well. Trust Ctr. for Mitochondrial Res., Newcastle upon Tyne, United Kingdom

    Eve M. Simcox

  3. Cell and Developmental Biology, University College London, London, United Kingdom

    Michael R. Duchen

  4. Newcastle University Medical School, Well. Trust Ctr. for Mitochondrial Res., Newcastle upon Tyne, United Kingdom

    Doug M. Turnbull

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Helliwell, S.B. (2016). Development of Treatments and Therapies to Target Mitochondrial Dysfunction. In: Reeve, A., Simcox, E., Duchen, M., Turnbull, D. (eds) Mitochondrial Dysfunction in Neurodegenerative Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-28637-2_15

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