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Huntington’s Disease pp 415–442Cite as

Assessing Mitochondrial Function in In Vitro and Ex Vivo Models of Huntington’s Disease

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Part of the book series: Methods in Molecular Biology ((MIMB,volume 1780))

Abstract

Mitochondrial dysfunction has gained a preponderant role in the pathogenesis of Huntington’s disease (HD). Mutant huntingtin (mHTT) directly interacts with mitochondria in a deleterious manner. As the central hub of the cell, not only mitochondrial bioenergetics is affected but there is also diminished mitochondrial membrane potential (Δψ m) and altered production of reactive oxygen species (ROS). Restoration of mitochondrial function has proven to be a major player in the search and establishment of therapeutics for HD patients. As such, performing an overall study of mitochondrial function is crucial. In this chapter, we describe some methodologies used to study mitochondrial function by determining the oxygen consumption, changes in Δψ m, mitochondrial calcium handling, and levels of mitochondrial ROS. Here we focus on biological samples derived from HD versus control cells and/or animal models, namely functional isolated brain mitochondria, an ex vivo animal model, and cultured cells, including cell lines and primary neural cultures, as in vitro models.

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Acknowledgments

This work was supported by Mantero Belard Neuroscience Prize 2013 (first edition), funded by Santa Casa da Misericórdia de Lisboa (SCML); FLAD Life Science 2020 project, FEDER through “Programa Operacional Factores de Competitividade–COMPETE” and Fundação para a Ciência e a Tecnologia—FCT (UID/NEU/04539/2013; PEst-C/SAU/LA0001/2013-2014); I.L.F. and S.I.M. were supported by the FCT postdoctoral fellowships SFRH/BPD/108493/2015 and SFRH/BPD/99219/2013, respectively; and L.N. was supported by PhD fellowship SFRH/BD/86655/2012.

Author information

Authors and Affiliations

  1. CNC—Center for Neuroscience and Cell Biology, University of Coimbra—polo I, Coimbra, Portugal

    I. Luísa Ferreira, Catarina Carmo, Luana Naia, Sandra I. Mota & A. Cristina Rego

  2. IIIUC—Institute for Interdisciplinary Research, University of Coimbra—polo II, Coimbra, Portugal

    I. Luísa Ferreira, Luana Naia, Sandra I. Mota & A. Cristina Rego

  3. FMUC-Faculty of Medicine, University of Coimbra—polo III, Coimbra, Portugal

    A. Cristina Rego

Authors
  1. I. Luísa Ferreira
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  2. Catarina Carmo
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  3. Luana Naia
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  4. Sandra I. Mota
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  5. A. Cristina Rego
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Editor information

Editors and Affiliations

  1. Brain Repair Group, Cardiff University, Cardiff, United Kingdom

    Sophie V. Precious

  2. Brain Repair Group, Cardiff University, Cardiff, United Kingdom

    Anne E. Rosser

  3. Brain Repair Group, Cardiff University, Cardiff, United Kingdom

    Stephen B. Dunnett

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Ferreira, I.L., Carmo, C., Naia, L., I. Mota, S., Cristina Rego, A. (2018). Assessing Mitochondrial Function in In Vitro and Ex Vivo Models of Huntington’s Disease. In: Precious, S., Rosser, A., Dunnett, S. (eds) Huntington’s Disease. Methods in Molecular Biology, vol 1780. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-7825-0_19

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  • DOI: https://doi.org/10.1007/978-1-4939-7825-0_19

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  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4939-7824-3

  • Online ISBN: 978-1-4939-7825-0

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