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

  • I. Luísa Ferreira
  • Catarina Carmo
  • Luana Naia
  • Sandra I. Mota
  • A. Cristina Rego
Part of the Methods in Molecular Biology book series (MIMB, volume 1780)


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 (Δψm) 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 Δψm, 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.


Mitochondria Cells Neurons Mitochondrial function Oxygen consumption Bioenergetics Mitochondrial membrane potential Mitochondrial calcium handling Mitochondrial reactive oxygen species 



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.


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© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • I. Luísa Ferreira
    • 1
    • 2
  • Catarina Carmo
    • 1
  • Luana Naia
    • 1
    • 2
  • Sandra I. Mota
    • 1
    • 2
  • A. Cristina Rego
    • 1
    • 2
    • 3
  1. 1.CNC—Center for Neuroscience and Cell Biology, University of Coimbra—polo ICoimbraPortugal
  2. 2.IIIUC—Institute for Interdisciplinary Research, University of Coimbra—polo IICoimbraPortugal
  3. 3.FMUC-Faculty of Medicine, University of Coimbra—polo IIICoimbraPortugal

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