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Mitochondrial dynamics and quality control in Huntington's disease

Pedro Guedes-Dias1, Brígida R Pinho2, Tânia R Soares2

  • 1REQUIMTE/LAQV, Department of Drug Sciences, Faculty of Pharmacy, University of Porto, Porto, Portugal; Department of Cell and Developmental Biology, University College London, London WC1E 6BT, UK.

Neurobiology of Disease
|September 22, 2015
PubMed

Insights

Huntington's disease (HD) involves mutant huntingtin (mHtt) disrupting mitochondrial function, especially in the striatum. This review details mHtt's impact on mitochondrial dynamics and quality control, crucial for understanding HD neurodegeneration.

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington's disease (HD) is an inherited neurodegenerative disorder.
  • It stems from polyglutamine expansion mutations in the huntingtin protein.
  • Mutant huntingtin (mHtt) particularly affects medium spiny neurons in the striatum.

Purpose of the Study:

  • To review evidence linking mHtt to mitochondrial dysfunction in HD.
  • To focus on brain and neuronal data, especially striatal vulnerability.
  • To explore mHtt's effects on mitochondrial dynamics and quality control.

Main Methods:

  • Literature review of current evidence.
  • Focus on studies examining mitochondrial biogenesis, protein import, and complex assembly.
  • Analysis of data on mitochondrial fission, fusion, transport, and mitophagy.

Main Results:

  • mHtt induces abnormalities in mitochondrial dynamics and quality control.
  • Specific effects include impaired mitochondrial biogenesis, protein import, and complex assembly.
  • Dysfunctional mitochondrial transport and mitophagy are also observed.

Conclusions:

  • Mitochondrial dysfunction is a key pathogenic mechanism in HD.
  • mHtt disrupts multiple aspects of mitochondrial health and function.
  • Further research into these mechanisms may reveal therapeutic targets for HD.

Keywords:
HuntingtinHuntington's diseaseMitochondriaMitochondrial dynamicsMitophagy

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