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Mitochondrial fragmentation in neuronal degeneration: Toward an understanding of HD striatal susceptibility

Marta Cherubini1, Silvia Ginés1

  • 1Departament de Biomedicina, Facultat de Medicina, Universitat de Barcelona, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Spain; Institut de Neurociències, Universitat de Barcelona, Barcelona, Spain.

Biochemical and Biophysical Research Communications
|August 13, 2016
PubMed

Insights

Huntington's disease (HD) involves a toxic huntingtin protein. Altered mitochondrial dynamics contribute to striatal neuron vulnerability in HD, suggesting mitochondrial function modulation as a therapeutic strategy.

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington's disease (HD) is a progressive neurodegenerative disorder.
  • HD is caused by an expanded CAG repeat in the HTT gene, leading to mutant huntingtin (mHtt) protein.
  • The selective vulnerability of the striatum in HD remains incompletely understood.

Purpose of the Study:

  • To review current knowledge linking mitochondrial dynamics to striatal susceptibility in HD.
  • To explore the potential of modulating mitochondrial function as a therapeutic strategy for HD.

Main Methods:

  • Literature review of studies on HD pathogenesis and mitochondrial biology.
  • Analysis of molecular mechanisms underlying striatal neurodegeneration in HD.

Main Results:

  • Altered mitochondrial dynamics are implicated in the preferential vulnerability of striatal neurons in HD.
  • Mutant huntingtin (mHtt) protein toxicity is associated with mitochondrial dysfunction.

Conclusions:

  • Mitochondrial dysfunction plays a key role in the selective neurodegeneration observed in HD.
  • Targeting mitochondrial function presents a promising therapeutic avenue for mitigating mHtt-induced toxicity and preventing striatal neurodegeneration.

Keywords:
HuntingtinMitochondria fissionStriatal vulnerability

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