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Related Experiment Videos

Mitochondrial Dysfunction in Huntington's Disease.

Catarina Carmo1, Luana Naia1,2, Carla Lopes1,2

  • 1CNC-Center for Neuroscience and Cell Biology, University of Coimbra, polo I, 3004-504, Coimbra, Portugal.

Advances in Experimental Medicine and Biology
|February 11, 2018
PubMed
Summary

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Mitochondrial dysfunction is an early sign of neurodegeneration in Huntington's disease (HD). Targeting mitochondria may offer new therapeutic strategies for HD patients.

Area of Science:

  • Neuroscience
  • Cell Biology
  • Genetics

Background:

  • Huntington's disease (HD) is a neurodegenerative disorder characterized by selective neuronal loss, particularly in the striatum and cerebral cortex.
  • Mitochondrial dysfunction is recognized as an early pathological event in HD pathogenesis.

Purpose of the Study:

  • To review the multifaceted roles of mitochondria in neuronal function.
  • To summarize evidence implicating mitochondrial dysfunction in the early stages of Huntington's disease.
  • To identify potential mitochondrial-based therapeutic targets for HD.

Main Methods:

  • Review of existing scientific literature and evidence regarding mitochondrial function and dysfunction in Huntington's disease.
  • Analysis of the diverse roles of mitochondria beyond energy production, including metabolism, dynamics, calcium homeostasis, and cell death.
Keywords:
Calcium dyshomeostasisCell deathMetabolic deficitsMitochondrial dynamicsOxidative stress

Related Experiment Videos

  • Identification of affected mitochondrial processes in HD models and patient data.

    • Main Results:

    • Mitochondria perform numerous vital functions in neurons, including metabolite synthesis, dynamic fission/fusion, axonal transport, calcium regulation, and free radical production.
    • These critical mitochondrial functions are demonstrably impaired in Huntington's disease, even in pre-symptomatic stages.
    • Dysfunctional mitochondria contribute significantly to neuronal impairment in HD.

    Conclusions:

    • Mitochondria play a central regulatory role in neurons affected by Huntington's disease.
    • Mitochondrial dysfunction is a key contributor to neurodegeneration in HD.
    • Targeting mitochondrial pathways presents a promising therapeutic avenue for treating Huntington's disease.