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Updated: Aug 5, 2025

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
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Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Published on: February 27, 2018

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Cell Rearrangement and Oxidant/Antioxidant Imbalance in Huntington's Disease

Francesco D'Egidio1, Vanessa Castelli1, Annamaria Cimini1

  • 1Department of Life, Health and Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy.

Antioxidants (Basel, Switzerland)
|March 29, 2023
PubMed

Insights

Huntington's Disease (HD) involves a toxic HTT protein causing neuronal death through oxidative stress. This review examines cellular responses to HD-induced stress and the antioxidant system's role.

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington's Disease (HD) is a hereditary neurodegenerative disorder.
  • Caused by CAG triplet repeat expansion in the HTT gene, leading to mutant huntingtin (Htt) protein accumulation.
  • Mutant Htt causes neuronal dysfunction and death via oxidative damage, excitotoxicity, inflammation, and mitochondrial impairment.

Purpose of the Study:

  • To review the cellular response to Huntington's Disease-induced stress.
  • To focus on the role of oxidative stress in HD pathogenesis.
  • To explore the balance between oxidative stress and the antioxidant system in HD.

Main Methods:

  • Literature review of cellular responses to neurodegenerative conditions.
  • Analysis of the mechanisms underlying neuronal dysfunction in HD.
  • Examination of oxidative stress pathways and antioxidant defenses.

Main Results:

  • Neurons exhibit altered metabolism, calcium signaling, and substrate transport under HD stress.
  • Oxidative damage is a key factor in HD-induced neuronal cell death.
  • The cellular antioxidant system is crucial in mitigating HD-related oxidative stress.

Conclusions:

  • Understanding cellular stress responses in HD is vital for therapeutic development.
  • Targeting oxidative stress and bolstering antioxidant systems may offer therapeutic benefits for Huntington's Disease.

Keywords:
ROSdiethuntingtinimpairmentmitochondrianeurodegenerationneuronoxidative stressvitamins

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