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Updated: Oct 18, 2025

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Signaling Overlap between the Golgi Stress Response and Cysteine Metabolism in Huntington's Disease

Bindu D Paul1,2,3

  • 1Department of Pharmacology and Molecular Sciences, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.

Antioxidants (Basel, Switzerland)
|September 28, 2021
PubMed

Insights

Huntington's disease involves mutant huntingtin protein aggregation and brain cell toxicity. This review explores the Golgi stress response and its link to cysteine metabolism in HD pathogenesis.

Area of Science:

  • Neuroscience
  • Cell Biology
  • Biochemistry

Background:

  • Huntington's disease (HD) stems from polyglutamine repeat expansion in the huntingtin protein.
  • Mutant huntingtin aggregation causes cellular toxicity, impacting organellar stress responses.
  • The Golgi apparatus, crucial for protein processing, also acts as a stress sensor via the Golgi stress response.

Purpose of the Study:

  • To review the molecular mechanisms of the Golgi stress response.
  • To investigate the intersection of Golgi stress and cysteine metabolism in HD.

Main Methods:

  • Literature review of molecular mechanisms.
  • Analysis of cellular processes in Huntington's disease models.

Main Results:

  • The Golgi stress response is less understood than the ER stress response.
  • Dysregulated organellar stress is a key feature of HD pathogenesis.

Conclusions:

  • Understanding Golgi stress mechanisms is vital for HD research.
  • Cysteine metabolism alterations may contribute to Golgi stress in HD.

Keywords:
Golgi apparatusGolgi stress responseHuntington’s diseasecysteineintegrated stress responsetranssulfuration

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