Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Oxidative damage in Huntington's disease pathogenesis.

Susan E Browne1, M Flint Beal

  • 1Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York, USA. Susan_Browne@Merck.com

Antioxidants & Redox Signaling
|October 13, 2006
PubMed
Summary

Huntington's disease (HD) involves neuronal death due to mutant huntingtin (mhtt). This review highlights evidence that oxidative damage plays a key role in triggering this neurodegeneration.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Validation of high-sensitivity assays to quantitate cerebrospinal fluid and serum β-galactosidase activity in patients with GM1-gangliosidosis.

Molecular therapy. Methods & clinical development·2024
Same author

The role of mitochondrial dysfunction in Alzheimer's disease pathogenesis.

Alzheimer's & dementia : the journal of the Alzheimer's Association·2022
Same author

Altered succinylation of mitochondrial proteins, APP and tau in Alzheimer's disease.

Nature communications·2022
Same author

Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model.

Neuropathology and applied neurobiology·2021
Same author

Isotope-reinforced polyunsaturated fatty acids improve Parkinson's disease-like phenotype in rats overexpressing α-synuclein.

Acta neuropathologica communications·2020
Same author

Changes of Coenzyme A and Acetyl-Coenzyme A Concentrations in Rats after a Single-Dose Intraperitoneal Injection of Hepatotoxic Thioacetamide Are Not Consistent with Rapid Recovery.

International journal of molecular sciences·2020

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington's disease (HD) is a fatal neurodegenerative disorder characterized by motor, cognitive, and psychiatric symptoms.
  • It results from a genetic mutation causing an expanded polyglutamine tract in the huntingtin protein (mutant htt or mhtt).
  • mhtt disrupts cellular functions, but the precise initiation mechanism remains unclear.

Purpose of the Study:

  • To review the evidence linking oxidative damage to neuronal degeneration in HD.
  • To explore the role of mitochondrial dysfunction and energy metabolism defects.

Main Methods:

  • Literature review of current evidence on HD pathogenesis.
  • Analysis of studies implicating oxidative stress and mitochondrial defects.

Related Experiment Videos

Main Results:

  • Evidence supports early mitochondrial dysfunction and impaired CNS energy metabolism in HD.
  • Downstream effects include excitotoxicity, apoptosis, and oxidative damage, contributing to neuronal loss.
  • Oxidative damage is implicated as a significant factor in HD etiology.

Conclusions:

  • Oxidative damage is a critical component in the pathogenesis of neuronal damage and degeneration in Huntington's disease.
  • Understanding these mechanisms may reveal therapeutic targets for HD.