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

Bioenergetics in Huntington's disease.

T Grünewald1, M F Beal

  • 1Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York 10021, USA.

Annals of the New York Academy of Sciences
|February 15, 2000
PubMed
Summary

Huntington's disease (HD) is an inherited neurodegenerative disorder linked to a CAG triplet repeat expansion. This review explores oxidative stress, excitotoxicity, and mitochondrial dysfunction in HD pathogenesis.

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

[Q fever : A rare differential diagnosis of granulomatous disease].

Der Pathologe·2016
Same author

Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy.

Cell death and differentiation·2015
Same author

[Musculoskeletal infections in the era of multiresistant pathogens].

Der Unfallchirurg·2012
Same author

Lack of evidence for systemic cytomegalovirus reactivation in maintenance hemodialysis patients.

European journal of clinical microbiology & infectious diseases : official publication of the European Society of Clinical Microbiology·2011
Same author

Impaired brain creatine kinase activity in Huntington's disease.

Neuro-degenerative diseases·2010
Same author

[Clostridium difficile infection].

Deutsche medizinische Wochenschrift (1946)·2010

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disorder.
  • HD is characterized by a CAG triplet repeat expansion in the huntingtin gene on chromosome 4.
  • The length of the polyglutamine tract correlates with disease onset and severity.

Purpose of the Study:

  • To review current concepts on the pathogenesis of Huntington's disease.
  • To discuss the involvement of oxidative stress, excitotoxicity, and mitochondrial dysfunction in HD.

Main Methods:

  • Literature review and synthesis of current research on Huntington's disease.
  • Discussion of proposed mechanisms of neurodegeneration in HD.

Main Results:

Related Experiment Videos

  • Mutant huntingtin induces neuronal cell death via apoptosis.
  • Disturbances in cellular energy homeostasis and oxidative damage contribute to neurodegeneration.
  • Free radical-induced oxidative stress, glutamate excitotoxicity, and mitochondrial respiratory chain defects are implicated in HD pathogenesis.

Conclusions:

  • Oxidative stress, excitotoxicity, and mitochondrial dysfunction are key contributors to neurodegeneration in Huntington's disease.
  • Understanding these mechanisms is crucial for developing therapeutic strategies for HD.