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

Platelet abnormalities in Huntington's disease.

Hélèna L Denis1,2, Jérôme Lamontagne-Proulx1,2, Isabelle St-Amour1,2

  • 1Centre de Recherche du CHU de Québec, Québec, QC, Canada.

Journal of Neurology, Neurosurgery, and Psychiatry
|December 21, 2018
PubMed
Summary

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

Mutant huntingtin in the extracellular matrix: A new perspective on Huntington's disease pathology.

Neurobiology of disease·2026
Same author

Isw2-mediated chromatin remodeling governs antifungal tolerance and heteroresistance in Candida albicans.

npj antimicrobials and resistance·2026
Same author

Megakaryocytes Internalize and Are Activated by Immune Complexes.

Circulation research·2026
Same author

The potential of in-ovo probiotics to replace antibiotic growth promoters in broiler diets: Effect on performance, meat quality, cecal fermentation and oxidative stress indicators.

Poultry science·2026
Same author

Met32 governs transcriptional control of sulfur metabolic flexibility and resistance to reactive sulfur species in the human fungal pathogen <i>Candida albicans</i>.

mBio·2026
Same author

Mitochondrial Transplantation in the Eye: A Review and Evaluation of Surgical Approaches.

bioRxiv : the preprint server for biology·2026
This summary is machine-generated.

Platelets in Huntington's disease (HD) are dysfunctional, impacting blood vessel health and function. This study reveals how mutant huntingtin (mHtt) affects platelet function, contributing to HD pathology.

Area of Science:

  • Neuroscience
  • Genetics
  • Vascular Biology

Background:

  • Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by a mutation in the huntingtin gene, leading to mutant huntingtin (mHtt) protein accumulation.
  • HD brains exhibit vascular abnormalities, including altered blood vessel density and increased blood-brain barrier (BBB) leakage.
  • Platelets contain high concentrations of mHtt and play a crucial role in vascular integrity.

Purpose of the Study:

  • To investigate the role of platelets in the vascular abnormalities observed in Huntington's disease.
  • To elucidate the origin of vascular and BBB abnormalities by studying platelet function in HD patients and a mouse model.

Main Methods:

  • Assessed platelet function using ELISA, western blot, and RNA sequencing in 71 HD patients and 68 controls.

Related Experiment Videos

  • Conducted hemostasis and platelet depletion tests in the R6/2 HD mouse model.

    • Main Results:

    • Platelets from HD patients exhibit dysfunction in releasing angiogenic factors.
    • HD platelets show impaired functions related to thrombosis, angiogenesis, and vascular hemostasis.
    • Mutant huntingtin (mHtt) significantly impacts platelet function.

    Conclusions:

    • The study provides a deeper understanding of how mutant huntingtin affects platelet function in Huntington's disease.
    • Platelet dysfunction is a key contributor to the vascular pathology in HD.
    • Targeting platelet function may offer therapeutic strategies for HD.