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

Impaired glutamate uptake in the R6 Huntington's disease transgenic mice.

J C Liévens1, B Woodman, A Mahal

  • 1Medical and Molecular Genetics, GKT School of Medicine, London, UK.

Neurobiology of Disease
|October 11, 2001
PubMed
Summary

Huntington's disease (HD) involves CAG repeat expansion, leading to neuronal dysfunction. This study shows reduced glutamate transporter GLT1 in R6 mice, suggesting impaired glutamate uptake contributes to HD progression.

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

CNS Embryonal Tumor with PLAGL Amplification, a New Tumor in Children and Adolescents: Insights from a Comprehensive MRI Analysis.

AJNR. American journal of neuroradiology·2024
Same author

Intravenous thrombolysis for multi-ethnic Asians with acute ischaemia stroke in Malaysian public primary stroke centres versus acute stroke ready hospitals: Comparison of real-world clinical outcomes.

The Medical journal of Malaysia·2023
Same author

Rehabilitation after proximal interphalangeal joint replacement: A structured review of the literature.

Hand surgery & rehabilitation·2021
Same author

The road map toward an hepatitis C virus-free transplant population.

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons·2018
Same author

Safety of sofosbuvir-based regimens after liver transplantation: longitudinal assessment of renal function in the prospective ANRS CO23 CUPILT study.

Alimentary pharmacology & therapeutics·2018
Same author

Safety of an anti-PD-1 immune checkpoint inhibitor in a liver transplant recipient.

Annals of oncology : official journal of the European Society for Medical Oncology·2018

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington's disease (HD) is a late-onset neurodegenerative disorder caused by CAG/polyglutamine repeat expansion.
  • R6 mouse models exhibit movement disorders and neuronal polyglutamine aggregates preceding selective cell death.

Purpose of the Study:

  • To investigate the role of glutamate transporters in the R6 mouse model of Huntington's disease.
  • To determine if altered glutamate uptake contributes to the HD phenotype.

Main Methods:

  • Analysis of mRNA levels for astroglial glutamate transporters (GLT1, GLAST) and EAAC1 in R6 mice.
  • Measurement of glutamate uptake in the striatum and cortex.
  • Assessment of glutamine synthetase mRNA levels.

Related Experiment Videos

Main Results:

  • A significant decrease in GLT1 mRNA and glutamate uptake was observed in the striatum and cortex of R6 mice.
  • GLAST and EAAC1 expression remained unchanged.
  • A decrease in astroglial glutamine synthetase mRNA was also detected prior to neurodegeneration.

Conclusions:

  • Impaired astrocytic glutamate uptake, specifically via GLT1, may contribute to neuronal dysfunction and cell death in Huntington's disease.
  • These molecular changes precede overt neurodegeneration, highlighting early pathological mechanisms in HD.