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Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

110
Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show...
110

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Related Experiment Video

Updated: May 4, 2026

Single Synapse Indicators of Glutamate Release and Uptake in Acute Brain Slices from Normal and Huntington Mice
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An experimental model for Huntington's chorea?

Dagmar H Zeef1, Ali Jahanshahi1, Rinske Vlamings2

  • 1Department of Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands; Department of Neurosurgery, Maastricht University Medical Center, Maastricht, The Netherlands; European Graduate School of Neuroscience (EURON) , The Netherlands.

Behavioural Brain Research
|January 14, 2014
PubMed
Summary
This summary is machine-generated.

This study validates a transgenic rat model for Huntington's disease (HD) chorea. Tetrabenazine effectively reduced hyperkinetic movements, confirming the model's utility for studying choreiform movement disorders.

Keywords:
ChoreaDopamineHuntington's diseaseTetrabenazineTransgenic rat model

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Area of Science:

  • Neuroscience
  • Genetics
  • Pharmacology

Background:

  • Huntington's disease (HD) is characterized by chorea, a hyperkinetic motor disorder.
  • A transgenic rat model exhibiting hyperkinetic movements is the only known experimental model for HD chorea.

Purpose of the Study:

  • To characterize hyperkinetic movements in the transgenic HD rat model.
  • To investigate the efficacy of tetrabenazine (TBZ) in mitigating these movements.

Main Methods:

  • Detailed assessment and characterization of hyperkinetic movements in transgenic HD rats.
  • Administration of tetrabenazine (TBZ) to evaluate its therapeutic effect.

Main Results:

  • The observed hyperkinetic movements met clinical-behavioral criteria for choreiform disorder.
  • Tetrabenazine significantly reduced the frequency of these hyperkinetic movements.

Conclusions:

  • The transgenic HD rat model exhibits genuine choreiform movement disorder.
  • This model is a valuable tool for research into chorea and its treatment.