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Huntington Disease l: Introduction

Huntington Disease l: Introduction

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 reduced penetrance,...

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Related Experiment Video

Updated: Jul 3, 2026

Single Synapse Indicators of Glutamate Release and Uptake in Acute Brain Slices from Normal and Huntington Mice

Single Synapse Indicators of Glutamate Release and Uptake in Acute Brain Slices from Normal and Huntington Mice

Published on: March 11, 2020

Restoring cortical disinhibition improves Huntington's disease phenotypes.

Sonja Blumenstock^1,2,3,4, David Arakelyan^5,6, Nicholas Del Grosso⁷

¹Department of Neurobiology, Center for Neural Circuits and Behavior, University of California San Diego, La Jolla, CA, USA. sblumenstock@ucsd.edu.

|July 1, 2026

Summary

Huntington's disease (HD) motor deficits involve specific cortical neuron issues. Stimulating vasoactive intestinal peptide (VIP) inhibitory neurons improved motor function in HD mice, suggesting a therapeutic target.

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Last Updated: Jul 3, 2026

Single Synapse Indicators of Glutamate Release and Uptake in Acute Brain Slices from Normal and Huntington Mice

Single Synapse Indicators of Glutamate Release and Uptake in Acute Brain Slices from Normal and Huntington Mice

Published on: March 11, 2020

The Double-H Maze: A Robust Behavioral Test for Learning and Memory in Rodents

The Double-H Maze: A Robust Behavioral Test for Learning and Memory in Rodents

Published on: July 8, 2015

Immunohistochemical Visualization of Hippocampal Neuron Activity After Spatial Learning in a Mouse Model of Neurodevelopmental Disorders

Immunohistochemical Visualization of Hippocampal Neuron Activity After Spatial Learning in a Mouse Model of Neurodevelopmental Disorders

Published on: May 12, 2015

Area of Science:

Neuroscience
Genetics
Neurology

Background:

Huntington's disease (HD) is a neurodegenerative disorder with poorly understood behavioral symptoms.
Cortical dysfunction is implicated in HD, but specific neuronal subtypes' roles are unclear.

Purpose of the Study:

Investigate cortical neuronal subtype activity in HD progression.
Identify potential therapeutic targets for HD.

Main Methods:

Longitudinal in vivo two-photon calcium imaging in R6/2 HD mouse model.
Examined activity of inhibitory neuron (IN) subtypes and corticostriatal (CStr) neurons.
Used optogenetics to activate VIP-INs and assess behavioral rescue.

Main Results:

HD motor deficits correlated with subtype-specific neuronal hypoactivity, notably in VIP-INs and CStr neurons.
Optogenetic VIP-IN activation normalized neuronal activity and ameliorated motor deficits in R6/2 mice.
Behavioral improvements persisted post-stimulation, indicating lasting therapeutic effects.

Conclusions:

Cortical inhibitory neurons, particularly VIP-INs, are critically involved in HD pathogenesis.
VIP-INs represent a promising therapeutic target for Huntington's disease.