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Updated: Jul 19, 2026

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Published on: February 27, 2018

Striosomes and mood dysfunction in Huntington's disease.

Lynette J Tippett¹, Henry J Waldvogel, Sally J Thomas

¹Department of Psychology, The University of Auckland, Auckland, New Zealand. l.tippett@auckland.ac.nz

Brain : a Journal of Neurology

|October 17, 2006

Summary

Mood dysfunction in Huntington's disease is linked to specific brain changes. Altered GABA(A) receptor expression in striosomes correlates with mood symptoms, suggesting a role for these circuits in mood regulation.

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Published on: December 10, 2021

Area of Science:

Neuroscience
Genetics
Neurology

Background:

Huntington's disease (HD) exhibits variable phenotypes, including mood and cognitive symptoms alongside motor deficits.
Degeneration in the striatum's striosome and matrix compartments contributes to HD's variable presentation.

Purpose of the Study:

To investigate the relationship between striatal compartmental organization and phenotypic variability in Huntington's disease.
To determine if GABA(A) receptor expression differences in striosomes and matrix correlate with specific clinical symptoms.

Main Methods:

A double-blind study analyzed GABA(A) receptor immunohistochemistry in the striatum of 35 HD cases and 13 controls.
Detailed clinical symptomatology data were collected from patient records and family members.

Main Results:

A significant association was found between pronounced mood dysfunction and differential loss of GABA(A) receptor marker in striosomes.
This striosome abnormality correlated with later onset age, lower disease grade, and shorter CAG repeat length in the HD gene.
No independent association was observed between CAG repeat length or age of onset and mood dysfunction.

Conclusions:

Variations in Huntington's disease clinical symptomatology are linked to differential GABA(A) receptor expression in striatal compartments.
Striosome-related circuits appear to modulate mood functioning in Huntington's disease.