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Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
Published on: February 27, 2018
Amyloid-like inclusions in Huntington's disease
D P McGowan1, W van Roon-Mom, H Holloway
1Department of Anatomy with Radiology, University of Auckland, Private Bag 92019, Symonds Street, Auckland, New Zealand.
Neuroscience
|October 19, 2000
Summary
Huntington's disease brain tissue contains neuronal inclusions with amyloid-like structures. This finding suggests a shared mechanism with other amyloid-associated neurodegenerative diseases like Alzheimer's.
Area of Science:
- Neuroscience
- Pathology
- Genetics
Background:
- Huntington's disease is a progressive, inherited neurodegenerative disorder.
- It is characterized by chorea, cognitive decline, and psychiatric issues.
- The disease is linked to expanded glutamine repeats in the huntingtin gene.
Purpose of the Study:
- To investigate the structural properties of neuronal inclusions in Huntington's disease.
- To determine if these inclusions exhibit amyloid-like characteristics.
- To explore potential parallels with other amyloid-associated diseases.
Main Methods:
- Examination of post mortem human brain tissue from Huntington's disease, Alzheimer's disease, and control cases.
- Analysis of brain tissue from transgenic mice (R6/2) expressing expanded polyglutamine huntingtin.
- Utilized Congo Red staining, polarizing microscopy, and confocal microscopy.
Main Results:
- Neuronal inclusions in Huntington's disease brain tissue were found to possess an amyloid-like structure.
- This amyloid-like structure was observed in both human and transgenic mouse models.
- The findings suggest a common pathological pathway in Huntington's disease and other amyloidopathies.
Conclusions:
- Some inclusions in Huntington's disease brain tissue exhibit amyloid-like structures.
- This finding supports the hypothesis that polyglutamine expansion leads to the formation of amyloid structures.
- The study highlights potential shared mechanisms between Huntington's disease and other amyloid-associated neurodegenerative disorders.
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