Related Experiment Video
Updated: Mar 24, 2026

09:22
In Vitro Aggregation Assays Using Hyperphosphorylated Tau Protein
Published on: January 2, 2015
19.1K
Is Huntington's disease a tauopathy?
Maud Gratuze1, Giulia Cisbani1, Francesca Cicchetti1
1Faculté de Médecine, département de Psychiatrie et Neurosciences, Université Laval, Québec, QC, Canada Centre de Recherche du CHU de Québec, Axe Neurosciences, Québec, QC, Canada.
Brain : a Journal of Neurology
|March 13, 2016
Summary
Huntington's disease may be a tauopathy. Research suggests mutant huntingtin protein affects tau, leading to aggregated tau inclusions in the brain, similar to other tauopathies.
Area of Science:
- Neuroscience
- Genetics
- Pathology
Background:
- Tauopathies are neurodegenerative diseases characterized by abnormal tau protein aggregation.
- Huntington's disease (HD) is a monogenic neurodegenerative disorder with motor, cognitive, and psychiatric symptoms.
Purpose of the Study:
- To review evidence suggesting Huntington's disease may be a tauopathy.
- To explore the relationship between mutant huntingtin and tau pathology.
Main Methods:
- Review of existing scientific literature and case reports.
- Analysis of studies on tau pathology in Huntington's disease patients.
Main Results:
- Patients with Huntington's disease exhibit aggregated tau inclusions in the brain.
- Tau haplotype impacts cognitive function in Huntington's disease patients.
- Mutant huntingtin protein may influence tau splicing, phosphorylation, and aggregation.
Conclusions:
- Emerging evidence supports classifying Huntington's disease as a tauopathy.
- Further research is warranted to elucidate the precise mechanisms linking HD and tau pathology.
Related Concept Videos
Alzheimer's Disease: Overview
2.0K
Alzheimer's Disease (AD) is a continually advancing neurodegenerative disorder, distinguished by escalating memory loss, cognitive dysfunction, and dementia. The disease unfolds in three stages: preclinical, mild cognitive impairment (MCI), and dementia. Its onset is insidious, and the progression gradual, with the cause not well explained by other disorders.
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ...
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ...
2.0K
Parkinson's Disease: Overview
2.3K
Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
2.3K
Amyloid Fibrils
12.9K
Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
12.9K
Lysosomal Hydrolases
4.7K
Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
4.7K

