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Updated: Dec 12, 2025
Dissection and Isolation of Murine Glia from Multiple Central Nervous System Regions
Published on: June 4, 2020
Targeting glial cells to elucidate the pathogenesis of Huntington's disease
1Division of Neuroscience, Institute of Biomedical Sciences, Academia Sinica, Taipei, 11529, Taiwan.
Insights
Huntington's disease (HD) involves expanded CAG repeats causing mutant Huntingtin (mHtt) protein aggregation and neuronal death. Glial cells and inflammation play key roles in HD pathogenesis, suggesting potential anti-inflammatory treatments.
Area of Science:
- Neuroscience
- Genetics
- Immunology
Background:
- Huntington's disease (HD) is a hereditary neurodegenerative disorder.
- It is caused by expanded CAG repeats in the Huntingtin (Htt) gene, leading to mutant Htt (mHtt) aggregation and neuronal dysfunction.
- Selective loss of neurons in the striatum and cortex characterizes HD, resulting in movement disorders, dementia, and death.
Purpose of the Study:
- To review the role of glial cells and inflammation in Huntington's disease pathogenesis.
- To discuss potential anti-inflammatory interventions for HD.
Main Methods:
- Literature review focusing on glial cells, inflammation, and Huntington's disease.
- Analysis of studies investigating non-cell-autonomous neurotoxicity in HD.
- Exploration of existing and potential anti-inflammatory therapeutic strategies.
Main Results:
- Mutant Huntingtin (mHtt) expression affects non-neuronal cells, indicating non-cell-autonomous neurotoxicity.
- Elevated inflammatory responses are a significant feature of HD pathology.
- Glial cells are implicated as key players in the progression of HD.
Conclusions:
- Glial cells and neuroinflammation are critical components of Huntington's disease pathogenesis.
- Targeting inflammatory pathways presents a promising therapeutic avenue for HD treatment.
- Further research into anti-inflammatory interventions is warranted for Huntington's disease.
Abstract:
Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by expended CAG repeats in the Huntingtin (Htt) gene. The resultant mutant Htt (mHtt) forms aggregates in neurons and causes neuronal dysfunctions. The major characteristic of HD is the selective loss of neurons in the striatum and cortex, which leads to movement disorders, dementia, and eventual death. Expression of mHtt was also found in non-neuronal cells in the brain, suggesting non-cell-autonomous neurotoxicity in HD. As was documented in many different neurodegenerative disorders, elevated inflammatory responses are also reported in HD. To date, effective treatments for this devastating disease remain to be developed. This review focuses on the importance of glial cells and inflammation in HD pathogenesis. Potential anti-inflammatory interventions for HD are also discussed.
