The choreography of neuroinflammation in Huntington's disease
Open accessAbstract
Currently, the concept of 'neuroinflammation' includes inflammation associated with neurodegenerative diseases, in which there is little or no infiltration of blood-derived immune cells into the brain. The roles of brain-resident and peripheral immune cells in these inflammatory settings are poorly understood, and it is unclear whether neuroinflammation results from immune reaction to neuronal dysfunction/degeneration, and/or represents cell-autonomous phenotypes of dysfunctional immune cells. Here, we review recent studies examining these questions in the context of Huntington's disease (HD), where mutant Huntingtin (HTT) is expressed in both neurons and glia. Insights into the cellular and molecular mechanisms underlying neuroinflammation in HD may provide a better understanding of inflammation in more complex neurodegenerative disorders, and of the contribution of the neuroinflammatory component to neurodegenerative disease pathogenesis.
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The involvement of microglia in frontotemporal dementia (FTD) and Huntington's disease (HD) is well documented.
“Similarly, the involvement of microglia in frontotemporal dementia (FTD) and Huntington’s disease (HD) is also well documented.90–92”
From Microbiota–gut–brain axis and its therapeutic applications in neurodegenerative diseases (2024) · cites this paper as [90]
The R6/2 model probably does not reproduce all neuroinflammatory features seen in end-stage human Huntington's disease.
“indicating that this model likely does not fully recapitulate the neuroinflammation observed in endstage human Huntington's disease”
From Microglial depletion prevents extracellular matrix changes and striatal volume reduction in a model of Huntington's disease (2020) · cites this paper as Crotti and Glass, 2015
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- Authors
- Crotti, A., & Glass, C. K.
- Affiliations
- Biogen (United States) (US)
- University of California San Diego (US)
- Corresponding author
- Glass, Christopher K.
- Venue
- Trends in Immunology
- Publisher
- Elsevier BV
- Published
- 2015-06
- Volume
- 36
- Issue
- 6
- Pages
- 364-373
- ISSN
- 1471-4906 (ISSN-L)
- PMC
- PMC4786070
- OpenAlex
- W1502625668
- Cited by
- 276
- References
- 108
- Citations by year
- 2026: 20 · 2025: 15 · 2024: 21 · 2023: 21 · 2022: 38 · 2021: 28 · 2020: 28 · 2019: 24 · 2018: 35 · 2017: 25 · 2016: 16 · 2015: 4 · 2014: 1
- FWCI
- 2.13
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Trends in Immunology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Funders
- National Institute of General Medical Sciences (U54GM069338)
- National Institute of Diabetes and Digestive and Kidney Diseases (R01DK091183)
- Topics
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Neuroscience › Neurology › Neurological Disease Mechanisms and Treatments
- Keywords
- neuroinflammation, Huntington's disease, mutant huntingtin, neurodegeneration, glial cells, peripheral immune cells, neuronal dysfunction, pathogenesis
- MeSH terms
- Huntingtin Protein, Animals, Humans, Huntington Disease, Inflammation, Mutation, Nerve Tissue Proteins, Neurons, Cytokines, Microglia, Models, Immunological
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