Microglial activation in the pathogenesis of Huntington's disease
Open accessAbstract
gene that encodes huntingtin protein. Although HD is characterized by a predominant loss of neurons in the striatum and cortex, previous studies point to a critical role of aberrant accumulation of mutant huntingtin in microglia that contributes to the progressive neurodegeneration in HD, through both cell-autonomous and non-cell-autonomous mechanisms. Microglia are resident immune cells in the central nervous system (CNS), which function to surveil the microenvironment at a quiescent state. In response to various pro-inflammatory stimuli, microglia become activated and undergo two separate phases (M1 and M2 phenotype), which release pro-inflammatory cytokines (IL-1β, IL-6, and TNF-α), anti-inflammatory cytokines, and growth factors (TGF-β, CD206, and Arg1), respectively. Immunoregulation by microglial activation could be either neurotoxic or neuroprotective. In this review, we summarized current understanding about microglial activation in the pathogenesis and progression of HD, with a primary focus of M1 and M2 phenotype of activated microglia and their corresponding signaling pathways.
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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 Yang et al., 2017
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- Authors
- Yang, H.-M., Yang, S., Huang, S.-S., Tang, B.-S., & Guo, J.-F.
- Affiliations
- Emory University (US)
- ORCID
- Yang, Su
- Venue
- Frontiers in Aging Neuroscience
- Publisher
- Frontiers Media SA
- Published
- 2017-06-19
- Volume
- 9
- Pages
- 193-193
- ISSN
- 1663-4365 (ISSN-L)
- PMC
- PMC5474461
- OpenAlex
- W2625794193
- Cited by
- 151
- References
- 118
- Citations by year
- 2026: 20 · 2025: 15 · 2024: 19 · 2023: 16 · 2022: 16 · 2021: 17 · 2020: 21 · 2019: 17 · 2018: 7 · 2017: 2
- FWCI
- 1.23
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by
- Locations
- Frontiers in Aging Neuroscience (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Natural Science Foundation of China (81430023, 2016CX025, 2016YFC1306000, 81371405, 81571248)
- Central South University (2016CX025)
- 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
- Huntington's disease, microglial activation, mutant huntingtin, M1/M2 microglial polarization, neuroinflammation, inflammatory cytokines, anti-inflammatory cytokines, neurodegeneration, striatum, signaling pathways, neuroprotection
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