Cited by 151Neuroinflammation and Neurodegeneration MechanismsGenetic Neurodegenerative DiseasesNeurological Disease Mechanisms and Treatments

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Microglial activation in the pathogenesis of Huntington's disease

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Yang, Hui-Ming, Yang, Su, Huang, Shan-Shan, Tang, Bei-Sha, Guo, Ji-Feng·2017·Frontiers in Aging Neuroscience

DOI 10.3389/fnagi.2017.00193PMC PMC5474461

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Abstract

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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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)
DOI: 10.3389/fnagi.2017.00193
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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Microglial activation in the pathogenesis of Huntington's disease

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