The DOI matches its Crossref record, with a small difference in title or year; 35 of the 35 references it cites were found in a registry or an index.
Microglial activation correlates with severity in Huntington disease: A clinical and PET study
Abstract
BACKGROUND: Huntington disease (HD) is characterized by the progressive death of medium spiny dopamine receptor bearing striatal GABAergic neurons. In addition, microglial activation in the areas of neuronal loss has recently been described in postmortem studies. Activated microglia are known to release neurotoxic cytokines, and these may contribute to the pathologic process. METHODS: To evaluate in vivo the involvement of microglia activation in HD, the authors studied patients at different stages of the disease using [(11)C](R)-PK11195 PET, a marker of microglia activation, and [(11)C]raclopride PET, a marker of dopamine D2 receptor binding and hence striatal GABAergic cell function. RESULTS: In HD patients, a significant increase in striatal [(11)C](R)-PK11195 binding was observed, which significantly correlated with disease severity as reflected by the striatal reduction in [(11)C]raclopride binding, the Unified Huntington's Disease Rating Scale score, and the patients' CAG index. Also detected were significant increases in microglia activation in cortical regions including prefrontal cortex and anterior cingulate. CONCLUSIONS: These [(11)C](R)-PK11195 PET findings show that the level of microglial activation correlates with Huntington disease (HD) severity. They lend support to the view that microglia contribute to the ongoing neuronal degeneration in HD and indicate that [(11)C](R)-PK11195 PET provides a valuable marker when monitoring the efficacy of putative neuroprotecting agents in this relentlessly progressive genetic disorder.
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“Huntington's disease is associated with a reactive microglial response and consequent inflammation.”
From Microglial depletion prevents extracellular matrix changes and striatal volume reduction in a model of Huntington's disease (2020) · cites this paper as Pavese et al., 2006
In the citing paper
“In Huntington's disease, microglial activation is detected years before the onset of clinical symptoms and correlates with striatal neuron dysfunction in subclinical (Tai et al. , 2007) as well as clinical stages of the disease (Pavese et al. , 2006; Politis et al. , 2011).”
From Microglial depletion prevents extracellular matrix changes and striatal volume reduction in a model of Huntington's disease (2020) · cites this paper as Pavese et al., 2006
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Microglia-mediated neurotoxicity: uncovering the molecular mechanisms
Block, M. L., Zecca, L., & Hong, J.-S. (2007) · Nature Reviews Neuroscience · doi:10.1038/nrn2038 · Cited by 4,110
Huntington's disease: from molecular pathogenesis to clinical treatment
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Microglia in neurodegeneration
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Does neuroinflammation fan the flame in neurodegenerative diseases?
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“Once symptoms have manifested, microglial activation correlates with disease severity [115,116].”
Metadata
- Authors
- Pavese, N., Gerhard, A., Tai, Y. F., Ho, A. K., Turkheimer, F., Barker, R. A., Brooks, D. J., & Piccini, P.
- Affiliations
- Hammersmith Hospital (GB)
- Venue
- Neurology
- Published
- 2006-06-13
- Volume
- 66
- Issue
- 11
- Pages
- 1638-1643
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W2025681964
- Cited by
- 462
- References
- 35
- Citations by year
- 2026: 12 · 2025: 19 · 2024: 30 · 2023: 26 · 2022: 22 · 2021: 26 · 2020: 27 · 2019: 19 · 2018: 18 · 2017: 31 · 2016: 25 · 2015: 25 · 2014: 38 · 2013: 19 · 2012: 14
- FWCI
- 8.08
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neurology (publishedVersion)
- PubMed (publishedVersion)
- CentAUR (University of Reading) (acceptedVersion)
- Research Explorer (The University of Manchester) (submittedVersion)
- Funders
- Wellcome Trust
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- Huntington's disease, microglial activation, dopamine D2 receptor binding, disease severity, neuroinflammation, neurodegeneration, prefrontal cortex, anterior cingulate cortex, CAG repeat expansion
- MeSH terms
- Adult, Aged, Amides, Corpus Striatum, Female, Humans, Huntington Disease, Isoquinolines, Male, Middle Aged, Sensitivity and Specificity, Severity of Illness Index, Statistics as Topic, Reproducibility of Results, Microglia, Radiopharmaceuticals, Raclopride, Positron-Emission Tomography
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