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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“(Pavese, Gerhard et al. 2006) In several important clinical trials, we can also find out that host immune reaction plays an important role in the pathogenesis of Huntington's disease.”
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Rodríguez, P. V. (2019) · Apollo (University of Cambridge) · doi:10.17863/cam.45106 · Cited by 1
“, 2004), and Huntington’s disease (Pavese et al., 2006).”
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“…excitotoxicity drives neuron dysfunction/death 93–96 , 2) pyroptosis, where neuroinflammatory responses promote neurodegeneration 66,97–99 , 3) synaptic signaling, where neuron-to-neuron communication is lost within and between brain regions 93,100–103 , and 4) nervous system development 104–108 .”
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“Activated microglia have been proposed to play a role in the pathogenesis of a range of neurodegenerative diseases, which include Alzheimer ’ s [44] , Parkinson ’ s [45] and Huntington ’ s diseases [46,47] .”
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Trollope, L. (2015) · White Rose eTheses Online (University of Leeds, The University of Sheffield, University of York)
Neues zur Huntington-Krankheit Huntington's Disease Update
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Ciarmiello, A., & Giovacchini, G. (2016) · Springer eBooks · doi:10.1007/978-3-319-31614-7_13
“Increased striatal binding of 11 CPK11195 was reported also in HD patients which signifi cantly correlated with disease severity as assessed by Unifi ed Huntington’s Disease Rating Scale score [ 38 ].”
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“HD CAG expansion carriers also exhibit low-level chronic inflammation in the CNS and periphery, evidenced by increased microglial activation within the striatum and cortex [32-35] and higher levels of inflammatory cytokines within brain tissue, cerebrospinal fluid, and blood plasma relative to control groups [36-40].”
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Self-citationLiu, M., Zhao, J., Xue, C., Yang, J., & Ying, L. (2024) · Figshare · doi:10.6084/m9.figshare.26738769
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Smith, K. M., & Saranathan, M. (2025) · Seminars in Neurology · doi:10.1055/a-2769-6508
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“…moteur de patients SLA, avec une forte corrélation entre son BP et le stade clinique de la maladie (Figure 10, (9)). d. La chorée de Huntington (Pavese et al 2006; Politis et al 2008) La chorée de Huntington est une maladie chronique héréditaire à transmission autosomique dominante touchant…”
BackgroundInfluentialAbjean, L. (2019) · theses.fr (ABES) · doi:10.70675/f664e003zf93fz467eza6a3z3d953eaffe4e
“…de mettre en évidence une activation microgliale chez les patients présymptomatiques de la MH, qui peut être détectée jusqu’à 15 ans avant l’apparition des premiers symptômes et qui suit ensuite l’évolution de la pathologie (Pavese et al., 2006; Tai et al., 2007a, 2007b; Politis et al., 2015).”
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Flores-Thomas, A. P., Retamosa-Sanchez, E., Gómez, A. A., Flores‐Cuadrado, A., Saiz‐Sánchez, D., Astillero‐Lopez, V., Rábano, A., Martı́nez-Marcos, A., & Úbeda‐Bañón, I. (2026) · Neurobiology of Disease · doi:10.1016/j.nbd.2026.107413
Moretto, M., Baldascino, G., Turkheimer, F., Warner, J. H., Wood, A., Williams, S., van Wamelen, D. J., Veronese, M., & iMarkHD study team (2026) · Research Square · doi:10.21203/rs.3.rs-9675918/v1
Self-citationLangness, V. F., Simmons, D. A., Kaur, S., Massa, S. M., & Longo, F. M. (2026) · Journal of Translational Medicine · doi:10.1186/s12967-026-08276-x
“TSPO-PET tracers have also detected neuroinflammation in AD and HD patients [163, 164], making it a potential molecular imaging bio-marker of LM11A-31 treatment response, particularly in conditions involving neuroinflammation.”
Pérez-Villavicencio, J., Villa-Robledo, O., Megchun-Vázquez, X., Uriarte-Jiménez, F., Rubio‐Osornio, M., & Serrano‐García, N. (2026) · Neuroglia · doi:10.3390/neuroglia7030023
“…regions that do not yet show structural atrophy on conventional magnetic resonance imaging (MRI) supports the hypothesis that immune-related cellular changes may precede structural damage, though the precise cellular contributors and their functional significance remain to be determined [78,79].”
Comparison of partial volume correction methods integrating PET and MRI data in Huntington's disease
Moretto, M., Baldascino, G., Turkheimer, F., Warner, J. H., Wood, A., Williams, S., van Wamelen, D. J., Veronese, M., Staunton, J., Martin, N. H., et al. (2026) · EANM Innovation · doi:10.1016/j.eanmi.2026.100300
Self-citationGene therapy in Huntington’s disease (HD)
Tirgar, F., Hosseini, M., Alijanpour, S., Aghanoori, M.-R., Alijanpour, S., & Mahdavinataj, H. (2026) · Elsevier eBooks · doi:10.1016/b978-0-443-40467-2.00011-7
Carlson, J. (2026) · Neurodegenerative Disease Management · doi:10.1080/17582024.2026.2739334
BindingDB (2012) · UC San Diego · doi:10.7270/q2222vp0
The role of protein aggregation in Huntington's disease.
Didszun, C. (2010) · OpenGrey (Institut de l'Information Scientifique et Technique)
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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