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.
Overview
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
In the citing paper
“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
Citations
Cites
Statistical parametric maps in functional imaging: A general linear approach
Friston, K. J., Holmes, A. P., Worsley, K. J., Poline, J. ‐., Frith, C. D., & Frackowiak, R. S. J. (1994) · Human Brain Mapping · doi:10.1002/hbm.460020402 · Cited by 9,688
Unified Huntington's disease rating scale: Reliability and consistency
Kremer, H. P. H. (1996) · Movement Disorders · doi:10.1002/mds.870110204 · Cited by 2,149
Parametric Imaging of Ligand-Receptor Binding in PET Using a Simplified Reference Region Model
Gunn, R. N., Lammertsma, A. A., Hume, S. P., & Cunningham, V. J. (1997) · NeuroImage · doi:10.1006/nimg.1997.0303 · Cited by 1,085
Unified Huntington's disease rating scale: reliability and consistency
Kremer, H. P. H. (1996) · Radboud Repository (Radboud University) · Cited by 1,059
Microglia as neuroprotective, immunocompetent cells of the CNS
Streit, W. J. (2002) · Glia · doi:10.1002/glia.10154 · Cited by 919
Dopamine release from nigral transplants visualized in vivo in a Parkinson's patient
Piccini, P. P., Brooks, D. J., Björklund, A., Gunn, R. N., Grasby, P. M., Rimoldi, O. E., Brundin, P., Hagell, P., Rehncrona, S., Widner, H., et al. (1999) · Nature Neuroscience · doi:10.1038/16060 · Cited by 700
Self-citationMicroglia in health and disease
Kim, S. U., & de Vellis, J. (2005) · Journal of Neuroscience Research · doi:10.1002/jnr.20562 · Cited by 687
CAG repeat number governs the development rate of pathology in Huntington's disease
Penney, J. B., Vonsattel, J., MacDonald, M. E., Gusella, J. F., & Myers, R. H. (1997) · Annals of Neurology · doi:10.1002/ana.410410521 · Cited by 670
The peripheral benzodiazepine binding site in the brain in multiple sclerosis
Banati, R. B., Newcombe, J., Gunn, R. N., Cagnin, A., Turkheimer, F., Heppner, F., Price, G., Wegner, F., Giovannoni, G., Miller, D. H., et al. (2000) · Brain · doi:10.1093/brain/123.11.2321 · Cited by 661
Self-citationEarly and Progressive Accumulation of Reactive Microglia in the Huntington Disease Brain
Sapp, E., Kegel, K. B., D, N. A. M., Hashikawa, T., Uchiyama, Y., Tohyama, K., Bhide, P. G., Vonsattel, J., & DiFiglia, M. (2001) · Journal of Neuropathology & Experimental Neurology · doi:10.1093/jnen/60.2.161 · Cited by 558
Research works printed but not linked: 1
- Talairach J Tournoux P.A coplanar sterotactic atlas of the human brain. New York: Thieme 1988.
Cited by
Sorted by citations
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
Ross, C. A., & Tabrizi, S. J. (2010) · The Lancet Neurology · doi:10.1016/s1474-4422(10)70245-3 · Cited by 1,816
Microglia in neurodegeneration
Hickman, S., Izzy, S., Sen, P., Morsett, L., & El Khoury, J. (2018) · Nature Neuroscience · doi:10.1038/s41593-018-0242-x · Cited by 1,710
Rupprecht, R., Papadopoulos, V., Rammes, G., Baghai, T. C., Fan, J., Akula, N., Groyer, G., Adams, D., & Schumacher, M. (2010) · Nature Reviews Drug Discovery · doi:10.1038/nrd3295 · Cited by 930
Does neuroinflammation fan the flame in neurodegenerative diseases?
Frank-Cannon, T. C., Alto, L. T., McAlpine, F. E., & Tansey, M. G. (2009) · Molecular Neurodegeneration · doi:10.1186/1750-1326-4-47 · Cited by 751
“Once symptoms have manifested, microglial activation correlates with disease severity [115,116].”
Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Zuccato, C., Valenza, M., & Cattaneo, E. (2010) · Physiological Reviews · doi:10.1152/physrev.00041.2009 · Cited by 848
“known to be activated in HD (330, 438, 563), have been”
BackgroundAlternatively activated microglia and macrophages in the central nervous system
Franco, R., & Fernández‐Suárez, D. (2015) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2015.05.003 · Cited by 672
“This compound has been tested in patients with different neurological disorders such as Parkinson’s disease, dementia, multiple system atrophy and Huntington’s disease (Pavese et al., 2006; Ouchi et al., 2005; Gerhard et al., 2003; Cicchetti et al., 2002; Cagnin et al., 2001).”
BackgroundBjörkqvist, M., Wild, E. J., Thiele, J., Silvestroni, A., André, R., Lahiri, N., Raibon, E., Lee, R. V., Benn, C., Soulet, D., et al. (2008) · The Journal of Experimental Medicine · doi:10.1084/jem.20080178 · Cited by 646
Basic mechanisms of neurodegeneration: a critical update
Jellinger, K. A. (2010) · Journal of Cellular and Molecular Medicine · doi:10.1111/j.1582-4934.2010.01010.x · Cited by 640
“Microglia activation in HD has been shown to correlate with severity of the disease [378], with a close spatial and temporal relationship to neuronal dysfunction, and has already been detected in presymptomatic HD gene carriers [379].”
BackgroundCerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity
Rosas, H. D., Salat, D. H., Lee, S. Y., Zaleta, A. K., Pappu, V., Fischl, B., Greve, D., Hevelone, N., & Hersch, S. M. (2008) · Brain · doi:10.1093/brain/awn025 · Cited by 460
“Indeed, a recent publication reported increased microglial activation in anterior cingulate cortex in HD (Pavese et al., 2006).”
BackgroundTranslocator protein 18 kDa (TSPO): Molecular sensor of brain injury and repair
Chen, M.-K., & Guilarte, T. R. (2008) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2007.12.004 · Cited by 506
“…al., 2001a; Cagnin et al., 2004; Gerhard et al., 2003; Gerhard et al., 2004; Gerhard et al., 2006; Messmer and Reynolds, 1998; Ouchi et al., 2005; Pavese et al., 2006), in ischemia or stroke (Demerle-Pallardy et al., 1991; Gerhard et al., 2005; Myers et al., 1991; Pappata et al., 2000; Price et…”
BackgroundMethodResultInfluentialMicroglial activation in presymptomatic Huntington's disease gene carriers
Tai, Y. F., Pavese, N., Gerhard, A., Tabrizi, S. J., Barker, R. A., Brooks, D. J., & Piccini, P. (2007) · Brain · doi:10.1093/brain/awm044 · Cited by 440
“Finally, we compared the PK binding of HD PGCs with the recently published data of symptomatic HD patients (Pavese et al., 2006) in order to assess the temporal evolution of microglial activation with disease progression.”
BackgroundMethodResultInfluentialSelf-citationNuclear imaging of neuroinflammation: a comprehensive review of [11C]PK11195 challengers
Chauveau, F., Boutin, H., Camp, N. V., Dollé, F., & Tavitian, B. (2008) · European Journal of Nuclear Medicine and Molecular Imaging · doi:10.1007/s00259-008-0908-9 · Cited by 419
“Therefore, until now, the interpretations of results have been limited to an emphasis on the general agreement between brain areas of increased [C] PK11195 uptake and the known distribution of a given pathology [45–50].”
BackgroundRAN Translation in Huntington Disease
Báñez-Coronel, M., Ayhan, F., Tarabochia, A. D., Zu, T., Pérez, B., Tusi, S. K., Pletniková, O., Borchelt, D., Ross, C. A., Margolis, R. L., et al. (2015) · Neuron · doi:10.1016/j.neuron.2015.10.038 · Cited by 353
“Taken together, these results raise the possibility that HD-RAN proteins trigger neuroinflammatory changes (Björkqvist et al., 2008, 2009; Myers et al., 1991; Pavese et al., 2006; Tai et al., 2007) and cell death in HD.”
Rüb, U., Schöls, L. J., Paulson, H. L., Auburger, G. W. J., Kermer, P., Jen, J. C., Seidel, K., Korf, H., & Deller, T. (2013) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2013.01.001 · Cited by 346
Venneti, S., Lopresti, B. J., & Wiley, C. A. (2006) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2006.10.002 · Cited by 373
Microglial Dynamics and Role in the Healthy and Diseased Brain: A Paradigm of Functional Plasticity
Gomez-Nicola, D., & Perry, V. H. (2015) · The Neuroscientist · doi:10.1177/1073858414530512 · Cited by 340
Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors
Crotti, A., Benner, C., Kerman, B. E., Gosselin, D., Lagier-Tourenne, C., Zuccato, C., Cattaneo, E., Gage, F. H., Cleveland, D. W., & Glass, C. K. (2014) · Nature Neuroscience · doi:10.1038/nn.3668 · Cited by 333
Neuron-glia signaling in synapse elimination
Wilton, D. K., Dissing-Olesen, L., & Stevens, B. (2019) · Annual Review of Neuroscience · doi:10.1146/annurev-neuro-070918-050306 · Cited by 332
Raymond, L. A., André, V. M., Cepeda, C., Gladding, C. M., Milnerwood, A. J., & Levine, M. S. (2011) · Neuroscience · doi:10.1016/j.neuroscience.2011.08.052 · Cited by 332
“Alterations in microglial activation, leading to enhanced release of the endogenous NMDAR agonist quinolinate (Guidetti et al., 2004; Pavese et al., 2006; Tai et al., 2007), could also contribute to stimulation of ExNMDARs.”
BackgroundCzéh, M., Gressèns, P., & Kaindl, A. M. (2011) · Developmental Neuroscience · doi:10.1159/000328989 · Cited by 337
The Neuropathology of Huntington’s Disease
Waldvogel, H. J., Kim, E. H., Tippett, L. J., Vonsattel, J., & Faull, R. L. M. (2014) · Current topics in behavioral neurosciences · doi:10.1007/7854_2014_354 · Cited by 327
“positive emission topography have investigated microglial activation in the brain of HD and have found widespread microglial activation throughout the striatum, pallidum, frontal and cingulate cortices and brainstem in HD cases, which was evident also in presymptomatic HTT gene carriers (Gomez-Tortosa et al. 2000; Pav…”
BackgroundNeuroinflammation in Neurodegenerative Disorders-a Review
Schain, M., & Kreisl, W. C. (2017) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-017-0733-2 · Cited by 301
Proteomic and oxidative stress analysis in human brain samples of Huntington disease
Sorolla, M. A., Reverter‐Branchat, G., Tamarit, J., Ferrer, I., Ros, J., & Cabiscol, E. (2008) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2008.05.014 · Cited by 310
NRF2 as a Therapeutic Target in Neurodegenerative Diseases
Brandes, M. S., & Gray, N. E. (2020) · ASN Neuro · doi:10.1177/1759091419899782 · Cited by 297
“, 2010), chronic inflammation is also evident in HD (Pavese et al., 2006; Tai et al., 2007; Bjorkqvist et al., 2008; Politis et al., 2011).”
BackgroundWerry, E. L., Bright, F., Piguet, O., Ittner, L. M., Halliday, G. M., Hodges, J. R., Kiernan, M. C., Loy, C. T., Kril, J. J., & Kassiou, M. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20133161 · Cited by 298
Simmons, D. A., Casale, M., Alcon, B., Pham, N., Narayan, N., & LYNCH, G. S. (2007) · Glia · doi:10.1002/glia.20526 · Cited by 277
“, 2007) and they correlate with the number of CAG repeats and the severity of the motor component of the disease (Pavese et al., 2006).”
BackgroundInfluentialReference and target region modeling of [11C]-(R)-PK11195 brain studies.
Turkheimer, F., Edison, P., Pavese, N., Roncaroli, F., Anderson, A. N., Hammers, A., Gerhard, A., Hinz, R., Tai, Y., & Brooks, D. J. (2007) · PubMed · Cited by 275
Self-citationRecent advances in our understanding of neurodegeneration
Jellinger, K. A. (2009) · Journal of Neural Transmission · doi:10.1007/s00702-009-0240-y · Cited by 274
“Microglia activation that may play a major role in the pathogenesis of HD has been shown to correlate with severity of the disease, with a close spatial and temporal relationship to neuronal dysfunction (Pavese et al. 2006; Tai et al. 2007a), and has already been detected in presymptomatic HD gene carriers (Tai et al.”
BackgroundChen, P. S., Wang, C.-C., Bortner, C. D., Peng, G.-S., Wu, X., Pang, H., Lu, R.-B., Gean, P.-W., Chuang, D.-M., & Hong, J.-S. (2007) · Neuroscience · doi:10.1016/j.neuroscience.2007.06.053 · Cited by 262
Parkin Deficiency Increases Vulnerability to Inflammation-Related Nigral Degeneration
Frank-Cannon, T. C., Tran, T. A. T., Ruhn, K. A., Martinez, T. N., Hong, J. J., Marvin, M. A., Hartley, M., Treviño, I., O’Brien, D. E., Casey, B. H., et al. (2008) · Journal of Neuroscience · doi:10.1523/jneurosci.3001-08.2008 · Cited by 268
“…examination of brains from PD patients confirms the presence of neuroinflammation, raising the possibility that inflammatory mediators contribute to the progression of PD (McGeer et al., 1988; Banati et al., 1998; Hunot et al., 1999; Cassarino et al., 2000; Pavese et al., 2006; Whitton, 2007).”
BackgroundRole of Microglia in Neurological Disorders and Their Potentials as a Therapeutic Target
Du, L., Zhang, Y., Chen, Y., Zhu, J., Yang, Y., & Zhang, H.-L. (2016) · Molecular Neurobiology · doi:10.1007/s12035-016-0245-0 · Cited by 280
Genetics and Neuropathology of Huntington's Disease
Reiner, A. J., Dragatsis, I., & Dietrich, P. (2011) · International review of neurobiology · doi:10.1016/b978-0-12-381328-2.00014-6 · Cited by 262
“For example, activated microglia are greatly increased in abundance in HD cortex, striatum, and globus pallidus, and their abundance increases with grade and neuron loss (Pavese et al., 2006; Sapp et al., 2001a).”
BackgroundMicroglia and neuroprotection: From in vitro studies to therapeutic applications
Polazzi, E., & Monti, B. (2010) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2010.06.009 · Cited by 269
PET imaging of neuroinflammation in neurological disorders
Kreisl, W. C., Kim, M.-J., Coughlin, J. M., Henter, I. D., Owen, D. R., & Innis, R. B. (2020) · The Lancet Neurology · doi:10.1016/s1474-4422(20)30346-x · Cited by 251
The Role of Microglia and Astrocytes in Huntington’s Disease
Palpagama, T. H., Waldvogel, H. J., Faull, R. L. M., & Kwakowsky, A. (2019) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2019.00258 · Cited by 246
Hsiao, H., Chen, Y., Chen, H., Tu, P., & Chern, Y. (2013) · Human Molecular Genetics · doi:10.1093/hmg/ddt036 · Cited by 242
Mochel, F., Charles, P., Seguin, F., Barritault, J., Coussieu, C., Périn, L., Bouc, Y. L., Gervais, C., Carcelain, G., Vassault, A. J., et al. (2007) · PLoS ONE · doi:10.1371/journal.pone.0000647 · Cited by 235
“pathophysiology of HD [13,14].”
Myelin breakdown and iron changes in Huntington's disease: pathogenesis and treatment implications
Bartzokis, G., Lu, P. H., Tishler, T. A., Fong, S. M., Oluwadara, B., Finn, J. P., Huang, D., Bordelon, Y., Mintz, J., & Perlman, S. (2007) · Neurochemical Research · doi:10.1007/s11064-007-9352-7 · Cited by 238
“HD pathology is associated with increased microglia numbers and their activation [78, 79], however, although”
BackgroundThe methodology of TSPO imaging with positron emission tomography
Turkheimer, F. E., Rizzo, G., Bloomfield, P. S., Howes, O., Zanotti-Fregonara, P., Bertoldo, A., & Veronese, M. (2015) · Biochemical Society Transactions · doi:10.1042/bst20150058 · Cited by 238
Self-citationNon-steroidal anti-inflammatory drugs in Parkinson's disease
Esposito, E., Matteo, V. D., Benigno, A., Pierucci, M., Crescimanno, G., & Giovanni, G. D. (2007) · Experimental Neurology · doi:10.1016/j.expneurol.2007.02.008 · Cited by 237
“…revealed in a broad spectrum of neurodegenerative diseases that share with it a common pathological process, such as Alzheimer's disease (AD), Huntington's disease (HD), amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS) (Minghetti, 2005; Kim and Joh, 2006; Pavese et al., 2006).”
BackgroundDalrymple, A., Wild, E. J., Joubert, R., Sathasivam, K., Björkqvist, M., Petersén, Å., Jackson, G. S., Isaacs, J. D., Kristiansen, M., Bates, G. P., et al. (2007) · Journal of Proteome Research · doi:10.1021/pr0700753 · Cited by 223
Therapeutic Inhibition of the Complement System in Diseases of the Central Nervous System
Carpanini, S. M., Torvell, M., & Morgan, B. P. (2019) · Frontiers in Immunology · doi:10.3389/fimmu.2019.00362 · Cited by 215
Clinical Features of Huntington’s Disease
Ghosh, R., & Tabrizi, S. J. (2018) · Advances in experimental medicine and biology · doi:10.1007/978-3-319-71779-1_1 · Cited by 207
Distinct neuroinflammatory profile in post-mortem human Huntington's disease
Silvestroni, A., Faull, R. L. M., Strand, A. D., & Möller, T. (2009) · Neuroreport · doi:10.1097/wnr.0b013e32832e34ee · Cited by 206
Parbo, P., Ismail, R., Hansen, K. V., Amidi, A., Mårup, F. H., Gottrup, H., Brændgaard, H., Eriksson, B. O., Eskildsen, S. F., Lund, T. E., et al. (2017) · Brain · doi:10.1093/brain/awx120 · Cited by 199
Self-citationThalamic metabolism and symptom onset in preclinical Huntington's disease
Feigin, A., Tang, C., Ma, Y., Mattis, P. J., Zgaljardic, D. J., Guttman, M., Paulsen, J. S., DHAWAN, V. M., & Eidelberg, D. (2007) · Brain · doi:10.1093/brain/awm217 · Cited by 198
“, 2004) and as a locus of primary cell loss (Pavese et al., 2006).”
BackgroundPositron emission tomography imaging in dementia
HERHOLZ, K., CARTER, S. F., & JONES, M. (2007) · The British Journal of Radiology · doi:10.1259/bjr/97295129 · Cited by 287
Dodel, R. C., Spottke, A. E., Gerhard, A., Reuß, A., Reinecker, S., Schimke, N., Trenkwalder, C., Sixel‐Döring, F., Herting, B., Kamm, C., et al. (2009) · Movement Disorders · doi:10.1002/mds.22732 · Cited by 187
Self-citationDupont, A.-C., Largeau, B., Santiago Ribeiro, M., Guilloteau, D., Tronel, C., & Arlicot, N. (2017) · International Journal of Molecular Sciences · doi:10.3390/ijms18040785 · Cited by 182
“Furthermore, in manifest HD patients, the correlation between striatal TSPO tracer binding and the severity of motor symptoms (evaluated by the unified Huntington’s disease rating scale) [85,88] could echo the ex vivo relationship found between neuronal loss and density of reactive microglia [84].”
BackgroundFujigaki, H., Yamamoto, Y., & Saito, K. (2016) · Neuropharmacology · doi:10.1016/j.neuropharm.2016.01.011 · Cited by 189
“Microglial activation appears to correlate with HD severity (Pavese et al., 2006), and reactive microglia accumulate in the brain regions associated with the greatest HDassociated neurodegeneration (Sapp et al., 2001).”
Oxidative stress and neurodegeneration: the involvement of iron
Carocci, A., Catalano, A. A., Sinicropi, M. S., & Genchi, G. (2018) · BioMetals · doi:10.1007/s10534-018-0126-2 · Cited by 187
Neuroinflammation in Huntington’s disease
Möller, T. (2010) · Journal of Neural Transmission · doi:10.1007/s00702-010-0430-7 · Cited by 180
Huntington's disease: The current state of research with peripheral tissues
Sassone, J., Colciago, C., Cislaghi, G., Silani, V., & Ciammola, A. (2009) · Experimental Neurology · doi:10.1016/j.expneurol.2009.05.012 · Cited by 175
Hypothalamic involvement in Huntington's disease: an in vivo PET study
Politis, M., Pavese, N., Tai, Y., Tabrizi, S. J., Barker, R., & Piccini, P. P. (2008) · Brain · doi:10.1093/brain/awn244 · Cited by 175
“…Huntington’s disease; positron emission tomography (PET); raclopride; PK11195 Abbreviations: BP=binding potential; PET=positron emission tomography; PK=11C-(R)-PK11195; RAC=11C-raclopride; ROI=region of interest; SD=standard deviation; SPM= statistical parametric mapping; VOI=volume of interest”
BackgroundMethodResultInfluentialSelf-citationCrapser, J. D., Ochaba, J., Soni, N., Reidling, J. C., Thompson, L. M., & Green, K. N. (2020) · Brain · doi:10.1093/brain/awz363 · Cited by 157
“, 2007) as well as clinical stages of the disease (Pavese et al., 2006; Politis et al., 2011).”
BackgroundStack, C., Ho, D. J., Wille, E. J., Calingasan, N. Y., Williams, C. R., Liby, K. T., Sporn, M. B., Dumont, M., & Beal, M. F. (2010) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2010.03.017 · Cited by 175
“In HD patients, microglia activation is evident in the neostriatum and cortex, and the level of microglial activation increases with increased neuronal loss and correlates with the severity of the disease [25-27].”
BackgroundImaging Microglial Activation During Neuroinflammation and Alzheimer’s Disease
Venneti, S., Wiley, C. A., & Kofler, J. (2008) · Journal of Neuroimmune Pharmacology · doi:10.1007/s11481-008-9142-2 · Cited by 171
“…2005), HIV infection (Hammoud et al. 2005; Wiley et al. 2006), hepatic encephalopathy (Cagnin et al. 2006b; Iversen et al. 2006), Huntington’s disease (Pavese et al. 2006), pre-symptomatic Huntington’s disease (in carriers; Tai et al. 2007), intractable epilepsy (Kumar et al. 2008),…”
BackgroundMechanism and therapeutic potential of targeting cGAS-STING signaling in neurological disorders
Huang, Y., Liu, B., Sinha, S. C., Amin, S., & Gan, L. (2023) · Molecular Neurodegeneration · doi:10.1186/s13024-023-00672-x · Cited by 164
Therapeutic approaches to Huntington disease: from the bench to the clinic
Caron, N. S., Dorsey, E. R., & Hayden, M. R. (2018) · Nature Reviews Drug Discovery · doi:10.1038/nrd.2018.133 · Cited by 161
Free Radicals in Human Health and Disease
Yadav, U. C. S., & Rani, V. (2014) · Springer eBooks · doi:10.1007/978-81-322-2035-0 · Cited by 163
“The weaker signals from striatum’s subthalamic nuclei thus cause reduced initiation and modulation of movement, resulting in the characteristic movements of the disorder [ 91 , 93 ].”
BackgroundImaging insights into basal ganglia function, Parkinson's disease, and dystonia
Stoessl, A. J., Lehéricy, S., & Strafella, A. P. (2014) · The Lancet · doi:10.1016/s0140-6736(14)60041-6 · Cited by 158
From Pathogenesis to Therapeutics: A Review of 150 Years of Huntington’s Disease Research
Jiang, A., Handley, R. R., Lehnert, K., & Snell, R. G. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms241613021 · Cited by 174
The Role of the Immune System in Huntington’s Disease
Ellrichmann, G., Reick, C., Saft, C., & Linker, R. A. (2013) · Clinical and Developmental Immunology · doi:10.1155/2013/541259 · Cited by 155
“In this paradigm,microglial activation correlates with the severity of disease progression and with loss of dopamine D2 receptor binding sites [44, 45].”
BackgroundMicroglial activation in the pathogenesis of Huntington's disease
Yang, H.-M., Yang, S., Huang, S.-S., Tang, B.-S., & Guo, J.-F. (2017) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2017.00193 · Cited by 151
“Indeed, in vivo positron emission tomography (PET) studies revealed that microglial activation was significant in affected HD brain regions and this pathology was more pronounced in more severe cases of HD (Pavese et al., 2006; Politis et al., 2011).”
BackgroundCyclic GMP-AMP synthase promotes the inflammatory and autophagy responses in Huntington disease
Sharma, M., Rajendrarao, S., Shahani, N., Ramírez-Jarquín, U. N., & Subramaniam, S. (2020) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2002144117 · Cited by 155
“The number of microglia, a cellular indicator of inflammation, has been shown to be increased in the striatum of HD animal models, cell culture models, and HD patients (9 – 11).”
Politis, M., Lahiri, N., Niccolini, F., Su, P., Wu, K., Giannetti, P., Scahill, R. I., Turkheimer, F., Tabrizi, S. J., & Piccini, P. P. (2015) · Neurobiology of Disease · doi:10.1016/j.nbd.2015.08.011 · Cited by 156
“…have demonstrated in vivo significantmicroglial activation in the striatumand cortical regions of premanifest and manifest HD gene carriers, which was associated with loss of dopamine D2 receptors and symptom severity (Pavese et al., 2006; Tai et al., 2007; Politis et al., 2008, 2011).”
BackgroundSelf-citationPlasma inflammatory biomarkers for Huntington’s disease patients and mouse model
Chang, K., Wu, Y., Chen, Y., & Chen, C. (2014) · Brain Behavior and Immunity · doi:10.1016/j.bbi.2014.09.011 · Cited by 152
“…(Bjorkqvist et al., 2008; Dalrymple et al., 2007; Hsiao et al., 2013, 2014; Moller, 2010), and microglial activation (Hsiao and Chern, 2010; Pavese et al., 2006; Sapp et al., 2001; Shin et al., 2005; Tai et al., 2007) have been shown to play important roles in the pathogenesis of…”
BackgroundNeuroimaging Biomarkers of Neurodegenerative Diseases and Dementia
Risacher, S. L., & Saykin, A. J. (2013) · Seminars in Neurology · doi:10.1055/s-0033-1359312 · Cited by 155
Simmons, D. A., Belichenko, N. P., Yang, T., Condon, C., Monbureau, M., Shamloo, M., Jing, D., Massa, S. M., & Longo, F. M. (2013) · Journal of Neuroscience · doi:10.1523/jneurosci.1310-13.2013 · Cited by 145
“LM22A-4 decreases inflammation in the striatum of R6/2 mice Inflammatory reactions, specifically microglial activation and/or cytokine release, have been described in brains of HD patients (Sapp et al., 2001; Pavese et al., 2006; Simmons et al., 2007; Silvestroni et al., 2009) and mouse models (Bjorkqvist et al.”
BackgroundDurrenberger, P. F., Fernando, F. S., Kashefi, S. N., Bonnert, T. P., Seilhean, D., Nait‐Oumesmar, B., Schmitt, A., Gebicke‐Haerter, P. J., Falkai, P., Grünblatt, E., et al. (2014) · Journal of Neural Transmission · doi:10.1007/s00702-014-1293-0 · Cited by 144
Analysis of potential transcriptomic biomarkers for Huntington's disease in peripheral blood
Runne, H., Kuhn, A., Wild, E. J., Pratyaksha, W., Kristiansen, M., Isaacs, J. D., Régulier, E., Delorenzi, M., Tabrizi, S. J., & Luthi‐Carter, R. (2007) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0703652104 · Cited by 137
Quinti, L., Dayalan Naidu, S., Träger, U., Chen, X., Kegel-Gleason, K., Llères, D., Connolly, C., Chopra, V., Low, C., Moniot, S., et al. (2017) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1614943114 · Cited by 140
Positron Emission Tomography Imaging of Neuroinflammation
Cagnin, A., Kassiou, M., Meikle, S. R., & Banati, R. B. (2007) · Neurotherapeutics · doi:10.1016/j.nurt.2007.04.006 · Cited by 137
“compared with healthy controls has been recently published.(52) In that study, [(11)C](R)-PK11195 binding corre-”
Imaging microglial activation in Huntington's disease
Tai, Y., Pavese, N., Gerhard, A., Tabrizi, S. J., Barker, R. A., Brooks, D. J., & Piccini, P. P. (2006) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2006.10.029 · Cited by 137
Self-citationThe role of microglia in human disease: therapeutic tool or target?
Cartier, N., Lewis, C. B., Zhang, R., & Rossi, F. M. V. (2014) · Acta Neuropathologica · doi:10.1007/s00401-014-1330-y · Cited by 142
“while it has been widely thought that the mechanism underlying HD pathology is likely cell autonomous and dependent of neuronal expression of the protein, microglia activation takes place prior to the onset of overt symptoms, its accumulation in the brains of Huntington patients takes place early during disease progre…”
BackgroundNeuropeptide Y (NPY) as a therapeutic target for neurodegenerative diseases
Duarte‐Neves, J., de Almeida, L. P., & Cavadas, C. (2016) · Neurobiology of Disease · doi:10.1016/j.nbd.2016.07.022 · Cited by 144
“…patients (Gerhard et al., 2006; Ouchi et al., 2005) and PD rat model (Cicchetti et al., 2002), in the striatum and cerebral cortex in HD patients (Pavese et al., 2006) and HD presymptomatic gene carriers (Tai et al., 2007) and in pons and globus pallidus of MJD patients (Evert et al., 2001;…”
BackgroundDetermination of [ 11 C]PBR28 Binding Potential in vivo: A First Human TSPO Blocking Study
Owen, D. R., Guo, Q., Kalk, N. J., Colasanti, A., Kalogiannopoulou, D., Dimber, R., Lewis, Y. L., Libri, V., Barletta, J., Ramada‐Magalhaes, J., et al. (2014) · Journal of Cerebral Blood Flow & Metabolism · doi:10.1038/jcbfm.2014.46 · Cited by 134
Franciosi, S., Ryu, J. K., Shim, Y. A., Hill, A. M., Connolly, C., Hayden, M. R., McLarnon, J. G., & Leavitt, B. R. (2011) · Neurobiology of Disease · doi:10.1016/j.nbd.2011.09.003 · Cited by 131
“Similarly, a correlation between increased microglial activation with HD progression has also been documented (Pavese et al., 2006).”
BackgroundBartels, A. L., & Leenders, K. L. (2007) · Movement Disorders · doi:10.1002/mds.21552 · Cited by 127
Iron dysregulation in Huntington's disease
Müller, M., & Leavitt, B. R. (2014) · Journal of Neurochemistry · doi:10.1111/jnc.12739 · Cited by 123
“Neuronal loss and astrogliosis proceed in a dorsomedial to ventrolateral direction resulting in striatal atrophy and an enlargement of lateral ventricles that associates with subclinical progression of disease (Albin and Tagle 1995; Pavese et al. 2006).”
BackgroundInfluentialKynurenine 3-Monooxygenase: An Influential Mediator of Neuropathology
Parrott, J. M., & O’Connor, J. C. (2015) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2015.00116 · Cited by 125
(2018) · Handbook of Clinical Neurology · doi:10.1016/b978-0-444-63233-3.00017-8 · Cited by 122
Takeuchi, H., & Suzumura, A. (2014) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2014.00189 · Cited by 127
“CELLULAR NEUROSCIENCE REVIEW ARTICLE published: 02 September 2014 doi: 10.3389/fncel.2014.00189 Gap junctions and hemichannels composed of connexins: potential therapeutic targets for neurodegenerative diseases”
InfluentialGuo, Q., Colasanti, A., Owen, D. R., Onega, M., Kamalakaran, A., Bennacef, I., Matthews, P. M., Rabiner, E. A., Turkheimer, F., & Gunn, R. N. (2013) · Journal of Nuclear Medicine · doi:10.2967/jnumed.113.121020 · Cited by 120
Self-citationVMAT2 and dopamine neuron loss in a primate model of Parkinson’s disease
Chen, M., Kuwabara, H., Zhou, Y., Adams, R. J., Brašić, J. R., McGlothan, J. L., Verina, T., Burton, N. C., Alexander, M., Kumar, A., et al. (2007) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2007.05108.x · Cited by 119
“This explanation is consistent with the finding that in Huntington’s disease, a neurodegenerative condition in which there is frank loss of neuronal cell bodies intrinsic to the striatum, there is a very robust TSPO response that can be measured by PET (Pavese et al. 2006).”
ResultVenneti, S., Wang, G., Nguyen, J., & Wiley, C. A. (2008) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e318188b204 · Cited by 122
“[(11)C](R)-PK11195 shows increased brain retention in the comparison of control human subjects to varying extents with patients with Alzheimer disease (AD) (3); amyotrophic lateral sclerosis (ALS) (4); multiple sclerosis (MS) (5Y7); Parkinson disease (8, 9); frontotemporal dementia (FTD) (10); corticobasal degeneratio…”
Phagocyte dysfunction, tissue aging and degeneration
Li, W. (2013) · Ageing Research Reviews · doi:10.1016/j.arr.2013.05.006 · Cited by 116
Neuroinflammation in Huntington’s Disease: A Starring Role for Astrocyte and Microglia
Saba, J., Couselo, F. L., Bruno, J., Carniglia, L., Durand, D., Lasaga, M., & Caruso, C. (2022) · Current Neuropharmacology · doi:10.2174/1570159x19666211201094608 · Cited by 117
“Microglial activation correlates with disease severity in HD patients [30], and can be detected before symptoms appear [31].”
The contribution of glial cells to Huntington's disease pathogenesis
Wilton, D. K., & Stevens, B. (2020) · Neurobiology of Disease · doi:10.1016/j.nbd.2020.104963 · Cited by 112
“pre-symptomatic HD patients that microglia are at the very least associated with and responding to some of the earliest pathological events in HD (Bjorkqvist et al., 2008; Chang et al., 2015a; Pavese et al., 2006; Politis et al., 2015; Politis et al., 2011; Rodrigues et al., 2016; Tai et al., 2007).”
BackgroundImaging of microglia in patients with neurodegenerative disorders
Politis, M. (2012) · Frontiers in Pharmacology · doi:10.3389/fphar.2012.00096 · Cited by 111
“The cortical Pavese et al. (2006) 11 manifest HD patients, 10 NC 11C-PK11195 Significantly increased 11C-PK11195 BPND in patients than controls Increased 11C-PK11195 uptake correlated positively with disease severity Tai et al. (2007) 11 premanifest HD subjects, 10 NC 11C-PK11195 Significantly…”
BackgroundTranslocator Protein 18 kDa Negatively Regulates Inflammation in Microglia
Bae, K.-R., Shim, H.-J., Balu, D., Kim, S. R., & Yu, S. (2014) · Journal of Neuroimmune Pharmacology · doi:10.1007/s11481-014-9540-6 · Cited by 124
“…Edison et al. 2008; Yasuno et al. 2008), fronto-temporal dementia (Cagnin et al. 2004), multiple sclerosis (Vowinckel et al. 1997; Versijpt et al. 2005), Huntington's disease (Pavese et al. 2006), amyotrophic lateral sclerosis (Turner et al. 2004) and PD (Langston et al. 1999; Ouchi et al. 2005).”
BackgroundScuderi, S. A., Ardizzone, A., Paterniti, I., Esposito, E., & Campolo, M. (2020) · Antioxidants · doi:10.3390/antiox9070630 · Cited by 114
“Many studies suggest that mHTT promotes the abnormal release of cytokines by activated microglia [48,49].”
BackgroundKöfalvi, A. (2007) · Springer eBooks · doi:10.1007/978-0-387-74349-3 · Cited by 110
Doorduin, J., de Vries, E. F. J., Dierckx, R. A. J. O., & Klein, H. (2008) · Current Pharmaceutical Design · doi:10.2174/138161208786549443 · Cited by 107
Menze, E. T., Tadros, M. G., Abdel-Tawab, A. M., & Khalifa, A. E. (2012) · NeuroToxicology · doi:10.1016/j.neuro.2012.07.007 · Cited by 107
Jang, M., & Cho, I. (2015) · Molecular Neurobiology · doi:10.1007/s12035-015-9230-2 · Cited by 107
“Microglial activation has been correlated with the severity of HD by a clinical and positron emission tomography study [42].”
MethodSavage, J. C., St‐Pierre, M., Carrier, M., Hajj, H. E., Novak, S. W., Sánchez, M. G., Cicchetti, F., & Tremblay, M. (2020) · Journal of Neuroinflammation · doi:10.1186/s12974-020-01782-9 · Cited by 103
Innate Immunity: A Common Denominator between Neurodegenerative and Neuropsychiatric Diseases
Novellino, F., Saccà, V., Donato, A., Zaffino, P., Spadea, M. F., Vismara, M. F. M., Arcidiacono, B., Malara, N., Presta, I., & Donato, G. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21031115 · Cited by 107
“In addition, microglial activation was found to be related to both disease progression and the depletion of terminals binding the dopamine D2 receptor [135,136].”
BackgroundCurrent status of PET imaging in Huntington’s disease
Pagano, G., Niccolini, F., & Politis, M. (2016) · European Journal of Nuclear Medicine and Molecular Imaging · doi:10.1007/s00259-016-3324-6 · Cited by 102
“Increased activation of striatal and cortical microglia has been found in manifest HD gene carriers [51] and in premanifest HD gene carriers and was associated with loss of striatal D2 receptors ([(11)C]raclopride binding) [52].”
BackgroundMethod
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
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
