{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T05:51:54Z","timestamp":1784872314424,"version":"3.55.0"},"reference-count":57,"publisher":"Frontiers Media SA","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Front. Aging Neurosci."],"DOI":"10.3389\/fnagi.2014.00198","type":"journal-article","created":{"date-parts":[[2014,8,4]],"date-time":"2014-08-04T15:31:36Z","timestamp":1407166296000},"source":"Crossref","is-referenced-by-count":66,"title":["Subtype and Regional-Specific Neuroinflammation in Sporadic Creutzfeldt\u00e2\u20ac\u201cJakob Disease"],"prefix":"10.3389","volume":"6","author":[{"given":"Franc","family":"Llorens","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene","family":"L\u00c3\u00b3pez-Gonz\u00c3\u00a1lez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katrin","family":"Th\u00c3\u00bcne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarita","family":"Carmona","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saima","family":"Zafar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Andr\u00c3\u00a9oletti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inga","family":"Zerr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isidre","family":"Ferrer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2014,8,4]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"156","DOI":"10.1126\/science.1227901","article-title":"Microglia: scapegoat, saboteur, or something else?","volume":"339","author":"Aguzzi","year":"2013","journal-title":"Science"},{"key":"B2","doi-asserted-by":"publisher","first-page":"e54454","DOI":"10.1371\/journal.pone.0054454","article-title":"Sod1 deficiency reduces incubation time in mouse models of prion disease","volume":"8","author":"Akhtar","year":"2013","journal-title":"PLoS ONE"},{"key":"B3","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.pneurobio.2008.01.004","article-title":"Neurotransmitter, peptide and cytokine processes in relation to depressive disorder: comorbidity between depression and neurodegenerative disorders","volume":"85","author":"Anisman","year":"2008","journal-title":"Prog. Neurobiol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"369","DOI":"10.1016\/j.neuroscience.2013.06.034","article-title":"Dysregulation of brain olfactory and taste receptors in AD, PSP and CJD, and AD-related model","volume":"248C","author":"Ansoleaga","year":"2013","journal-title":"Neuroscience"},{"key":"B5","doi-asserted-by":"publisher","first-page":"208","DOI":"10.1001\/archneurol.2008.533","article-title":"Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants","volume":"66","author":"Appleby","year":"2009","journal-title":"Arch. Neurol."},{"key":"B6","doi-asserted-by":"publisher","first-page":"4532","DOI":"10.1074\/jbc.M113.502690","article-title":"Analysis of the hippocampal proteome in me7 prion disease reveals a predominant astrocytic signature and highlights the brain-restricted production of clusterin in chronic neurodegeneration","volume":"289","author":"Asuni","year":"2014","journal-title":"J. Biol. Chem."},{"key":"B7","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1038\/nrn2038","article-title":"Microglia-mediated neurotoxicity: uncovering the molecular mechanisms","volume":"8","author":"Block","year":"2007","journal-title":"Nat. Rev. Neurosci."},{"key":"B8","doi-asserted-by":"publisher","first-page":"999","DOI":"10.1016\/j.molimm.2006.03.007","article-title":"Role of complement in neurodegeneration and neuroinflammation","volume":"44","author":"Bonifati","year":"2007","journal-title":"Mol. Immunol."},{"key":"B9","doi-asserted-by":"publisher","first-page":"2605","DOI":"10.1099\/vir.0.19137-0","article-title":"Inducible cytokine gene expression in the brain in the ME7\/CV mouse model of scrapie is highly restricted, is at a strikingly low level relative to the degree of gliosis and occurs only late in disease","volume":"84","author":"Brown","year":"2003","journal-title":"J. Gen. Virol."},{"key":"B10","doi-asserted-by":"crossref","first-page":"2383","DOI":"10.1128\/JVI.68.4.2383-2387.1994","article-title":"Activation of cerebral cytokine gene expression and its correlation with onset of reactive astrocyte and acute-phase response gene expression in scrapie","volume":"68","author":"Campbell","year":"1994","journal-title":"J. Virol."},{"key":"B11","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1016\/0896-6273(94)90159-7","article-title":"Tumor necrosis factors protect neurons against metabolic-excitotoxic insults and promote maintenance of calcium homeostasis","volume":"12","author":"Cheng","year":"1994","journal-title":"Neuron"},{"key":"B12","doi-asserted-by":"publisher","first-page":"a006833","DOI":"10.1101\/cshperspect.a006833","article-title":"Prions","volume":"3","author":"Colby","year":"2011","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"B13","doi-asserted-by":"publisher","first-page":"2147","DOI":"10.1046\/j.1460-9568.2003.02662.x","article-title":"Synaptic changes characterize early behavioural signs in the ME7 model of murine prion disease","volume":"17","author":"Cunningham","year":"2003","journal-title":"Eur. J. Neurosci."},{"key":"B14","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1385\/JMN:20:1:25","article-title":"Cyclooxygenase-1 and -2 in brains of patients who died with sporadic Creutzfeldt-Jakob disease","volume":"20","author":"Deininger","year":"2003","journal-title":"J. Mol. Neurosci."},{"key":"B15","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1038\/nn2028","article-title":"A versatile prion replication assay in organotypic brain slices","volume":"11","author":"Falsig","year":"2008","journal-title":"Nat. Neurosci."},{"key":"B16","doi-asserted-by":"publisher","first-page":"391","DOI":"10.2174\/187152711794653751","article-title":"Targeting TNF-alpha to elucidate and ameliorate neuroinflammation in neurodegenerative diseases","volume":"10","author":"Frankola","year":"2011","journal-title":"CNS Neurol. Disord. Drug Targets"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12137","article-title":"Deregulation and expression of cytokines and mediators of the immune response in Parkinson\u00e2\u20ac\u2122s disease brain is region dependent","author":"Garcia-Esparcia","year":"2014","journal-title":"Brain Pathol."},{"key":"B18","doi-asserted-by":"publisher","first-page":"449","DOI":"10.1111\/j.1750-3639.1998.tb00167.x","article-title":"Role of microglia in neuronal cell death in prion disease","volume":"8","author":"Giese","year":"1998","journal-title":"Brain Pathol."},{"key":"B19","doi-asserted-by":"publisher","first-page":"2481","DOI":"10.1523\/JNEUROSCI.4440-12.2013","article-title":"Regulation of microglial proliferation during chronic neurodegeneration","volume":"33","author":"Gomez-Nicola","year":"2013","journal-title":"J. Neurosci."},{"key":"B20","doi-asserted-by":"publisher","first-page":"918","DOI":"10.1016\/j.cell.2007.07.009","article-title":"NF-kappaB is a negative regulator of IL-1beta secretion as revealed by genetic and pharmacological inhibition of IKKbeta","volume":"130","author":"Greten","year":"2007","journal-title":"Cell"},{"key":"B21","doi-asserted-by":"crossref","first-page":"470S","DOI":"10.1093\/ajcn\/83.2.470S","article-title":"Inflammation and neurodegenerative diseases","volume":"83","author":"Griffin","year":"2006","journal-title":"Am. J. Clin. Nutr."},{"key":"B22","doi-asserted-by":"publisher","first-page":"526","DOI":"10.1007\/BF00294180","article-title":"Relationship of microglia and scrapie amyloid-immunoreactive plaques in kuru, Creutzfeldt-Jakob disease and Gerstmann-Straussler syndrome","volume":"87","author":"Guiroy","year":"1994","journal-title":"Acta Neuropathol."},{"key":"B23","doi-asserted-by":"publisher","first-page":"1545","DOI":"10.1099\/vir.0.83622-0","article-title":"Prion propagation in mice lacking central nervous system NF-kappaB signalling","volume":"89","author":"Julius","year":"2008","journal-title":"J. Gen. Virol."},{"key":"B24","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/S0169-328X(99)00229-6","article-title":"Expression of cytokine genes and increased nuclear factor-kappa B activity in the brains of scrapie-infected mice","volume":"73","author":"Kim","year":"1999","journal-title":"Brain Res. Mol. Brain Res."},{"key":"B25","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/S0378-1119(02)00398-0","article-title":"Signaling through the JAK\/STAT pathway, recent advances and future challenges","volume":"285","author":"Kisseleva","year":"2002","journal-title":"Gene"},{"key":"B26","doi-asserted-by":"publisher","first-page":"192","DOI":"10.1016\/j.brainres.2010.01.089","article-title":"Distribution of apoptosis-related proteins in sporadic Creutzfeldt-Jakob disease","volume":"1323","author":"Kovacs","year":"2010","journal-title":"Brain Res."},{"issue":"Suppl. B","key":"B27","first-page":"39","article-title":"An outline of the neuropathology of transmissible spongiform encephalopathies (prion diseases)","volume":"42","author":"Liberski","year":"2004","journal-title":"Folia Neuropathol."},{"key":"B28","doi-asserted-by":"publisher","first-page":"383","DOI":"10.4161\/pri.26416","article-title":"PrP mRNA and protein expression in brain and PrP(c) in CSF in Creutzfeldt-Jakob disease MM1 and VV2","volume":"7","author":"Llorens","year":"2013","journal-title":"Prion"},{"key":"B29","doi-asserted-by":"publisher","first-page":"485","DOI":"10.1038\/86562","article-title":"Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie","volume":"7","author":"Mabbott","year":"2001","journal-title":"Nat. Med."},{"key":"B30","doi-asserted-by":"publisher","first-page":"247","DOI":"10.1172\/JCI11916","article-title":"NF-kappaB in neuronal plasticity and neurodegenerative disorders","volume":"107","author":"Mattson","year":"2001","journal-title":"J. Clin. Invest."},{"key":"B31","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1016\/S0074-7742(07)82014-9","article-title":"Cyclooxygenase-2, prostaglandin E2, and microglial activation in prion diseases","volume":"82","author":"Minghetti","year":"2007","journal-title":"Int. Rev. Neurobiol."},{"key":"B32","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1111\/j.1365-2990.1995.tb01097.x","article-title":"Reactive microglia in Creutzfeldt-Jakob disease","volume":"21","author":"Muhleisen","year":"1995","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"B33","doi-asserted-by":"publisher","first-page":"637","DOI":"10.1111\/j.1471-4159.2007.04769.x","article-title":"JAK-STAT signaling pathway mediates astrogliosis in brains of scrapie-infected mice","volume":"103","author":"Na","year":"2007","journal-title":"J. Neurochem."},{"key":"B34","doi-asserted-by":"publisher","first-page":"e72112","DOI":"10.1371\/journal.pone.0072112","article-title":"The toll-like receptor agonist imiquimod is active against prions","volume":"8","author":"Oumata","year":"2013","journal-title":"PLoS ONE"},{"key":"B35","doi-asserted-by":"publisher","first-page":"e1001319","DOI":"10.1371\/journal.ppat.1001319","article-title":"Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice","volume":"7","author":"Padilla","year":"2011","journal-title":"PLoS Pathog."},{"key":"B36","doi-asserted-by":"publisher","first-page":"517","DOI":"10.1007\/s00401-012-1002-8","article-title":"Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA","volume":"124","author":"Parchi","year":"2012","journal-title":"Acta Neuropathol."},{"key":"B37","doi-asserted-by":"publisher","first-page":"224","DOI":"10.1002\/1531-8249(199908)46:2224::AID-ANA123.3.CO;2-N","article-title":"Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects","volume":"46","author":"Parchi","year":"1999","journal-title":"Ann. Neurol."},{"key":"B38","doi-asserted-by":"publisher","first-page":"659","DOI":"10.1007\/s00401-009-0585-1","article-title":"Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification","volume":"118","author":"Parchi","year":"2009","journal-title":"Acta Neuropathol."},{"key":"B39","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1186\/1746-6148-2-37","article-title":"Intracerebral administration of interleukin-12 (IL-12) and IL-18 modifies the course of mouse scrapie","volume":"2","author":"Pasquali","year":"2006","journal-title":"BMC Vet. Res."},{"key":"B40","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1038\/sj.embor.embor731","article-title":"Prion pathogenesis in the absence of Toll-like receptor signalling","volume":"4","author":"Prinz","year":"2003","journal-title":"EMBO Rep."},{"key":"B41","doi-asserted-by":"crossref","first-page":"3620","DOI":"10.1523\/JNEUROSCI.18-10-03620.1998","article-title":"Multiple routes to astrocytic differentiation in the CNS","volume":"18","author":"Rajan","year":"1998","journal-title":"J. Neurosci."},{"key":"B42","doi-asserted-by":"publisher","first-page":"337","DOI":"10.1007\/BF00369445","article-title":"Immunohistochemical study of microglia in the Creutzfeldt-Jakob diseased brain","volume":"86","author":"Sasaki","year":"1993","journal-title":"Acta Neuropathol."},{"key":"B43","doi-asserted-by":"publisher","first-page":"1133","DOI":"10.1172\/JCI200215644","article-title":"Series introduction. JAK-STAT signaling in human disease","volume":"109","author":"Schindler","year":"2002","journal-title":"J. Clin. Invest."},{"key":"B44","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1016\/S0002-9440(10)63331-7","article-title":"Role of interleukin-1 in prion disease-associated astrocyte activation","volume":"165","author":"Schultz","year":"2004","journal-title":"Am. J. Pathol."},{"key":"B45","first-page":"173","article-title":"Detection of PrP(Sc) in subclinical BSE with the paraffin-embedded tissue (PET) blot","volume":"16","author":"Schulz-Schaeffer","year":"2000","journal-title":"Arch. Virol. Suppl."},{"key":"B46","doi-asserted-by":"publisher","first-page":"1289","DOI":"10.1212\/WNL.52.6.1289","article-title":"Heightened intrathecal release of proinflammatory cytokines in Creutzfeldt-Jakob disease","volume":"52","author":"Sharief","year":"1999","journal-title":"Neurology"},{"key":"B47","doi-asserted-by":"publisher","first-page":"216","DOI":"10.1186\/1743-422X-10-216","article-title":"Brain microglia were activated in sporadic CJD but almost unchanged in fatal familial insomnia and G114V genetic CJD","volume":"10","author":"Shi","year":"2013","journal-title":"Virol. J."},{"key":"B48","doi-asserted-by":"publisher","first-page":"2011","DOI":"10.4049\/jimmunol.173.3.2011","article-title":"Cyclooxygenase (COX)-2 inhibitor celecoxib abrogates TNF-induced NF-kappa B activation through inhibition of activation of I kappa B alpha kinase and Akt in human non-small cell lung carcinoma: correlation with suppression of COX-2 synthesis","volume":"173","author":"Shishodia","year":"2004","journal-title":"J. Immunol."},{"key":"B49","doi-asserted-by":"publisher","first-page":"10701","DOI":"10.1128\/JVI.00522-08","article-title":"Accelerated prion disease pathogenesis in Toll-like receptor 4 signaling-mutant mice","volume":"82","author":"Spinner","year":"2008","journal-title":"J. Virol."},{"key":"B50","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1016\/j.jneuroim.2005.10.008","article-title":"Pro- and anti-inflammatory cytokines in the CSF of patients with Creutzfeldt-Jakob disease","volume":"172","author":"Stoeck","year":"2006","journal-title":"J. Neuroimmunol."},{"key":"B51","first-page":"202","article-title":"The brain immune response in human prion diseases. Microglial activation and microglial disease. I. Sporadic Creutzfeldt-Jakob disease","volume":"44","author":"Szpak","year":"2006","journal-title":"Folia Neuropathol."},{"key":"B52","doi-asserted-by":"publisher","first-page":"1777","DOI":"10.1099\/vir.0.2008\/001255-0","article-title":"Genes contributing to prion pathogenesis","volume":"89","author":"Tamg\u00c3\u00bcney","year":"2008","journal-title":"J. Gen. Virol."},{"key":"B53","doi-asserted-by":"publisher","first-page":"13697","DOI":"10.1128\/JVI.78.24.13697-13707.2004","article-title":"Accelerated prion disease in the absence of interleukin-10","volume":"78","author":"Thackray","year":"2004","journal-title":"J. Virol."},{"key":"B54","doi-asserted-by":"publisher","first-page":"11244","DOI":"10.1128\/JVI.01413-09","article-title":"Analysis of protein levels of 24 cytokines in scrapie agent-infected brain and glial cell cultures from mice differing in prion protein expression levels","volume":"83","author":"Tribouillard-Tanvier","year":"2009","journal-title":"J. Virol."},{"key":"B55","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1016\/S0197-4580(01)00236-6","article-title":"The role of cytokines, astrocytes, microglia and apoptosis in Creutzfeldt-Jakob disease","volume":"23","author":"Van","year":"2002","journal-title":"Neurobiol. Aging"},{"key":"B56","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1093\/jnen\/60.2.173","article-title":"Absence of detectable IL-1beta production in murine prion disease: a model of chronic neurodegeneration","volume":"60","author":"Walsh","year":"2001","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"B57","doi-asserted-by":"publisher","first-page":"433","DOI":"10.1006\/exnr.1997.6424","article-title":"PrP deposition, microglial activation, and neuronal apoptosis in murine scrapie","volume":"144","author":"Williams","year":"1997","journal-title":"Exp. Neurol."}],"container-title":["Frontiers in Aging Neuroscience"],"original-title":[],"deposited":{"date-parts":[[2020,8,22]],"date-time":"2020-08-22T22:10:33Z","timestamp":1598134233000},"score":1,"resource":{"primary":{"URL":"http:\/\/journal.frontiersin.org\/article\/10.3389\/fnagi.2014.00198\/abstract"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,8,4]]},"references-count":57,"URL":"https:\/\/doi.org\/10.3389\/fnagi.2014.00198","relation":{},"ISSN":["1663-4365"],"issn-type":[{"value":"1663-4365","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,8,4]]}}}