{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T23:07:05Z","timestamp":1791328025912,"version":"4.3.1"},"reference-count":40,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2011,7,13]],"date-time":"2011-07-13T00:00:00Z","timestamp":1310515200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2011,8]]},"abstract":"<jats:p>B. A. Faucheux, E. Morain, V. Diouron, J.\u2010P. Brandel, D. Salomon, V. Sazdovitch, N. Privat, J.\u2010L. Laplanche, J.\u2010J. Hauw and S. Ha\u00efk (2011) <jats:italic>Neuropathology and Applied Neurobiology<\/jats:italic><jats:bold>37,<\/jats:bold> 500\u2013512<\/jats:p><jats:p> <jats:bold>Quantification of surviving cerebellar granule neurones and abnormal prion protein (PrP<jats:sup>Sc<\/jats:sup>) deposition in sporadic Creutzfeldt\u2013Jakob disease supports a pathogenic role for small PrP<jats:sup>Sc<\/jats:sup> deposits common to the various molecular subtypes<\/jats:bold> <\/jats:p><jats:p> <jats:bold>Aims:<\/jats:bold> Neuronal death is a major neuropathological hallmark in prion diseases. The association between the accumulation of the disease\u2010related prion protein (PrP<jats:sup>Sc<\/jats:sup>) and neuronal loss varies within the wide spectrum of prion diseases and their experimental models. In this study, we investigated the relationships between neuronal loss and PrP<jats:sup>Sc<\/jats:sup> deposition in the cerebellum from cases of the six subtypes of sporadic Creutzfeldt\u2013Jakob disease (sCJD; <jats:italic>n<\/jats:italic>\u2003=\u2003100) that can be determined according to the M129V polymorphism of the human prion protein gene (<jats:italic>PRNP<\/jats:italic>) and PrP<jats:sup>Sc<\/jats:sup> molecular types. <jats:bold>Methods:<\/jats:bold> The numerical density of neurones was estimated with a computer\u2010assisted image analysis system and the accumulation of PrP<jats:sup>Sc<\/jats:sup> deposits was scored. <jats:bold>Results:<\/jats:bold> The scores of PrP<jats:sup>Sc<\/jats:sup> immunoreactive deposits of the punctate type (synaptic type) were correlated with neurone counts \u2013 the higher the score the higher the neuronal loss \u2013 in all sCJD subtypes. Large 5\u2010 to 50\u2010\u00b5m\u2010wide deposits (focal type) were found in sCJD\u2010MV2 and sCJD\u2010VV2 subtypes, and occasionally in a few cases of the other studied groups. By contrast, the highest scores for 5\u2010 to 50\u2010\u00b5m\u2010wide deposits observed in sCJD\u2010MV2 subtype were not associated with higher neuronal loss. In addition, these scores were inversely correlated with neuronal counts in the sCJD\u2010VV2 subtype. <jats:bold>Conclusions:<\/jats:bold> These results support a putative pathogenic role for small PrP<jats:sup>Sc<\/jats:sup> deposits common to the various sCJD subtypes. Furthermore, the observation of a lower loss of neurones associated with PrP<jats:sup>Sc<\/jats:sup> type\u20102 large deposits is consistent with a possible \u2018protective\u2019 role of aggregated deposits in both sCJD\u2010MV2 and sCJD\u2010VV2 subtypes.<\/jats:p>","DOI":"10.1111\/j.1365-2990.2011.01179.x","type":"journal-article","created":{"date-parts":[[2011,3,31]],"date-time":"2011-03-31T08:46:29Z","timestamp":1301561189000},"page":"500-512","source":"Crossref","is-referenced-by-count":20,"title":["Quantification of surviving cerebellar granule neurones and abnormal prion protein (PrP<sup>Sc<\/sup>) deposition in sporadic Creutzfeldt\u2013Jakob disease supports a pathogenic role for small PrP<sup>Sc<\/sup> deposits common to the various molecular subtypes"],"prefix":"10.1111","volume":"37","author":[{"given":"B. 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