{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T04:57:29Z","timestamp":1761541049531},"reference-count":27,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2011,7,7]],"date-time":"2011-07-07T00:00:00Z","timestamp":1309996800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2012,2]]},"abstract":"<jats:p>Human genetic Creutzfeldt\u2010Jakob disease (gCJD; one of the prion diseases) is caused by point mutations and insertions in the prion protein gene (<jats:italic>PRNP<\/jats:italic>). Previously we have reported a Chinese gCJD case with a substitution of valine (V) for glycine (G) at codon 114. To investigate the detailed pathogenic and pathologic characteristics of G114V gCJD, 10 different brain regions were thoroughly analyzed. PrP\u2010specific Western blots and immunohistochemical (IHC) assays identified larger amounts of PrP<jats:sup>Sc<\/jats:sup> in the regions of brain cortex. Assays of the transcriptions of PrP\u2010specific mRNA by RT\u2010PCR and real\u2010time PCR showed comparable levels in 10 brain regions. In line with the distribution of PrP<jats:sup>Sc<\/jats:sup>, typical vacuolations in brains, markedly in four cortex regions, were detected. Contrast to the distributing features of spongiform and of PrP<jats:sup>Sc<\/jats:sup>, massive gliosis was detected in all brain regions by GFAP\u2010specific IHC tests. Moreover, two\u2010dimensional gel immunoblots found three major sets of PrP<jats:sup>Sc<\/jats:sup> spots, indicating that PrP<jats:sup>Sc<\/jats:sup> in brain tissues was a mixture of molecules with different biochemical properties. The data here provide the pathogenic and neuropathological features of G114V gCJD.<\/jats:p>","DOI":"10.1111\/j.1440-1789.2011.01237.x","type":"journal-article","created":{"date-parts":[[2011,7,7]],"date-time":"2011-07-07T08:42:23Z","timestamp":1310028143000},"page":"51-59","source":"Crossref","is-referenced-by-count":15,"title":["The diversities of PrP<sup>Sc<\/sup> distributions and pathologic changes in various brain regions from a Chinese patient with G114V genetic 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