{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T02:59:19Z","timestamp":1788317959595,"version":"build-2803163510"},"reference-count":24,"publisher":"Wiley","issue":"23-24","license":[{"start":{"date-parts":[[2012,12,12]],"date-time":"2012-12-12T00:00:00Z","timestamp":1355270400000},"content-version":"vor","delay-in-days":11,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["analyticalsciencejournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Proteomics"],"published-print":{"date-parts":[[2012,12]]},"abstract":"<jats:p>Sporadic <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>reutzfeldt\u2013<jats:styled-content style=\"fixed-case\">J<\/jats:styled-content>akob disease (s<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>) is characterized by wide clinical and pathological variability, which is mainly influenced by the conformation of the misfolded prion protein, and by the methionine and valine polymorphism at codon 129 of the prion protein gene. This heterogeneity likely implies differences in the molecular cascade that leads to the development of certain disease phenotypes. In this study, we investigated the proteome of the frontal cortex of patients with the two most common s<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> subtypes (<jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>1 and <jats:styled-content style=\"fixed-case\">VV<\/jats:styled-content>2) using 2<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">DIGE<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">MS<\/jats:styled-content>. Analysis of 2<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content> maps revealed that 46 proteins are differentially expressed in the s<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>. Common differential expression was detected for seven proteins, four showed opposite direction of differential expression, and the remaining ones displayed subtype\u2010specific alteration. The highest number of differentially expressed proteins was associated with signal transduction and neuronal activity. Moreover, functional groups of proteins involved in cell cycle and death, as well as in structure and motility included subtype\u2010specific expressed proteins exclusively. The expression of <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>ab <jats:styled-content style=\"fixed-case\">GDP<\/jats:styled-content> dissociation inhibitor alpha, which regulates <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>ab3a\u2010mediated neurotransmitter release, was affected in both s<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> subtypes that were analyzed. Therefore, we also investigated as to whether <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>ab3a recycling is altered. Indeed, we found an accumulation of the membrane\u2010associated form, thus the active one, which suggests that dysfunction of the <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>ab3a\u2010mediated exocytosis might be implicated in s<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> pathology.<\/jats:p>","DOI":"10.1002\/pmic.201200201","type":"journal-article","created":{"date-parts":[[2012,10,16]],"date-time":"2012-10-16T04:32:03Z","timestamp":1350361923000},"page":"3610-3620","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Sporadic <scp>C<\/scp>reutzfeldt\u2013<scp>J<\/scp>akob disease subtype\u2010specific alterations of the brain proteome: Impact on <scp>R<\/scp>ab3a recycling"],"prefix":"10.1002","volume":"12","author":[{"given":"Joanna","family":"Gawinecka","sequence":"first","affiliation":[{"name":"National Reference Center for TSE Surveillance Medical Center Georg\u2010August University  Goettingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franco","family":"Cardone","sequence":"additional","affiliation":[{"name":"Department of Cell Biology and Neurosciences Istituto Superiore di Sanit\u00e0  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdul R.","family":"Asif","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry Medical Center Georg\u2010August University  Goettingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"De Pascalis","sequence":"additional","affiliation":[{"name":"Department of Cell Biology and Neurosciences Istituto Superiore di Sanit\u00e0  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wiebke M.","family":"Wemheuer","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Medical Center Georg\u2010August University  Goettingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walter J.","family":"Schulz\u2010Schaeffer","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Medical Center Georg\u2010August University  Goettingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Pocchiari","sequence":"additional","affiliation":[{"name":"Department of Cell Biology and Neurosciences Istituto Superiore di Sanit\u00e0  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inga","family":"Zerr","sequence":"additional","affiliation":[{"name":"National Reference Center for TSE Surveillance Medical Center Georg\u2010August University  Goettingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,12,12]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1002\/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W","article-title":"Classification of sporadic Creutzfeldt\u2013Jakob disease based on molecular and phenotypic analysis of 300 subjects","volume":"46","author":"Parchi P.","year":"1998","journal-title":"Ann. 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