{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T15:37:59Z","timestamp":1791128279243,"version":"4.1.0"},"reference-count":97,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2012,5,11]],"date-time":"2012-05-11T00:00:00Z","timestamp":1336694400000},"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":[[2012,6]]},"abstract":"<jats:p>M. W. Head and J. W. Ironside (2012) <jats:italic>Neuropathology and Applied Neurobiology<\/jats:italic><jats:bold>38,<\/jats:bold> 296\u2013310<\/jats:p><jats:p><jats:bold>Creutzfeldt\u2013Jakob disease: prion protein type, disease phenotype and agent strain<\/jats:bold><\/jats:p><jats:p>The human transmissible spongiform encephalopathies or human prion diseases are one of the most intensively investigated groups of rare human neurodegenerative conditions. They are generally held to be unique in terms of their complex epidemiology and phenotypic variability, but they may also serve as a paradigm with which other more common protein misfolding disorders might be compared and contrasted. The clinico\u2010pathological phenotype of human prion diseases appears to depend on a complex interaction between the prion protein genotype of the affected individual and the physico\u2010chemical properties of the neurotoxic and transmissible agent, thought to comprise of misfolded prion protein. A major focus of research in recent years has been to define the phenotypic heterogeneity of the recognized human prion diseases, correlate this with molecular\u2010genetic features and then determine whether this molecular\u2010genetic classification of human prion disease defines the biological properties of the agent as determined by animal transmission studies. This review seeks to survey the field as it currently stands, summarize what has been learned, and explore what remains to be investigated in order to obtain a more complete scientific understanding of prion diseases and to protect public health.<\/jats:p>","DOI":"10.1111\/j.1365-2990.2012.01265.x","type":"journal-article","created":{"date-parts":[[2012,3,7]],"date-time":"2012-03-07T12:45:17Z","timestamp":1331124317000},"page":"296-310","source":"Crossref","is-referenced-by-count":81,"title":["Review: Creutzfeldt\u2013Jakob disease: prion protein type, disease phenotype and agent strain"],"prefix":"10.1111","volume":"38","author":[{"given":"M. W.","family":"Head","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. 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