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This was driven partly by the novelty of the prion hypothesis, which sought to provide an explanation for the pathogenesis of transmissible spongiform encephalopathies, involving a unique epigenetic mechanism, and partly by events in the <jats:styled-content style=\"fixed-case\">UK<\/jats:styled-content>, where an outbreak of a new prion disease in cattle (bovine spongiform encephalopathy or <jats:styled-content style=\"fixed-case\">BSE<\/jats:styled-content>) potentially exposed a large section of the <jats:styled-content style=\"fixed-case\">UK<\/jats:styled-content> population to prion infectivity through a dietary route. The numbers of cases of the resultant novel disease variant <jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> (v<jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>), have so far been limited and peaked in the <jats:styled-content style=\"fixed-case\">UK<\/jats:styled-content> in the year 2000 and have subsequently declined. However, the effects of <jats:styled-content style=\"fixed-case\">BSE<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">vCJD<\/jats:styled-content> have been far\u2010reaching. The estimated prevalence of <jats:styled-content style=\"fixed-case\">vCJD<\/jats:styled-content> infection in the <jats:styled-content style=\"fixed-case\">UK<\/jats:styled-content> is substantially higher than the numbers of clinical cases would suggest, posing a difficult dilemma for those involved in blood transfusion, tissue transplantation and cellular therapies. The clinico\u2010pathological phenotype of human prion diseases has come under close scrutiny and molecular classification systems have been developed to account for the different diseases and their phenotypic spectra. Moreover, enhanced human and animal surveillance and better diagnostic tools have identified new human and animal prion diseases. Lastly, as the prion hypothesis has gained widespread acceptance, the concepts involved have been applied to other areas, including extra\u2010chromosomal inheritance in fungi, long\u2010term potentiation in memory formation and the spread of molecular pathology in diverse conditions, such as <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>lzheimer's disease, <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>arkinson's disease and amyotrophic lateral sclerosis. Studies at the molecular and cellular level have helped to provide a better understanding of human prion diseases, aided pathological diagnosis and helped inform public health decision\u2010making.<\/jats:p>","DOI":"10.1111\/neup.12016","type":"journal-article","created":{"date-parts":[[2013,1,21]],"date-time":"2013-01-21T04:47:16Z","timestamp":1358743636000},"page":"221-236","source":"Crossref","is-referenced-by-count":32,"title":["Human prion diseases: Molecular, cellular and population biology"],"prefix":"10.1111","volume":"33","author":[{"given":"Mark W.","family":"Head","sequence":"first","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit Centre for Clinical Brain Sciences School of Clinical Sciences The University of Edinburgh  Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,1,20]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.6801762"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.20025"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2010.01.008"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1567-1364.2010.00666.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1222951"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1138718"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awq234"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-010-0761-3"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2011.07399.x"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.274.5295.2079"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3181cd7374"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/128_2011_157"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/379339a0"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/16.20.6057"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(98)12075-5"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.1580"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(06)68930-7"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0800190105"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.3201\/eid1806.120116"},{"key":"e_1_2_7_21_1","unstructured":"The National CJD Research & Surveillance Unit (NCJDRSU) The University of Edinburgh. 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