{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T21:36:14Z","timestamp":1792791374886,"version":"4.5.0"},"reference-count":123,"publisher":"Elsevier","isbn-type":[{"value":"9780128112267","type":"print"}],"license":[{"start":{"date-parts":[[2017,1,1]],"date-time":"2017-01-01T00:00:00Z","timestamp":1483228800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017]]},"DOI":"10.1016\/bs.pmbts.2017.06.014","type":"book-chapter","created":{"date-parts":[[2017,8,7]],"date-time":"2017-08-07T14:31:00Z","timestamp":1502116260000},"page":"181-201","source":"Crossref","is-referenced-by-count":25,"title":["Transmission and Replication of Prions"],"prefix":"10.1016","author":[{"given":"Alba","family":"Mar\u00edn-Moreno","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"Fern\u00e1ndez-Borges","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan C.","family":"Espinosa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Andr\u00e9oletti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan M.","family":"Torres","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/bs.pmbts.2017.06.014_bb0010","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/S0368-1742(18)80015-7","article-title":"Scrapie","volume":"31","author":"M\u2019Fadyean","year":"1918","journal-title":"J Comp Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0015","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1038\/297107a0","article-title":"Scrapie agent: prions or virinos?","volume":"297","author":"Kimberlin","year":"1982","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0020","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/0006-291X(66)90478-5","article-title":"The exceptionally small size of the scrapie agent","volume":"22","author":"Alper","year":"1966","journal-title":"Biochem Biophys Res Commun"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0025","doi-asserted-by":"crossref","first-page":"6675","DOI":"10.1073\/pnas.78.11.6675","article-title":"Scrapie agent contains a hydrophobic protein","volume":"78","author":"Prusiner","year":"1981","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0030","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/0021-9975(67)90039-4","article-title":"Further studies of the infectivity and stability of extracts and homogenates derived from scrapie affected mouse brains","volume":"77","author":"Hunter","year":"1967","journal-title":"J Comp Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0035","doi-asserted-by":"crossref","first-page":"4883","DOI":"10.1021\/bi00562a028","article-title":"Molecular properties, partial purification, and assay by incubation period measurements of the hamster scrapie agent","volume":"19","author":"Prusiner","year":"1980","journal-title":"Biochemistry"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0040","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/0021-9975(67)90039-4","article-title":"Attempts to release the scrapie agent from tissue debris","volume":"88","author":"Hunter","year":"1967","journal-title":"J Comp Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0045","doi-asserted-by":"crossref","first-page":"4606","DOI":"10.1073\/pnas.78.7.4606","article-title":"Thiocyanate and hydroxyl ions inactivate the scrapie agent","volume":"78","author":"Prusiner","year":"1981","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0050","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1126\/science.6801762","article-title":"Novel proteinaceous infectious particles cause scrapie","volume":"216","author":"Prusiner","year":"1982","journal-title":"Science"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0055","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1038\/2151043a0","article-title":"Self-replication and scrapie","volume":"215","author":"Griffith","year":"1967","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0060","doi-asserted-by":"crossref","first-page":"1515","DOI":"10.1126\/science.1675487","article-title":"Molecular biology of prion diseases","volume":"252","author":"Prusiner","year":"1991","journal-title":"Science"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0065","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1002\/jms.1516","article-title":"High-resolution differentiation of transmissible spongiform encephalopathy strains by quantitative n-terminal amino acid profiling (n-taap) of pk-digested abnormal prion protein","volume":"44","author":"Gielbert","year":"2009","journal-title":"J Mass Spectrom"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0070","doi-asserted-by":"crossref","first-page":"13363","DOI":"10.1073\/pnas.95.23.13363","article-title":"Prions","volume":"95","author":"Prusiner","year":"1998","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0075","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1016\/0092-8674(86)90662-8","article-title":"Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene","volume":"46","author":"Basler","year":"1986","journal-title":"Cell"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0080","doi-asserted-by":"crossref","first-page":"2591","DOI":"10.1002\/j.1460-2075.1986.tb04539.x","article-title":"The major polypeptide of scrapie-associated fibrils (SAF) has the same size, charge distribution and N-terminal protein sequence as predicted for the normal brain protein (PrP)","volume":"5","author":"Hope","year":"1986","journal-title":"EMBO J"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0085","doi-asserted-by":"crossref","first-page":"1991","DOI":"10.1021\/bi00059a016","article-title":"Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing","volume":"32","author":"Stahl","year":"1993","journal-title":"Biochemistry"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0090","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/S0014-5793(97)00920-4","article-title":"NMR characterization of the full-length recombinant murine prion protein, mPrP (23\u2212231)","volume":"413","author":"Riek","year":"1997","journal-title":"FEBS Lett"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0095","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1038\/nsb0901-770","article-title":"Crystal structure of the human prion protein reveals a mechanism for oligomerization","volume":"8","author":"Knaus","year":"2001","journal-title":"Nat Struct Biol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0100","first-page":"12","article-title":"The structural architecture of an infectious mammalian prion using electron cryomicroscopy","volume":"8","author":"V\u00e1zquez-Fern\u00e1ndez","year":"2016","journal-title":"PLoS Pathog"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0105","doi-asserted-by":"crossref","first-page":"60","DOI":"10.4161\/pri.28368","article-title":"The structure of the infectious prion protein: experimental data and molecular models","volume":"8","author":"Requena","year":"2014","journal-title":"Prion"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0110","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1038\/356577a0","article-title":"Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein","volume":"356","author":"Bueler","year":"1992","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0115","doi-asserted-by":"crossref","first-page":"1339","DOI":"10.1016\/0092-8674(93)90360-3","article-title":"Mice devoid of PrP are resistant to scrapie","volume":"13","author":"Bueler","year":"1993","journal-title":"Cell"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0120","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1083\/jcb.110.3.743","article-title":"Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells","volume":"110","author":"Borchelt","year":"1990","journal-title":"J Cell Biol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0125","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1016\/0092-8674(90)90134-Z","article-title":"Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication","volume":"63","author":"Prusiner","year":"1990","journal-title":"Cell"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0130","doi-asserted-by":"crossref","first-page":"35245","DOI":"10.1074\/jbc.M603964200","article-title":"Ultra-efficient replication of infectious prions by automated protein misfolding cyclic amplification","volume":"281","author":"Saa","year":"2006","journal-title":"J Biol Chem"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0135","doi-asserted-by":"crossref","first-page":"26873","DOI":"10.1074\/jbc.M503973200","article-title":"Protease-resistant prion protein amplification reconstituted with partially purified substrates and synthetic polyanions","volume":"280","author":"Deleault","year":"2005","journal-title":"J Biol Chem"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0140","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1038\/nmeth1066","article-title":"Ultrasensitive detection of scrapie prion protein using seeded conversion of recombinant prion protein","volume":"4","author":"Atarashi","year":"2007","journal-title":"Nat Methods"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0145","doi-asserted-by":"crossref","first-page":"1132","DOI":"10.1126\/science.1183748","article-title":"Generating a prion with bacterially expressed recombinant prion protein","volume":"327","author":"Wang","year":"2010","journal-title":"Science"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0150","doi-asserted-by":"crossref","first-page":"3193","DOI":"10.1093\/emboj\/18.12.3193","article-title":"Specific binding of normal prion protein to the scrapie form via a localized domain initiates its conversion to the protease-resistant state","volume":"18","author":"Horiuchi","year":"1999","journal-title":"EMBO J"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0155","doi-asserted-by":"crossref","first-page":"6245","DOI":"10.1128\/JVI.73.8.6245-6250.1999","article-title":"Species-independent inhibition of abnormal prion protein (PrP) formation by a peptide containing a conserved PrP sequence","volume":"73","author":"Chabry","year":"1999","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0160","doi-asserted-by":"crossref","first-page":"5836","DOI":"10.1073\/pnas.110523897","article-title":"Interactions between heterologous forms of prion protein: binding, inhibition of conversion, and species barriers","volume":"97","author":"Horiuchi","year":"2000","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0165","doi-asserted-by":"crossref","first-page":"10106","DOI":"10.1128\/JVI.75.21.10106-10112.2001","article-title":"Long-term subclinical carrier state precedes scrapie replication and adaptation in a resistant species: analogies to bovine spongiform encephalopathy and variant Creutzfeldt\u2013Jakob disease in humans","volume":"75","author":"Race","year":"2001","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0170","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1016\/0092-8674(89)90608-9","article-title":"Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques","volume":"59","author":"Scott","year":"1989","journal-title":"Cell"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0175","doi-asserted-by":"crossref","first-page":"18217","DOI":"10.1016\/S0021-9258(18)55257-1","article-title":"The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive","volume":"266","author":"Caughey","year":"1991","journal-title":"J Biol Chem"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0180","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1016\/0003-9861(87)90380-8","article-title":"Isolation and structural studies of the intact scrapie agent protein","volume":"258","author":"Bolton","year":"1987","journal-title":"Arch Biochem Biophys"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0185","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1126\/science.275.5298.402","article-title":"Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein","volume":"275","author":"Lasmezas","year":"1997","journal-title":"Science"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0190","doi-asserted-by":"crossref","first-page":"35878","DOI":"10.1074\/jbc.M704329200","article-title":"High titers of transmissible spongiform encephalopathy infectivity associated with extremely low levels of PrPSc in vivo","volume":"282","author":"Barron","year":"2007","journal-title":"J Biol Chem"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0195","doi-asserted-by":"crossref","first-page":"5895","DOI":"10.1128\/JVI.03469-12","article-title":"Dissociation between transmissible spongiform encephalopathy (TSE) infectivity and proteinase K-resistant PrP(Sc) levels in peripheral tissue from a murine transgenic model of TSE disease","volume":"87","author":"Dobie","year":"2013","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0200","doi-asserted-by":"crossref","first-page":"1157","DOI":"10.1038\/2654","article-title":"Eight prion strains have PrP(Sc) molecules with different conformations","volume":"4","author":"Safar","year":"1998","journal-title":"Nat Med"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0205","doi-asserted-by":"crossref","first-page":"3501","DOI":"10.1073\/pnas.0409651102","article-title":"Diagnosis of human prion disease","volume":"102","author":"Safar","year":"2005","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0210","doi-asserted-by":"crossref","first-page":"15710","DOI":"10.1021\/bi0615442","article-title":"Isolation and characterization of a proteinase K-sensitive PrPSc fraction","volume":"45","author":"Pastrana","year":"2006","journal-title":"Biochemistry"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0215","doi-asserted-by":"crossref","DOI":"10.1371\/journal.ppat.1002547","article-title":"PK-sensitive PrPSc is infectious and shares basic structural features with PK-resistant PrPSc","volume":"8","author":"Sajnani","year":"2012","journal-title":"PLoS Pathog"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0220","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1016\/0021-9975(79)90046-X","article-title":"Pathogenesis of mouse scrapie: dynamics of agent replication in spleen, spinal cord and brain after infection by different routes","volume":"89","author":"Kimberlin","year":"1979","journal-title":"J Comp Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0225","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1099\/0022-1317-67-2-255","article-title":"Pathogenesis of scrapie (strain 263k) in hamsters infected intracerebrally, intraperitoneally or intraocularly","volume":"67","author":"Kimberlin","year":"1986","journal-title":"J Gen Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0230","doi-asserted-by":"crossref","first-page":"2005","DOI":"10.1099\/0022-1317-67-9-2005","article-title":"Pathogenesis of scrapie: study of the temporal development of clinical symptoms, of infectivity titers and scrapie-associated fibrils in brains of hamsters infected intraperitoneally","volume":"67","author":"Czub","year":"1986","journal-title":"J Gen Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0235","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1038\/nature09768","article-title":"Prion propagation and toxicity in vivo occur in two distinct mechanistic phases","volume":"470","author":"Sandberg","year":"2011","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0240","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1038\/nature03989","article-title":"The most infectious prion protein particles","volume":"437","author":"Silveira","year":"2005","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0245","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1126\/science.1138718","article-title":"A general model of prion strains and their pathogenicity","volume":"318","author":"Collinge","year":"2007","journal-title":"Science"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0250","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1126\/science.7909169","article-title":"Structural clues to prion replication","volume":"264","author":"Cohen","year":"1994","journal-title":"Science"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0255","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/0092-8674(94)90177-5","article-title":"Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins","volume":"16","author":"Westaway","year":"1994","journal-title":"Cell"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0260","doi-asserted-by":"crossref","first-page":"6418","DOI":"10.1073\/pnas.91.14.6418","article-title":"Structure and polymorphism of the mouse prion protein gene","volume":"91","author":"Westaway","year":"1994","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0265","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/0092-8674(95)90236-8","article-title":"Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein","volume":"83","author":"Telling","year":"1995","journal-title":"Cell"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0270","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1016\/0092-8674(93)90635-4","article-title":"Seeding \u201cone-dimensional crystallization\u201d of amyloid: a pathogenic mechanism in Alzheimer's disease and scrapie?","volume":"13","author":"Jarrett","year":"1993","journal-title":"Cell"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0275","doi-asserted-by":"crossref","first-page":"9741","DOI":"10.1073\/pnas.0702662104","article-title":"Formation of native prions from minimal components in vitro","volume":"104","author":"Deleault","year":"2007","journal-title":"Proc Natl Acad Sci USA"},{"issue":"4","key":"10.1016\/bs.pmbts.2017.06.014_bb0280","doi-asserted-by":"crossref","DOI":"10.1371\/journal.ppat.1002589","article-title":"The role of cofactors in prion propagation and infectivity","volume":"8","author":"Ma","year":"2012","journal-title":"PLoS Pathog"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0285","series-title":"Transgenetic investigations of the species barrier and prion strains. Prion Biology and Diseases","author":"Scott","year":"2004"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0290","doi-asserted-by":"crossref","first-page":"1793","DOI":"10.1126\/science.1103932","article-title":"Human prion protein with valine 129 prevents expression of variant CJD phenotype","volume":"306","author":"Wadsworth","year":"2004","journal-title":"Science"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0295","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1098\/rstb.1994.0036","article-title":"Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier","volume":"343","author":"Bruce","year":"1994","journal-title":"Phil Trans R Soc Lond"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0300","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1038\/352679a0","article-title":"A \u201cunified theory\u201d of prion propagation","volume":"352","author":"Weissmann","year":"1991","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0305","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/S0368-1742(61)80013-1","article-title":"Scrapie produced experimentally in goats with special reference to the clinical syndrome","volume":"71","author":"Pattison","year":"1961","journal-title":"J Comp Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0310","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/0021-9975(73)90024-8","article-title":"Scrapie in mice. Agent-strain differences in the distribution and intensity of grey matter vacuolation","volume":"83","author":"Fraser","year":"1973","journal-title":"J Comp Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0315","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1016\/S0896-6273(02)00726-2","article-title":"A change in the conformation of prions accompanies the emergence of a new prion strain","volume":"34","author":"Peretz","year":"2002","journal-title":"Neuron"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0320","doi-asserted-by":"crossref","first-page":"32230","DOI":"10.1074\/jbc.273.48.32230","article-title":"Strain-dependent differences in beta-sheet conformations of abnormal prion protein","volume":"273","author":"Caughey","year":"1998","journal-title":"J Biol Chem"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0325","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1038\/386232a0","article-title":"Typing prion isoforms","volume":"386","author":"Parchi","year":"1997","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0330","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1038\/383685a0","article-title":"Molecular analysis of prion strain variation and the aetiology of \u201cnew variant\u201d CJD","volume":"383","author":"Collinge","year":"1996","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0335","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","year":"1999","journal-title":"Ann Neurol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0340","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1136\/jnnp.2004.048660","article-title":"Molecular neurology of prion disease","volume":"76","author":"Collinge","year":"2005","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0345","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1146\/annurev-animal-022114-111001","article-title":"Chronic wasting disease of cervids: current knowledge and future perspectives","volume":"3","author":"Haley","year":"2015","journal-title":"Annu Rev Anim Biosci"},{"issue":"1","key":"10.1016\/bs.pmbts.2017.06.014_bb0350","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1186\/s13567-016-0375-4","article-title":"First case of chronic wasting disease in Europe in a Norwegian free-ranging reindeer","volume":"47","author":"Benestad","year":"2016","journal-title":"Vet Res"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0355","doi-asserted-by":"crossref","first-page":"1154","DOI":"10.1126\/science.1187107","article-title":"Prion strain mutation determined by prion protein conformational compatibility and primary structure","volume":"328","author":"Angers","year":"2010","journal-title":"Science"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0360","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/j.virusres.2014.11.026","article-title":"Prion and prion-like diseases in animals","volume":"207","author":"Aguilar-Calvo","year":"2015","journal-title":"Virus Res"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0365","doi-asserted-by":"crossref","first-page":"3065","DOI":"10.1073\/pnas.0305777101","article-title":"Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt\u2013Jakob disease","volume":"101","author":"Casalone","year":"2004","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0370","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1038\/sj.embor.7400054","article-title":"Distinct molecular phenotypes in bovine prion diseases","volume":"5","author":"Biacabe","year":"2004","journal-title":"EMBO Rep"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0375","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1136\/vr.153.7.202","article-title":"Cases of scrapie with unusual features in Norway and designation of a new type, Nor98","volume":"153","author":"Benestad","year":"2003","journal-title":"Vet Rec"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0380","first-page":"254","article-title":"Experiments with scrapie with special reference to the nature of the agent and the pathology of the disease","volume":"44","author":"Pattison","year":"1968","journal-title":"Res Publ Assoc Res Nerv Ment Dis"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0385","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1002\/ana.410210611","article-title":"Immunoblotting of Creutzfeldt\u2013Jakob disease prion proteins: host species-specific epitopes","volume":"21","author":"Bockman","year":"1987","journal-title":"Ann Neurol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0390","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1038\/39057","article-title":"Transmissions to mice indicate that \u2018new variant' CJD is caused by the BSE agent","volume":"389","author":"Bruce","year":"1997","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0395","doi-asserted-by":"crossref","first-page":"7490","DOI":"10.1074\/jbc.M110.198465","article-title":"Generation of a new form of human PrP(Sc) in vitro by interspecies transmission from cervid prions","volume":"286","author":"Barria","year":"2011","journal-title":"J Biol Chem"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0400","doi-asserted-by":"crossref","first-page":"2096","DOI":"10.1128\/JVI.66.4.2096-2101.1992","article-title":"Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent","volume":"66","author":"Bessen","year":"1992","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0405","doi-asserted-by":"crossref","DOI":"10.1371\/journal.ppat.1004366","article-title":"Sialylation of prion protein controls the rate of prion amplification, the cross-species barrier, the ratio of PrPSc glycoform and prion infectivity","volume":"10","author":"Katorcha","year":"2014","journal-title":"PLoS Pathog"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0410","doi-asserted-by":"crossref","first-page":"4738","DOI":"10.1128\/JVI.02296-14","article-title":"The glycosylation status of PrPC is a key factor in determining transmissible spongiform encephalopathy transmission between species","volume":"89","author":"Wiseman","year":"2015","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0415","doi-asserted-by":"crossref","DOI":"10.1038\/srep33119","article-title":"Reversible off and on switching of prion infectivity via removing and reinstalling prion sialylation","volume":"6","author":"Katorcha","year":"2016","journal-title":"Sci Rep"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0420","doi-asserted-by":"crossref","first-page":"10427","DOI":"10.1128\/JVI.01586-15","article-title":"Host determinants of prion strain diversity independent of prion protein genotype","volume":"89","author":"Crowell","year":"2015","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0425","doi-asserted-by":"crossref","first-page":"10660","DOI":"10.1128\/JVI.01592-16","article-title":"PrPC governs susceptibility to prion strains in bank vole, while other host factors modulate strain features","volume":"90","author":"Espinosa","year":"2016","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0430","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0061118","article-title":"Prion protein gene variability in spanish goats. Inference through susceptibility to classical scrapie strains and pathogenic distribution of peripheral prp(sc.)","volume":"8","author":"Ac\u00edn","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0435","doi-asserted-by":"crossref","first-page":"2670","DOI":"10.1128\/JVI.02074-13","article-title":"Role of the goat K222-PrP(C) polymorphic variant in prion infection resistance","volume":"88","author":"Aguilar-Calvo","year":"2014","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0440","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1093\/infdis\/jiv112","article-title":"Effect of Q211 and K222 PRNP polymorphic variants in the susceptibility of goats to oral infection with goat bovine spongiform encephalopathy","volume":"212","author":"Aguilar-Calvo","year":"2015","journal-title":"J Infect Dis"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0445","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1186\/s13567-016-0380-7","article-title":"Goat K222-PrPC polymorphic variant does not provide resistance to atypical scrapie in transgenic mice","volume":"47","author":"Aguilar-Calvo","year":"2016","journal-title":"Vet Res"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0450","first-page":"50","article-title":"Variant Creutzfeldt\u2013Jakob disease: an update","volume":"50","author":"Ironside","year":"2012","journal-title":"Folia Neuropathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0455","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/128_2011_157","article-title":"Genetics of prion disease","volume":"305","author":"Lloyd","year":"2011","journal-title":"Top Curr Chem"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0460","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1136\/vr.121.18.419","article-title":"A novel progressive spongiform encephalopathy in cattle","volume":"121","author":"Wells","year":"1987","journal-title":"Vet Rec"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0465","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1016\/S0140-6736(96)91412-9","article-title":"A new variant of Creutzfeldt\u2013Jakob disease in the UK","volume":"347","author":"Will","year":"1996","journal-title":"Lancet"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0470","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1056\/NEJMc1610003","article-title":"Variant Creutzfeldt\u2013Jakob disease in a patient with heterozygosity at PRNP codon 129","volume":"376","author":"Mok","year":"2017","journal-title":"N Engl J Med"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0475","doi-asserted-by":"crossref","first-page":"21659","DOI":"10.1074\/jbc.M113.470328","article-title":"Characterization of variant Creutzfeldt\u2013Jakob disease prions in prion protein-humanized mice carrying distinct codon 129 genotypes","volume":"288","author":"Takeuchi","year":"2013","journal-title":"J Biol Chem"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0480","doi-asserted-by":"crossref","DOI":"10.1371\/journal.ppat.1001319","article-title":"Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice","volume":"7","author":"Padilla","year":"2011","journal-title":"PLoS Pathog"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0485","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/BF00294214","article-title":"The mRNA encoding the scrapie agent protein is present in a variety of non-neuronal cells","volume":"80","author":"Brown","year":"1990","journal-title":"Acta Neuropathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0490","first-page":"1","article-title":"Scrapie prion proteins are synthesized in neurons","volume":"122","author":"Kretzschmar","year":"1986","journal-title":"Am J Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0495","doi-asserted-by":"crossref","first-page":"E10","DOI":"10.3171\/2016.5.FOCUS15126","article-title":"Human prion diseases: surgical lessons learned from iatrogenic prion transmission","volume":"41","author":"Bonda","year":"2016","journal-title":"Neurosurg Focus"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0500","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1016\/S0140-6736(06)68930-7","article-title":"Kuru in the 21st century\u2014an acquired human prion disease with very long incubation periods","volume":"367","author":"Collinge","year":"2006","journal-title":"Lancet"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0505","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1038\/382779a0","article-title":"Transmission dynamics and epidemiology of BSE in British cattle","volume":"382","author":"Anderson","year":"1996","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0510","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1002\/path.1880","article-title":"Pathogenesis and prevalence of variant Creutzfeldt\u2013Jakob disease","volume":"208","author":"Hilton","year":"2006","journal-title":"J Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0515","doi-asserted-by":"crossref","first-page":"539","DOI":"10.20506\/rst.11.2.606","article-title":"Transmissible mink encephalopathy","volume":"11","author":"Marsh","year":"1992","journal-title":"Rev Sci Tech"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0520","doi-asserted-by":"crossref","first-page":"9320","DOI":"10.1128\/JVI.75.19.9320-9327.2001","article-title":"Early spread of scrapie from the gastrointestinal tract to the central nervous system involves autonomic fibers of the splanchnic and vagus nerves","volume":"75","author":"McBride","year":"2001","journal-title":"J Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0525","doi-asserted-by":"crossref","first-page":"4046","DOI":"10.1073\/pnas.96.7.4046","article-title":"Natural and experimental oral infection of nonhuman primates by bovine spongiform encephalopathy agents","volume":"96","author":"Bons","year":"1999","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0530","doi-asserted-by":"crossref","first-page":"3115","DOI":"10.1099\/0022-1317-81-12-3115","article-title":"Early accumulation of PrP(Sc) in gut-associated lymphoid and nervous tissues of susceptible sheep from a Romanov flock with natural scrapie","volume":"81","author":"Andr\u00e9oletti","year":"2000","journal-title":"J Gen Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0535","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1038\/nm0901-976","article-title":"Transepithelial prion transport by M cells","volume":"7","author":"Heppner","year":"2001","journal-title":"Nat Med"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0540","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1146\/annurev.immunol.14.1.275","article-title":"Antigen sampling across epithelial barriers and induction of mucosal immune responses","volume":"14","author":"Neutra","year":"1996","journal-title":"Annu Rev Immunol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0545","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1038\/mi.2011.68","article-title":"M cell-depletion blocks oral prion disease pathogenesis","volume":"5","author":"Donaldson","year":"2012","journal-title":"Mucosal Immunol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0550","doi-asserted-by":"crossref","first-page":"11280","DOI":"10.1523\/JNEUROSCI.2864-04.2004","article-title":"Protease-resistant human prion protein and ferritin are cotransported across Caco-2 epithelial cells: implications for species barrier in prion uptake from the intestine","volume":"24","author":"Mishra","year":"2004","journal-title":"J Neurosci"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0555","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1016\/S0002-9440(10)61192-3","article-title":"Bovine prion is endocytosed by human enterocytes via the 37 kDa\/67 kDa laminin receptor","volume":"167","author":"Morel","year":"2005","journal-title":"Am J Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0560","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1086\/595791","article-title":"Bacterial colitis increases susceptibility to oral prion disease","volume":"199","author":"Sigurdson","year":"2008","journal-title":"J Infect Dis"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0565","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1038\/nrmicro1346","article-title":"Prions and their lethal journey to the brain","volume":"4","author":"Mabbott","year":"2006","journal-title":"Nat Rev Microbiol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0570","doi-asserted-by":"crossref","first-page":"7758","DOI":"10.4049\/jimmunol.179.11.7758","article-title":"In vivo depletion of CD11c+ cells impairs scrapie agent neuroinvasion from the intestine","volume":"179","author":"Raymond","year":"2007","journal-title":"J Immunol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0575","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1038\/295149a0","article-title":"Neuronal spread of scrapie agent and targeting of lesions within the retino-tectal pathway","volume":"295","author":"Fraser","year":"1982","journal-title":"Nature"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0580","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/0378-1135(93)90055-C","article-title":"Conjunctival instillation of scrapie in mice can produce disease","volume":"34","author":"Scott","year":"1993","journal-title":"Vet Microbiol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0585","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/0021-9975(78)90059-2","article-title":"Pathogenesis of mouse scrapie: effect of route of inoculation on infectivity titers and dose\u2013response curves","volume":"88","author":"Kimberlin","year":"1978","journal-title":"J Comp Pathol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0590","doi-asserted-by":"crossref","DOI":"10.1371\/journal.ppat.1002782","article-title":"Highly efficient prion transmission by blood transfusion","volume":"8","author":"Andr\u00e9oletti","year":"2012","journal-title":"PLoS Pathog"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0595","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1016\/S0140-6736(04)15486-X","article-title":"Possible transmission of variant Creutzfeldt\u2013Jakob disease by blood transfusion","volume":"363","author":"Llewelyn","year":"2004","journal-title":"Lancet"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0600","doi-asserted-by":"crossref","first-page":"275","DOI":"10.4161\/pri.4.4.13678","article-title":"Prion transmission: prion excretion and occurrence in the environment","volume":"4","author":"Gough","year":"2010","journal-title":"Prion"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0605","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1016\/j.addr.2003.09.008","article-title":"Involvement of gut-associated lymphoid tissue of ruminants in the spread of transmissible spongiform encephalopathies","volume":"56","author":"Press","year":"2004","journal-title":"Adv Drug Deliv Rev"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0610","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1086\/431602","article-title":"Highly bovine spongiform encephalopathy-sensitive transgenic mice confirm the essential restriction of infectivity to the nervous system in clinically diseased cattle","volume":"192","author":"Buschmann","year":"2005","journal-title":"J Infect Dis"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0615","doi-asserted-by":"crossref","first-page":"1379","DOI":"10.1099\/vir.0.82647-0","article-title":"Progression of prion infectivity in asymptomatic cattle after oral bovine spongiform encephalopathy challenge","volume":"88","author":"Espinosa","year":"2007","journal-title":"J Gen Virol"},{"key":"10.1016\/bs.pmbts.2017.06.014_bb0620","doi-asserted-by":"crossref","first-page":"399","DOI":"10.18388\/abp.2006_3355","article-title":"PrP(d) accumulation in organs of ARQ\/ARQ sheep experimentally infected with BSE by peripheral routes","volume":"53","author":"Lezmi","year":"2006","journal-title":"Acta Biochim Pol"}],"container-title":["Progress in Molecular Biology and Translational Science","Prion Protein"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1877117317300959?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1877117317300959?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,7,31]],"date-time":"2022-07-31T17:22:32Z","timestamp":1659288152000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1877117317300959"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017]]},"ISBN":["9780128112267"],"references-count":123,"URL":"https:\/\/doi.org\/10.1016\/bs.pmbts.2017.06.014","relation":{},"ISSN":["1877-1173"],"issn-type":[{"value":"1877-1173","type":"print"}],"subject":[],"published":{"date-parts":[[2017]]}}}