{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,20]],"date-time":"2026-03-20T23:26:49Z","timestamp":1774049209223,"version":"3.50.1"},"reference-count":34,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2020,10,1]],"date-time":"2020-10-01T00:00:00Z","timestamp":1601510400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["grant number 26460557"],"award-info":[{"award-number":["grant number 26460557"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["grant number 19H03548"],"award-info":[{"award-number":["grant number 19H03548"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Conformational conversion of the cellular prion protein, PrPC, into the abnormally folded isoform, PrPSc, is a key pathogenic event in prion diseases. However, the exact conversion mechanism remains largely unknown. Transgenic mice expressing PrP with a deletion of the central residues 91\u2013106 were generated in the absence of endogenous PrPC, designated Tg(PrP\u220691\u2013106)\/Prnp0\/0 mice and intracerebrally inoculated with various prions. Tg(PrP\u220691\u2013106)\/Prnp0\/0 mice were resistant to RML, 22L and FK-1 prions, neither producing PrPSc\u220691\u2013106 or prions in the brain nor developing disease after inoculation. However, they remained marginally susceptible to bovine spongiform encephalopathy (BSE) prions, developing disease after elongated incubation times and accumulating PrPSc\u220691\u2013106 and prions in the brain after inoculation with BSE prions. Recombinant PrP\u220691-104 converted into PrPSc\u220691\u2013104 after incubation with BSE-PrPSc-prions but not with RML- and 22L\u2013PrPSc-prions, in a protein misfolding cyclic amplification assay. However, digitonin and heparin stimulated the conversion of PrP\u220691\u2013104 into PrPSc\u220691\u2013104 even after incubation with RML- and 22L-PrPSc-prions. These results suggest that residues 91\u2013106 or 91\u2013104 of PrPC are crucially involved in prion pathogenesis in a strain-dependent manner and may play a similar role to digitonin and heparin in the conversion of PrPC into PrPSc.<\/jats:p>","DOI":"10.3390\/ijms21197260","type":"journal-article","created":{"date-parts":[[2020,10,1]],"date-time":"2020-10-01T09:04:12Z","timestamp":1601543052000},"page":"7260","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Strain-Dependent Prion Infection in Mice Expressing Prion Protein with Deletion of Central Residues 91\u2013106"],"prefix":"10.3390","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5667-3509","authenticated-orcid":false,"given":"Keiji","family":"Uchiyama","sequence":"first","affiliation":[{"name":"Division of Molecular Neurobiology, The Institute for Enzyme Research (KOSOKEN), Tokushima University, 3-18-15 Kuramoto, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hironori","family":"Miyata","sequence":"additional","affiliation":[{"name":"Animal Research Center, School of Medicine, University of Occupational and Environmental Health, Yahatanishi, Kitakyushu 807-8555, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshitaka","family":"Yamaguchi","sequence":"additional","affiliation":[{"name":"Division of Molecular Neurobiology, The Institute for Enzyme Research (KOSOKEN), Tokushima University, 3-18-15 Kuramoto, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Morikazu","family":"Imamura","sequence":"additional","affiliation":[{"name":"Division of Microbiology, Department of Infectious Diseases, Faculty of Medicine, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mariya","family":"Okazaki","sequence":"additional","affiliation":[{"name":"Division of Molecular Neurobiology, The Institute for Enzyme Research (KOSOKEN), Tokushima University, 3-18-15 Kuramoto, Tokushima 770-8503, Japan"},{"name":"Student Laboratory, Tokushima University, Faculty of Medicine, 3-18-15 Kuramoto, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Agriani Dini","family":"Pasiana","sequence":"additional","affiliation":[{"name":"Division of Molecular Neurobiology, The Institute for Enzyme Research (KOSOKEN), Tokushima University, 3-18-15 Kuramoto, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Junji","family":"Chida","sequence":"additional","affiliation":[{"name":"Division of Molecular Neurobiology, The Institute for Enzyme Research (KOSOKEN), Tokushima University, 3-18-15 Kuramoto, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hideyuki","family":"Hara","sequence":"additional","affiliation":[{"name":"Division of Molecular Neurobiology, The Institute for Enzyme Research (KOSOKEN), Tokushima University, 3-18-15 Kuramoto, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryuichiro","family":"Atarashi","sequence":"additional","affiliation":[{"name":"Division of Microbiology, Department of Infectious Diseases, Faculty of Medicine, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hitomi","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Laboratory of Integrative Biological Science, Institute for Frontier Life and Medical Sciences, Kyoto University, Yoshida-Konoe-cho, Sakyo-ku, Kyoto 606-8501, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gen","family":"Kondoh","sequence":"additional","affiliation":[{"name":"Laboratory of Integrative Biological Science, Institute for Frontier Life and Medical Sciences, Kyoto University, Yoshida-Konoe-cho, Sakyo-ku, Kyoto 606-8501, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suehiro","family":"Sakaguchi","sequence":"additional","affiliation":[{"name":"Division of Molecular Neurobiology, The Institute for Enzyme Research (KOSOKEN), Tokushima University, 3-18-15 Kuramoto, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,10,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1146\/annurev.neuro.31.060407.125620","article-title":"The prion\u2019s elusive reason for being","volume":"31","author":"Aguzzi","year":"2008","journal-title":"Annu. 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