{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T10:32:25Z","timestamp":1791282745630,"version":"4.1.0"},"reference-count":30,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2022,6,10]],"date-time":"2022-06-10T00:00:00Z","timestamp":1654819200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Prion diseases are a group of devastating neurodegenerative disorders, which include Creutzfeldt\u2013Jakob disease (CJD) in humans, and scrapie and bovine spongiform encephalopathy (BSE) in animals [...]<\/jats:p>","DOI":"10.3390\/ijms23126490","type":"journal-article","created":{"date-parts":[[2022,6,10]],"date-time":"2022-06-10T10:25:12Z","timestamp":1654856712000},"page":"6490","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Prion Pathogenesis Revealed in a Series of the Special Issues \u201cPrions and Prion Diseases\u201d"],"prefix":"10.3390","volume":"23","author":[{"given":"Suehiro","family":"Sakaguchi","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":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,6,10]]},"reference":[{"key":"ref_1","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":"ref_2","first-page":"785","article-title":"Etiology and pathogenesis of prion diseases","volume":"146","author":"DeArmond","year":"1995","journal-title":"Am. J. Pathol."},{"key":"ref_3","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":"ref_4","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1038\/370295a0","article-title":"Prion protein is necessary for normal synaptic function","volume":"370","author":"Collinge","year":"1994","journal-title":"Nature"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1038\/380639a0","article-title":"Altered circadian activity rhythms and sleep in mice devoid of prion protein","volume":"380","author":"Tobler","year":"1996","journal-title":"Nature"},{"key":"ref_6","first-page":"689","article-title":"A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination","volume":"79","author":"Nishida","year":"1999","journal-title":"Lab. Investig."},{"key":"ref_7","first-page":"169","article-title":"Late-onset olfactory deficits and mitral cell loss in mice lacking prion protein with ectopic expression of Doppel","volume":"20","author":"Kim","year":"2007","journal-title":"Int. J. Mol. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1038\/nn.2238","article-title":"Olfactory behavior and physiology are disrupted in prion protein knockout mice","volume":"12","author":"Valley","year":"2009","journal-title":"Nat. Neurosci."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Martellucci, S., Santacroce, C., Santilli, F., Manganelli, V., Sorice, M., and Mattei, V. (2020). Prion Protein in Stem Cells: A Lipid Raft Component Involved in the Cellular Differentiation Process. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21114168"},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Prado, M.B., Melo Escobar, M.I., Alves, R.N., Coelho, B.P., Fernandes, C.F.L., Boccacino, J.M., Iglesia, R.P., and Lopes, M.H. (2020). Prion Protein at the Leading Edge: Its Role in Cell Motility. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21186677"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Miranzadeh Mahabadi, H., and Taghibiglou, C. (2020). Cellular Prion Protein (PrPc): Putative Interacting Partners and Consequences of the Interaction. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21197058"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1126\/science.1090187","article-title":"Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis","volume":"302","author":"Mallucci","year":"2003","journal-title":"Science"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1846","DOI":"10.1038\/ncomms2873","article-title":"Prions disturb post-Golgi trafficking of membrane proteins","volume":"4","author":"Uchiyama","year":"2013","journal-title":"Nat. Commun."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1074\/mcp.M900271-MCP200","article-title":"Mutant prion protein expression is associated with an alteration of the Rab GDP dissociation inhibitor alpha (GDI)\/Rab11 pathway","volume":"9","author":"Massignan","year":"2010","journal-title":"Mol. Cell. Proteomics"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1016\/j.neuron.2012.02.027","article-title":"Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC alpha(2)delta-1 Subunit","volume":"74","author":"Senatore","year":"2012","journal-title":"Neuron"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"21658","DOI":"10.1038\/srep21658","article-title":"Prion infection impairs lysosomal degradation capacity by interfering with rab7 membrane attachment in neuronal cells","volume":"6","author":"Shim","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Cherry, P., and Gilch, S. (2020). The Role of Vesicle Trafficking Defects in the Pathogenesis of Prion and Prion-Like Disorders. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21197016"},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Alves, R.N., Iglesia, R.P., Prado, M.B., Melo Escobar, M.I., Boccacino, J.M., Fernandes, C.F.L., Coelho, B.P., Fortes, A.C., and Lopes, M.H. (2020). A New Take on Prion Protein Dynamics in Cellular Trafficking. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21207763"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"4540","DOI":"10.1016\/j.molcel.2021.08.011","article-title":"High-resolution structure and strain comparison of infectious mammalian prions","volume":"81","author":"Kraus","year":"2021","journal-title":"Mol. Cell"},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Manka, S.W., Zhang, W., Wenborn, A., Betts, J., Joiner, S., Saibil, H.R., Collinge, J., and Wadsworth, J.D.F. (2021). 2.7 \u00c5 cryo-EM structure of ex vivo RML prion fibrils. bioRxiv.","DOI":"10.1101\/2021.12.13.472424"},{"key":"ref_21","doi-asserted-by":"crossref","unstructured":"Uchiyama, K., Miyata, H., Yamaguchi, Y., Imamura, M., Okazaki, M., Pasiana, A.D., Chida, J., Hara, H., Atarashi, R., and Watanabe, H. (2020). Strain-Dependent Prion Infection in Mice Expressing Prion Protein with Deletion of Central Residues 91\u2013106. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21197260"},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Hara, H., and Sakaguchi, S. (2020). N-Terminal Regions of Prion Protein: Functions and Roles in Prion Diseases. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21176233"},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Carlson, G.A., and Prusiner, S.B. (2021). How an Infection of Sheep Revealed Prion Mechanisms in Alzheimer\u2019s Disease and Other Neurodegenerative Disorders. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22094861"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"e153","DOI":"10.1212\/WNL.0000000000008680","article-title":"Prion disease incidence in the United States: 2003\u20132015","volume":"94","author":"Maddox","year":"2020","journal-title":"Neurology"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1016\/B978-0-444-63945-5.00015-5","article-title":"Fatal familial insomnia and sporadic fatal insomnia","volume":"153","author":"Cracco","year":"2018","journal-title":"Handb. Clin. Neurol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1093\/bmb\/66.1.255","article-title":"Acquired prion disease: Iatrogenic CJD, variant CJD, kuru","volume":"66","author":"Will","year":"2003","journal-title":"Br. Med. Bull."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"10109","DOI":"10.1038\/s41598-021-89586-6","article-title":"Neurotropic influenza A virus infection causes prion protein misfolding into infectious prions in neuroblastoma cells","volume":"11","author":"Hara","year":"2021","journal-title":"Sci. Rep."},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Hara, H., and Sakaguchi, S. (2021). Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms222212439"},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Uchiyama, K., Hara, H., Chida, J., Pasiana, A.D., Imamura, M., Mori, T., Takatsuki, H., Atarashi, R., and Sakaguchi, S. (2021). Ethanolamine Is a New Anti-Prion Compound. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms222111742"},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Fiorini, M., Iselle, G., Perra, D., Bongianni, M., Capaldi, S., Sacchetto, L., Ferrari, S., Mombello, A., Vascellari, S., and Testi, S. (2020). High Diagnostic Accuracy of RT-QuIC Assay in a Prospective Study of Patients with Suspected sCJD. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21030880"}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/12\/6490\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:27:33Z","timestamp":1760138853000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/12\/6490"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,10]]},"references-count":30,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2022,6]]}},"alternative-id":["ijms23126490"],"URL":"https:\/\/doi.org\/10.3390\/ijms23126490","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,10]]}}}