{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T17:40:47Z","timestamp":1791999647416,"version":"4.4.0"},"reference-count":20,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,12,27]],"date-time":"2025-12-27T00:00:00Z","timestamp":1766793600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,12,27]],"date-time":"2025-12-27T00:00:00Z","timestamp":1766793600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100011033","name":"Agencia Estatal de Investigaci\u00f3n","doi-asserted-by":"publisher","award":["PID2021\u2010122201OB\u2010C21"],"award-info":[{"award-number":["PID2021\u2010122201OB\u2010C21"]}],"id":[{"id":"10.13039\/501100011033","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100011033","name":"Agencia Estatal de Investigaci\u00f3n","doi-asserted-by":"publisher","award":["PID2024\u2010160022OB\u2010I00"],"award-info":[{"award-number":["PID2024\u2010160022OB\u2010I00"]}],"id":[{"id":"10.13039\/501100011033","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    We describe two patients with variably protease\u2010sensitive prionopathy (VPSPr) who developed progressive upper motor neuron symptoms, insomnia, behavioral and cognitive decline, compatible with primary lateral sclerosis associated with frontotemporal dementia (FTD). Neuropathology revealed a spongiform encephalopathy with frontotemporal and pronounced thalamic involvement, associated with fine synaptic abnormal prion protein conformer (PrP\n                    <jats:sup>Sc<\/jats:sup>\n                    ) deposits, microplaques, and intraneuronal aggregates. Western blot analysis revealed a characteristic VPSPr proteolytic profile, lacking the diglycosylated band. Both patients were methionine homozygous at\n                    <jats:italic>PRNP<\/jats:italic>\n                    codon 129 and carried no pathogenic mutations. These cases illustrate that VPSPr can present with a prominent motor neuron syndrome and FTD features.\n                  <\/jats:p>","DOI":"10.1002\/acn3.70294","type":"journal-article","created":{"date-parts":[[2025,12,28]],"date-time":"2025-12-28T04:40:23Z","timestamp":1766896823000},"page":"827-833","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Variably Protease\u2010Sensitive Prionopathy: Two New Cases With Motor Neuron\u2010Dementia Syndrome"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9707-4190","authenticated-orcid":false,"given":"Mar\u00eda Elena","family":"Erro","sequence":"first","affiliation":[{"name":"Department of Neurology Hospital Universitario de Navarra (HUN)  Pamplona Spain"},{"name":"Neuroepigenetics Laboratory\u2010Navarrabiomed\u2010Universidad P\u00fablica de Navarra (UPNA)\u2010Hospital Universitario de Navarra (HUN), IdiSNA  Pamplona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Victoria","family":"Zelaya","sequence":"additional","affiliation":[{"name":"Department of Pathology Hospital Universitario de Navarra (HUN)  Pamplona Spain"},{"name":"Brain Biobank\u2010Navarrabiomed\u2010Universidad P\u00fablica de Navarra (UPNA)\u2010Hospital Universitario de Navarra (HUN), IdiSNA  Pamplona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8776-4211","authenticated-orcid":false,"given":"Hasier","family":"Era\u00f1a","sequence":"additional","affiliation":[{"name":"CIC BioGUNE, Basque Research and Technology Alliance (BRTA) Prion Research Lab  Derio Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Infecciosas (CIBERINFEC) Carlos III National Health Institute  Madrid Spain"},{"name":"ATLAS Molecular Pharma S. L.  Derio Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Javier S\u00e1nchez Ruiz","family":"de Gordoa","sequence":"additional","affiliation":[{"name":"Department of Neurology Hospital Universitario de Navarra (HUN)  Pamplona Spain"},{"name":"Neuroepigenetics Laboratory\u2010Navarrabiomed\u2010Universidad P\u00fablica de Navarra (UPNA)\u2010Hospital Universitario de Navarra (HUN), IdiSNA  Pamplona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ferm\u00edn","family":"Garc\u00eda\u2010Amigot","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics Hospital Universitario de Navarra (HUN)  Pamplona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anika","family":"Simonovska\u2010Serra","sequence":"additional","affiliation":[{"name":"Division of Neuropathology and Neurochemistry, Department of Neurology Medical University of Vienna, and Austrian Reference Center for Human Prion Diseases (OERPE)  Vienna Austria"},{"name":"Comprehensive Center for Clinical Neurosciences &amp; Mental Health Vienna Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Cristina","family":"Caballero","sequence":"additional","affiliation":[{"name":"Department of Pathology Hospital Universitario de Navarra (HUN)  Pamplona Spain"},{"name":"Brain Biobank\u2010Navarrabiomed\u2010Universidad P\u00fablica de Navarra (UPNA)\u2010Hospital Universitario de Navarra (HUN), IdiSNA  Pamplona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isidre","family":"Ferrer","sequence":"additional","affiliation":[{"name":"Biomedical Network Research Centre of Neurodegenerative Diseases (CIBERNED)  Hospitalet de Llobregat Spain"},{"name":"University of Barcelona, Bellvitge, University Hospital\u2010IDIBELL  Hospitalet de Llobregat Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen","family":"Gelpi","sequence":"additional","affiliation":[{"name":"Division of Neuropathology and Neurochemistry, Department of Neurology Medical University of Vienna, and Austrian Reference Center for Human Prion Diseases (OERPE)  Vienna Austria"},{"name":"Comprehensive Center for Clinical Neurosciences &amp; Mental Health Vienna Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivonne","family":"Jeric\u00f3","sequence":"additional","affiliation":[{"name":"Department of Neurology Hospital Universitario de Navarra (HUN)  Pamplona Spain"},{"name":"Neuromuscular and Motor Neuron Diseases Unit\u2010Navarrabiomed Hospital Universitario de Navarra (HUN), Navarra Institute for Health Research (IdiSNA)  Pamplona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joaqu\u00edn","family":"Castilla","sequence":"additional","affiliation":[{"name":"CIC BioGUNE, Basque Research and Technology Alliance (BRTA) Prion Research Lab  Derio Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Infecciosas (CIBERINFEC) Carlos III National Health Institute  Madrid Spain"},{"name":"IKERBASQUE Basque Foundation for Science  Bilbao Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,12,27]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1013343"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000000197"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21420"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/neup.12911"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aws366"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22094"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.3109\/17482968.2011.603732"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnagi.2022.917706"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-023-02661-2"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2377-10-99"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1186\/1752-1947-6-348"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.632"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5201544"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2014-309871"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.3390\/v14020367"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.3390\/pathogens2030457"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0000000000000049"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2023-258199"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0058786"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.3390\/pathogens10050513"}],"container-title":["Annals of Clinical and Translational Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acn3.70294","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/acn3.70294","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acn3.70294","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,13]],"date-time":"2026-04-13T04:17:20Z","timestamp":1776053840000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/acn3.70294"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,27]]},"references-count":20,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4]]}},"alternative-id":["10.1002\/acn3.70294"],"URL":"https:\/\/doi.org\/10.1002\/acn3.70294","archive":["Portico"],"relation":{},"ISSN":["2328-9503","2328-9503"],"issn-type":[{"value":"2328-9503","type":"print"},{"value":"2328-9503","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,27]]},"assertion":[{"value":"2025-10-31","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-02","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-27","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}