{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T09:41:12Z","timestamp":1790847672730,"version":"4.1.0"},"reference-count":65,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2023,3,28]],"date-time":"2023-03-28T00:00:00Z","timestamp":1679961600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000265","name":"UK Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"name":"MRC Prion Unit"},{"name":"National Prion Clinic"},{"name":"National Institute of Health Research\u2019s"},{"name":"Biomedical Research Centre at University College London Hospitals NHS Foundation Trust"},{"name":"Alzheimer\u2019s Society, UK","award":["341"],"award-info":[{"award-number":["341"]}]},{"name":"Alzheimer\u2019s Society, UK","award":["AS-CTF-16b-007"],"award-info":[{"award-number":["AS-CTF-16b-007"]}]},{"name":"CJD Support Network UK Research Support Grants"},{"name":"Association of British Neurologists and Alzheimer\u2019s Research UK"},{"name":"NIHR Senior Investigators"},{"name":"Alzheimer\u2019s Association Clinician Scientist Fellowship"},{"name":"the NIHR BRC and the UK Dementia Research Institute"},{"name":"National Institute of Health Research\u2019s"},{"DOI":"10.13039\/501100018956","name":"Cambridge Biomedical Research Centre","doi-asserted-by":"publisher","award":["NIHR203312"],"award-info":[{"award-number":["NIHR203312"]}],"id":[{"id":"10.13039\/501100018956","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010269","name":"Wellcome 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the Alzheimer\u2019s Association","award":["#ADSF-21-831376-C"],"award-info":[{"award-number":["#ADSF-21-831376-C"]}]},{"name":"AD Strategic Fund and the Alzheimer\u2019s Association","award":["#ADSF-21-831381-C"],"award-info":[{"award-number":["#ADSF-21-831381-C"]}]},{"name":"AD Strategic Fund and the Alzheimer\u2019s Association","award":["#ADSF-21-831377-C"],"award-info":[{"award-number":["#ADSF-21-831377-C"]}]},{"name":"Bluefield Project, the Olav Thon Foundation"},{"DOI":"10.13039\/100007436","name":"Erling-Persson Family Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007436","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Stiftelsen f\u00f6r Gamla Tj\u00e4narinnor, Hj\u00e4rnfonden","award":["#FO2022-0270"],"award-info":[{"award-number":["#FO2022-0270"]}]},{"name":"the European Union\u2019s Horizon 2020"},{"name":"Marie Sk\u0142odowska-Curie","award":["860197"],"award-info":[{"award-number":["860197"]}]},{"name":"the European Union Joint Programme\u2014Neurodegenerative Disease Research","award":["JPND2021-00694"],"award-info":[{"award-number":["JPND2021-00694"]}]},{"DOI":"10.13039\/501100017510","name":"UK Dementia Research Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100017510","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000765","name":"UCL","doi-asserted-by":"publisher","award":["UKDRI-1003"],"award-info":[{"award-number":["UKDRI-1003"]}],"id":[{"id":"10.13039\/501100000765","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Intramural Research Program of the National Institute"},{"name":"Allergy and Infectious Diseases"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006662","name":"NIHR","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006662","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000276","name":"Department of Health and Social Care","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000276","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Human prion diseases are remarkable for long incubation times followed typically by rapid clinical decline. Seed amplification assays and neurodegeneration biofluid biomarkers are remarkably useful in the clinical phase, but their potential to predict clinical onset in healthy people remains unclear. This is relevant not only to the design of preventive strategies in those at-risk of prion diseases, but more broadly, because prion-like mechanisms are thought to underpin many neurodegenerative disorders. Here, we report the accrual of a longitudinal biofluid resource in patients, controls and healthy people at risk of prion diseases, to which ultrasensitive techniques such as real-time quaking-induced conversion (RT-QuIC) and single molecule array (Simoa) digital immunoassays were applied for preclinical biomarker discovery.<\/jats:p>\n                  <jats:p>We studied 648 CSF and plasma samples, including 16 people who had samples taken when healthy but later developed inherited prion disease (IPD) (\u2018converters\u2019; range from 9.9 prior to, and 7.4 years after onset). Symptomatic IPD CSF samples were screened by RT-QuIC assay variations, before testing the entire collection of at-risk samples using the most sensitive assay. Glial fibrillary acidic protein (GFAP), neurofilament light (NfL), tau and UCH-L1 levels were measured in plasma and CSF. Second generation (IQ-CSF) RT-QuIC proved 100% sensitive and specific for sporadic Creutzfeldt-Jakob disease (CJD), iatrogenic and familial CJD phenotypes, and subsequently detected seeding activity in four presymptomatic CSF samples from three E200K carriers; one converted in under 2 months while two remain asymptomatic after at least 3 years\u2019 follow-up.<\/jats:p>\n                  <jats:p>A bespoke HuPrP P102L RT-QuIC showed partial sensitivity for P102L disease. No compatible RT-QuIC assay was discovered for classical 6-OPRI, A117V and D178N, and these at-risk samples tested negative with bank vole RT-QuIC. Plasma GFAP and NfL, and CSF NfL levels emerged as proximity markers of neurodegeneration in the typically slow IPDs (e.g. P102L), with significant differences in mean values segregating healthy control from IPD carriers (within 2 years to onset) and symptomatic IPD cohorts; plasma GFAP appears to change before NfL, and before clinical conversion.<\/jats:p>\n                  <jats:p>In conclusion, we show distinct biomarker trajectories in fast and slow IPDs. Specifically, we identify several years of presymptomatic seeding positivity in E200K, a new proximity marker (plasma GFAP) and sequential neurodegenerative marker evolution (plasma GFAP followed by NfL) in slow IPDs. We suggest a new preclinical staging system featuring clinical, seeding and neurodegeneration aspects, for validation with larger prion at-risk cohorts, and with potential application to other neurodegenerative proteopathies.<\/jats:p>","DOI":"10.1093\/brain\/awad101","type":"journal-article","created":{"date-parts":[[2023,3,25]],"date-time":"2023-03-25T04:00:48Z","timestamp":1679716848000},"page":"2570-2583","source":"Crossref","is-referenced-by-count":44,"title":["Seed amplification and neurodegeneration marker trajectories in individuals at risk of prion disease"],"prefix":"10.1093","volume":"146","author":[{"given":"Tze How","family":"Mok","sequence":"first","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"},{"name":"NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akin","family":"Nihat","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"},{"name":"NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nour","family":"Majbour","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle","family":"Sequeira","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"},{"name":"NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leah","family":"Holm-Mercer","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"},{"name":"NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Coysh","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"},{"name":"NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lee","family":"Darwent","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Batchelor","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bradley R","family":"Groveman","sequence":"additional","affiliation":[{"name":"Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health , Hamilton, MT 59840 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina D","family":"Orr","sequence":"additional","affiliation":[{"name":"Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health , Hamilton, MT 59840 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew G","family":"Hughson","sequence":"additional","affiliation":[{"name":"Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health , Hamilton, MT 59840 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda","family":"Heslegrave","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square , London WC1N 3BG , UK"},{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhiannon","family":"Laban","sequence":"additional","affiliation":[{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Veleva","sequence":"additional","affiliation":[{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ross W","family":"Paterson","sequence":"additional","affiliation":[{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"},{"name":"Dementia Research Centre, Department of Neurodegenerative Disease, University College London Queen Square Institute of Neurology , London WC1N 3AR , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashvini","family":"Keshavan","sequence":"additional","affiliation":[{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"},{"name":"Dementia Research Centre, Department of Neurodegenerative Disease, University College London Queen Square Institute of Neurology , London WC1N 3AR , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2059-024X","authenticated-orcid":false,"given":"Jonathan M","family":"Schott","sequence":"additional","affiliation":[{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"},{"name":"Dementia Research Centre, Department of Neurodegenerative Disease, University College London Queen Square Institute of Neurology , London WC1N 3AR , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Imogen J","family":"Swift","sequence":"additional","affiliation":[{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"},{"name":"Dementia Research Centre, Department of Neurodegenerative Disease, University College London Queen Square Institute of Neurology , London WC1N 3AR , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolin","family":"Heller","sequence":"additional","affiliation":[{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"},{"name":"Dementia Research Centre, Department of Neurodegenerative Disease, University College London Queen Square Institute of Neurology , London WC1N 3AR , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D","family":"Rohrer","sequence":"additional","affiliation":[{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"},{"name":"Dementia Research Centre, Department of Neurodegenerative Disease, University College London Queen Square Institute of Neurology , London WC1N 3AR , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8071-6062","authenticated-orcid":false,"given":"Alexander","family":"Gerhard","sequence":"additional","affiliation":[{"name":"Division of Neuroscience and Experimental Psychology, Wolfson Molecular Imaging Centre, University of Manchester , Manchester M13 9PL , UK"},{"name":"Department of Geriatric Medicine, Center for Translational Neuro- and Behavioral Sciences, University Medicine Essen , 45147 Essen , Germany"},{"name":"Department of Nuclear Medicine, Center for Translational Neuro- and Behavioral Sciences, University Medicine Essen , 45147 Essen , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"Butler","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences, Medical Sciences Division, University of Oxford , Oxford OX3 9DU , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James B","family":"Rowe","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust and Medical Research Council Cognition and Brain Sciences Unit, University of Cambridge , Cambridge CB2 7EF , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6244-2096","authenticated-orcid":false,"given":"Mario","family":"Masellis","sequence":"additional","affiliation":[{"name":"Sunnybrook Health Sciences Centre, Sunnybrook Research Institute, University of Toronto , Toronto, ON M4N 3M5 , Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miles","family":"Chapman","sequence":"additional","affiliation":[{"name":"Neuroimmunology and CSF Laboratory, University College London Hospitals NHS Trust National Hospital of Neurology and Neurosurgery , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3174-6027","authenticated-orcid":false,"given":"Michael P","family":"Lunn","sequence":"additional","affiliation":[{"name":"Neuroimmunology and CSF Laboratory, University College London Hospitals NHS Trust National Hospital of Neurology and Neurosurgery , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Bieschke","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graham S","family":"Jackson","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3930-4354","authenticated-orcid":false,"given":"Henrik","family":"Zetterberg","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square , London WC1N 3BG , UK"},{"name":"United Kingdom Dementia Research Institute at University College London , London WC1E 6BT , UK"},{"name":"Department of Psychiatry and Neurochemistry, Sahlgrenska Academy at the University of Gothenburg , S-43180 M\u00f6lndal , Sweden"},{"name":"Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, The Sahlgrenska Academy at the University of Gothenburg , S-431 80 M\u00f6lndal , Sweden"},{"name":"Hong Kong Center for Neurodegenerative Diseases , Hong Kong , China"},{"name":"Wisconsin Alzheimer\u2019s Disease Research Center, University of Wisconsin School of Medicine and Public Health, University of Wisconsin-Madison , Madison, WI 53792-2420 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Byron","family":"Caughey","sequence":"additional","affiliation":[{"name":"Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute for Allergy and Infectious Diseases, National Institutes of Health , Hamilton, MT 59840 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Rudge","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"},{"name":"NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Collinge","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"},{"name":"NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Mead","sequence":"additional","affiliation":[{"name":"Medical Research Council Prion Unit at University College London, UCL Institute of Prion Diseases , London W1W 7FF , UK"},{"name":"NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,3,28]]},"reference":[{"key":"2023060100282874300_awad101-B1","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1146\/annurev.neuro.24.1.519","article-title":"Prion diseases of humans and animals: Their causes and molecular basis","volume":"24","author":"Collinge","year":"2001","journal-title":"Annu Rev Neurosci"},{"key":"2023060100282874300_awad101-B2","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":"2023060100282874300_awad101-B3","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1146\/annurev-genet-120213-092352","article-title":"Genetic factors in mammalian prion diseases","volume":"53","author":"Mead","year":"2019","journal-title":"Annu Rev Genet"},{"key":"2023060100282874300_awad101-B4","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1038\/sj.ejhg.5201544","article-title":"Prion disease genetics","volume":"14","author":"Mead","year":"2006","journal-title":"Eur J Hum Genet"},{"key":"2023060100282874300_awad101-B5","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/S0140-6736(99)05128-4","article-title":"Variant creutzfeldt-jakob disease","volume":"354","author":"Collinge","year":"1999","journal-title":"Lancet"},{"issue":"Pt 11","key":"2023060100282874300_awad101-B6","doi-asserted-by":"crossref","first-page":"3386","DOI":"10.1093\/brain\/awv235","article-title":"Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years","volume":"138","author":"Rudge","year":"2015","journal-title":"Brain"},{"key":"2023060100282874300_awad101-B7","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1212\/WNL.55.8.1075","article-title":"Iatrogenic creutzfeldt-jakob disease at the millennium","volume":"55","author":"Brown","year":"2000","journal-title":"Neurology"},{"key":"2023060100282874300_awad101-B8","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1016\/S0140-6736(06)68930-7","article-title":"Kuru in the 21st century\u2013an acquired human prion disease with very long incubation periods","volume":"367","author":"Collinge","year":"2006","journal-title":"Lancet"},{"key":"2023060100282874300_awad101-B9","doi-asserted-by":"crossref","first-page":"3725","DOI":"10.1098\/rstb.2008.0068","article-title":"A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea","volume":"363","author":"Collinge","year":"2008","journal-title":"Philos Trans R Soc Lond, B, Biol Sci"},{"key":"2023060100282874300_awad101-B10","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":"2023060100282874300_awad101-B11","doi-asserted-by":"crossref","first-page":"4347","DOI":"10.1038\/ncomms5347","article-title":"Prion neuropathology follows the accumulation of alternate prion protein isoforms after infective titre has peaked","volume":"5","author":"Sandberg","year":"2014","journal-title":"Nat Commun"},{"key":"2023060100282874300_awad101-B12","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1038\/nature01457","article-title":"Monoclonal antibodies inhibit prion replication and delay the development of prion disease","volume":"422","author":"White","year":"2003","journal-title":"Nature"},{"key":"2023060100282874300_awad101-B13","doi-asserted-by":"crossref","first-page":"e131175","DOI":"10.1172\/jci.insight.131175","article-title":"Antisense oligonucleotides extend survival of prion-infected mice","volume":"4","author":"Raymond","year":"2019","journal-title":"JCI Insight"},{"key":"2023060100282874300_awad101-B14","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1212\/01.WNL.0000084000.27403.15","article-title":"Creutzfeldt-Jakob disease in United Kingdom patients treated with human pituitary growth hormone","volume":"61","author":"Swerdlow","year":"2003","journal-title":"Neurology"},{"key":"2023060100282874300_awad101-B15","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1038\/s41431-022-01132-8","article-title":"Estimation of the number of inherited prion disease mutation carriers in the UK","volume":"30","author":"Corbie","year":"2022","journal-title":"Eur J Hum Genet"},{"key":"2023060100282874300_awad101-B16","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1038\/s41591-018-0304-3","article-title":"Serum neurofilament dynamics predicts neurodegeneration and clinical progression in presymptomatic Alzheimer's disease","volume":"25","author":"Preische","year":"2019","journal-title":"Nat Med"},{"key":"2023060100282874300_awad101-B17","doi-asserted-by":"crossref","first-page":"e125-e134","DOI":"10.1212\/WNL.0000000000007745","article-title":"Age at onset in genetic prion disease and the design of preventive clinical trials","volume":"93","author":"Minikel","year":"2019","journal-title":"Neurology"},{"key":"2023060100282874300_awad101-B18","doi-asserted-by":"crossref","first-page":"e00078-11","DOI":"10.1128\/mBio.00078-11","article-title":"Prion disease blood test using immunoprecipitation and improved quaking-induced conversion","volume":"2","author":"Orru","year":"2011","journal-title":"MBio"},{"key":"2023060100282874300_awad101-B19","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1038\/nm.2294","article-title":"Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion","volume":"17","author":"Atarashi","year":"2011","journal-title":"Nat 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