Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases

Lambert, Zoe J.; Greenlee, Justin J.; Cassmann, Eric D.; West Greenlee, M. Heather

doi:10.3390/v13122453

Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases

Journal Article · Mon Dec 06 23:00:00 EST 2021 · Viruses

DOI:https://doi.org/10.3390/v13122453· OSTI ID:1983142

^[1]; Greenlee, Justin J. ^[2]; ^[2]; ^[3]

Iowa State Univ., Ames, IA (United States). College of Veterinary Medicine; US Dept. of Agriculture (USDA), Ames, IA (United States). National Animal Disease Center; Oak Ridge Institute for Science and Education (ORISE), Oak Ridge, TN (United States)
US Dept. of Agriculture (USDA), Ames, IA (United States). National Animal Disease Center
Iowa State Univ., Ames, IA (United States). College of Veterinary Medicine

Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of neurodegenerative protein misfolding diseases that invariably cause death. TSEs occur when the endogenous cellular prion protein (PrP^C) misfolds to form the pathological prion protein (PrP^Sc), which templates further conversion of PrP^C to PrP^Sc, accumulates, and initiates a cascade of pathologic processes in cells and tissues. Different strains of prion disease within a species are thought to arise from the differential misfolding of the prion protein and have different clinical phenotypes. Different strains of prion disease may also result in differential accumulation of PrP^Sc in brain regions and tissues of natural hosts. Here, we review differential accumulation that occurs in the retinal ganglion cells, cerebellar cortex and white matter, and plexuses of the enteric nervous system in cattle with bovine spongiform encephalopathy, sheep and goats with scrapie, cervids with chronic wasting disease, and humans with prion diseases. By characterizing TSEs in their natural host, we can better understand the pathogenesis of different prion strains. This information is valuable in the pursuit of evaluating and discovering potential biomarkers and therapeutics for prion diseases.

View Accepted Manuscript (DOE)

Research Organization:: Oak Ridge Institute for Science and Education (ORISE), Oak Ridge, TN (United States)

Sponsoring Organization:: USDOE Office of Science (SC); USDA

Grant/Contract Number:: SC0014664

OSTI ID:: 1983142

Journal Information:: Viruses, Journal Name: Viruses Journal Issue: 12 Vol. 13; ISSN 1999-4915

Publisher:: MDPICopyright Statement

Country of Publication:: United States

Language:: English

References (194)

Abnormal Eye Movements in Gerstmann-Sträussler-Scheinker Disease Yee, Robert D. Archives of Ophthalmology, Vol. 110, Issue 1 https://doi.org/10.1001/archopht.1992.01080130070028	journal	January 1992
Neuropathology of Gerstmann-Str�ussler-Scheinker disease Bugiani, Orso; Giaccone, Giorgio; Piccardo, Pedro Microscopy Research and Technique, Vol. 50, Issue 1 https://doi.org/10.1002/1097-0029(20000701)50:1<10::AID-JEMT3>3.0.CO;2-6	journal	January 2000
Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects Parchi, Piero; Giese, Armin; Capellari, Sabina Annals of Neurology, Vol. 46, Issue 2 https://doi.org/10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W	journal	August 1999
Diagnosis of new variant Creutzfeldt-Jakob disease Will, R. G.; Zeidler, M.; Stewart, G. E. Annals of Neurology, Vol. 47, Issue 5 https://doi.org/10.1002/1531-8249(200005)47:5<575::AID-ANA4>3.0.CO;2-W	journal	May 2000
Retinal imaging in Alzheimer's and neurodegenerative diseases Snyder, Peter J.; Alber, Jessica; Alt, Clemens Alzheimer's & Dementia, Vol. 17, Issue 1 https://doi.org/10.1002/alz.12179	journal	October 2020
Creutzfeldt-Jakob disease: Clinical analysis of a consecutive series of 230 neuropathologically verified cases Brown, Paul; Cathala, Fran�oise; Castaigne, Paul Annals of Neurology, Vol. 20, Issue 5 https://doi.org/10.1002/ana.410200507	journal	November 1986
Molecular basis of phenotypic variability in sporadc creudeldt-jakob disease Parchi, Piero; Castellani, Rudolph; Capellari, Sabina Annals of Neurology, Vol. 39, Issue 6 https://doi.org/10.1002/ana.410390613	journal	June 1996
Descriptive epidemiology of Creutzfeldt-Jakob disease in six european countries, 1993-1995 Will, R. G.; Alperovitch, A.; Poser, S. Annals of Neurology, Vol. 43, Issue 6 https://doi.org/10.1002/ana.410430611	journal	June 1998
Photoreceptor layer thinning in idiopathic Parkinson's disease Roth, Nicolas M.; Saidha, Shiv; Zimmermann, Hanna Movement Disorders, Vol. 29, Issue 9 https://doi.org/10.1002/mds.25896	journal	April 2014
Transportation of prion protein across the intestinal mucosa of scrapie-susceptible and scrapie-resistant sheep Jeffrey, M.; González, L.; Espenes, A. The Journal of Pathology, Vol. 209, Issue 1 https://doi.org/10.1002/path.1962	journal	January 2006
The Host Range of Chronic Wasting Disease Is Altered on Passage in Ferrets Bartz, Jason C.; Marsh, Richard F.; McKenzie, Debbie I. Virology, Vol. 251, Issue 2 https://doi.org/10.1006/viro.1998.9427	journal	November 1998
Topographic distribution of scrapie amyloid-immunoreactive plaques in chronic wasting disease in captive mule deer (Odocoileus hemionus hemionus) Guiroy, D. C.; Williams, E. S.; Yanagihara, R. Acta Neuropathologica, Vol. 81, Issue 5 https://doi.org/10.1007/BF00310125	journal	April 1991
Creutzfeldt-Jakob disease: a case with extensive white matter degeneration and optic atrophy Kitagawa, Y.; Gotoh, F.; Koto, A. Journal of Neurology, Vol. 229, Issue 2 https://doi.org/10.1007/BF00313448	journal	April 1983
Differentiation of prion protein glycoforms from naturally occurring sheep scrapie, sheep-passaged scrapie strains (CH1641 and SSBP1), bovine spongiform encephalopathy (BSE) cases and Romney and Cheviot breed sheep experimentally inoculated with BSE using two monoclonal antibodies Stack, Michael; Chaplin, Melanie; Clark, Jemma Acta Neuropathologica, Vol. 104, Issue 3 https://doi.org/10.1007/s00401-002-0556-2	journal	September 2002
Neuroanatomical distribution of abnormal prion protein in naturally occurring atypical scrapie cases in Great Britain Moore, Sarah Jo; Simmons, Marion; Chaplin, Melanie Acta Neuropathologica, Vol. 116, Issue 5 https://doi.org/10.1007/s00401-008-0433-8	journal	September 2008
Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification Parchi, Piero; Strammiello, Rosaria; Notari, Silvio Acta Neuropathologica, Vol. 118, Issue 5 https://doi.org/10.1007/s00401-009-0585-1	journal	August 2009
Disease associated prion protein may deposit in the peripheral nervous system in human transmissible spongiform encephalopathies Hainfellner, Johannes A.; Budka, H. Acta Neuropathologica, Vol. 98, Issue 5 https://doi.org/10.1007/s004010051109	journal	November 1999
Electroretinograms in three cases of Creutzfeldt-Jakob disease with visual disturbances Ishikawa, Akira; Tanikawa, Atsuhiro; Shimada, Yoshiaki Japanese Journal of Ophthalmology, Vol. 53, Issue 1 https://doi.org/10.1007/s10384-008-0608-9	journal	January 2009
Reflections on the Utility of the Retina as a Biomarker for Alzheimer’s Disease: A Literature Review Ngolab, Jennifer; Honma, Patrick; Rissman, Robert A. Neurology and Therapy, Vol. 8, Issue S2 https://doi.org/10.1007/s40120-019-00173-4	journal	December 2019
Creutzfeldt-Jakob Disease and Optic Atrophy Lesser, R. L.; Albert, D. M.; Bobowick, A. R. American Journal of Ophthalmology, Vol. 87, Issue 3 https://doi.org/10.1016/0002-9394(79)90070-9	journal	March 1979
Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome Goldgaber, Dmitry; Goldfarb, Lev G.; Brown, Paul Experimental Neurology, Vol. 106, Issue 2 https://doi.org/10.1016/0014-4886(89)90095-2	journal	November 1989
The sequential development of the brain lesions of scrapie in three strains of mice Fraser, H.; Dickinson, A. G. Journal of Comparative Pathology, Vol. 78, Issue 3 https://doi.org/10.1016/0021-9975(68)90006-6	journal	July 1968
A clinico-pathological study of a case of kuru Scrimgeour, Euan M.; Masters, Colin L.; Alpers, Michael P. Journal of the Neurological Sciences, Vol. 59, Issue 2 https://doi.org/10.1016/0022-510X(83)90044-8	journal	May 1983
Precise targeting of the pathology of the sialoglycoprotein, PrP, and vacuolar degeneration in mouse scrapie Bruce, M. E.; McBride, P. A.; Farquhar, C. F. Neuroscience Letters, Vol. 102, Issue 1 https://doi.org/10.1016/0304-3940(89)90298-X	journal	July 1989
Immunolocalization of scrapie amyloid (PrP27-30) in chronic wasting disease of Rocky Mountain elk and hybrids of captive mule deer and white-tailed deer Guiroy, Don C.; Williams, Elizabeth S.; Yanagihara, Richard Neuroscience Letters, Vol. 126, Issue 2 https://doi.org/10.1016/0304-3940(91)90552-5	journal	May 1991
Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay Wadsworth, Jdf; Joiner, S.; Hill, Af The Lancet, Vol. 358, Issue 9277 https://doi.org/10.1016/S0140-6736(01)05403-4	journal	July 2001
Variant CJD in an individual heterozygous for PRNP codon 129 Kaski, Diego; Mead, Simon; Hyare, Harpreet The Lancet, Vol. 374, Issue 9707 https://doi.org/10.1016/S0140-6736(09)61568-3	journal	December 2009
A new variant of Creutzfeldt-Jakob disease in the UK Will, R. G.; Ironside, J. W.; Zeidler, M. The Lancet, Vol. 347, Issue 9006 https://doi.org/10.1016/S0140-6736(96)91412-9	journal	April 1996
Prion protein deposition and abnormal synaptic protein expression in the cerebellum in Creutzfeldt–Jakob disease Ferrer, I.; Puig, B.; Blanco, R. Neuroscience, Vol. 97, Issue 4 https://doi.org/10.1016/S0306-4522(00)00045-2	journal	May 2000
Scrapie Produced Experimentally in Goats With Special Reference To the Clinical Syndrome Pattison, I. H.; Millson, G. C. Journal of Comparative Pathology and Therapeutics, Vol. 71 https://doi.org/10.1016/S0368-1742(61)80013-1	journal	January 1961
Prion immunoreactivity in brain, tonsil, gastrointestinal epithelial cells, and blood and lymph vessels in lemurian zoo primates with spongiform encephalopathy Bons, Noëlle; Mestre-Franœs, Nadine; Guiraud, Isabelle Comptes Rendus de l'Académie des Sciences - Series III - Sciences de la Vie, Vol. 320, Issue 12 https://doi.org/10.1016/S0764-4469(97)82470-5	journal	December 1997
The epidemiology of variant Creutzfeldt–Jakob disease in Europe Ghani, Azra C. Microbes and Infection, Vol. 4, Issue 3 https://doi.org/10.1016/S1286-4579(02)01551-4	journal	March 2002
Involvement of gut-associated lymphoid tissue of ruminants in the spread of transmissible spongiform encephalopathies Press, C. Advanced Drug Delivery Reviews, Vol. 56, Issue 6 https://doi.org/10.1016/j.addr.2003.09.008	journal	April 2004
Spread of Classic BSE Prions from the Gut via the Peripheral Nervous System to the Brain Kaatz, Martin; Fast, Christine; Ziegler, Ute The American Journal of Pathology, Vol. 181, Issue 2 https://doi.org/10.1016/j.ajpath.2012.05.001	journal	August 2012
Atypical and Classical Forms of the Disease-Associated State of the Prion Protein Exhibit Distinct Neuronal Tropism, Deposition Patterns, and Lesion Profiles Kovacs, Gabor G.; Makarava, Natallia; Savtchenko, Regina The American Journal of Pathology, Vol. 183, Issue 5 https://doi.org/10.1016/j.ajpath.2013.07.024	journal	November 2013
Temporal Resolution of Misfolded Prion Protein Transport, Accumulation, Glial Activation, and Neuronal Death in the Retinas of Mice Inoculated with Scrapie West Greenlee, M. Heather; Lind, Melissa; Kokemuller, Robyn The American Journal of Pathology, Vol. 186, Issue 9 https://doi.org/10.1016/j.ajpath.2016.05.018	journal	September 2016
Experimental Study Using Multiple Strains of Prion Disease in Cattle Reveals an Inverse Relationship between Incubation Time and Misfolded Prion Accumulation, Neuroinflammation, and Autophagy Mammadova, Najiba; West Greenlee, M. Heather; Moore, S. Jo The American Journal of Pathology, Vol. 190, Issue 7 https://doi.org/10.1016/j.ajpath.2020.03.006	journal	July 2020
Multiple myenteric networks in the human appendix Hanani, Menachem Autonomic Neuroscience, Vol. 110, Issue 1 https://doi.org/10.1016/j.autneu.2003.09.001	journal	January 2004
The early history of the transmissible spongiform encephalopathies exemplified by scrapie Schneider, Kurt; Fangerau, Heiner; Michaelsen, Britta Brain Research Bulletin, Vol. 77, Issue 6 https://doi.org/10.1016/j.brainresbull.2008.09.012	journal	December 2008
Retinal Cell Types are Differentially Affected in Sheep with Scrapie Smith, J. D.; Greenlee, J. J.; Hamir, A. N. Journal of Comparative Pathology, Vol. 138, Issue 1 https://doi.org/10.1016/j.jcpa.2007.09.002	journal	January 2008
Influence of Polymorphisms in the Prion Protein Gene on the Pathogenesis and Neuropathological Phenotype of Sheep Scrapie after Oral Infection González, L.; Pitarch, J. L.; Martin, S. Journal of Comparative Pathology, Vol. 150, Issue 1 https://doi.org/10.1016/j.jcpa.2013.10.001	journal	January 2014
Immunohistochemical approach to the pathogenesis of bovine spongiform encephalopathy in its early stages Vidal, E.; Márquez, M.; Tortosa, R. Journal of Virological Methods, Vol. 134, Issue 1-2 https://doi.org/10.1016/j.jviromet.2005.11.010	journal	June 2006
Genetic and evolutionary considerations of the Chronic Wasting Disease – Human species barrier Zink, Robert M. Infection, Genetics and Evolution, Vol. 84 https://doi.org/10.1016/j.meegid.2020.104484	journal	October 2020
Optical coherence tomography as a biomarker of neurodegeneration in multiple sclerosis: A review Alonso, Ricardo; Gonzalez-Moron, Dolores; Garcea, Orlando Multiple Sclerosis and Related Disorders, Vol. 22 https://doi.org/10.1016/j.msard.2018.03.007	journal	May 2018
Region-specific glial homeostatic signature in prion diseases is replaced by a uniform neuroinflammation signature, common for brain regions and prion strains with different cell tropism Makarava, Natallia; Chang, Jennifer Chen-Yu; Molesworth, Kara Neurobiology of Disease, Vol. 137 https://doi.org/10.1016/j.nbd.2020.104783	journal	April 2020
Estimation of the age-dependent risk of infection to BSE of dairy cattle in Great Britain Arnold, M. E.; Wilesmith, J. W. Preventive Veterinary Medicine, Vol. 66, Issue 1-4 https://doi.org/10.1016/j.prevetmed.2004.07.007	journal	December 2004
Pathogenesis of classical and atypical BSE in cattle Balkema-Buschmann, A.; Fast, C.; Kaatz, M. Preventive Veterinary Medicine, Vol. 102, Issue 2 https://doi.org/10.1016/j.prevetmed.2011.04.006	journal	November 2011
Efficient transmission of classical scrapie agent x124 by intralingual route to genetically susceptible sheep with a low dose inoculum Mammadova, Najiba; Cassmann, Eric; Greenlee, Justin J. Research in Veterinary Science, Vol. 132 https://doi.org/10.1016/j.rvsc.2020.06.010	journal	October 2020
Successful transmission of the chronic wasting disease (CWD) agent to white-tailed deer by intravenous blood transfusion Mammadova, Najiba; Cassmann, Eric; Greenlee, Justin J. Research in Veterinary Science, Vol. 133 https://doi.org/10.1016/j.rvsc.2020.10.009	journal	December 2020
Distribution of a pathological form of prion protein in the brainstem and cerebellum in classical and atypical cases of bovine spongiform encephalopathy Polak, Miroslaw P.; Zmudzinski, Jan F. The Veterinary Journal, Vol. 191, Issue 1 https://doi.org/10.1016/j.tvjl.2010.12.019	journal	January 2012
Early and late pathogenesis of natural scrapie infection in sheep Van Keulen, L. J. M.; Vromans, M. E. W.; Van Zijderveld, F. G. APMIS, Vol. 110, Issue 1 https://doi.org/10.1034/j.1600-0463.2002.100104.x	journal	January 2002
Distribution of Experimentally Induced Scrapie Lesions in the Brain Fraser, H.; Dickinson, A. G. Nature, Vol. 216, Issue 5122 https://doi.org/10.1038/2161310a0	journal	December 1967
Eight prion strains have PrPSc molecules with different conformations Safar, Jiri; Wille, Holger; Itri, Vincenza Nature Medicine, Vol. 4, Issue 10 https://doi.org/10.1038/2654	journal	October 1998
Fibrils from brains of cows with new cattle disease contain scrapie-associated protein Hope, James; Reekie, Laura J. D.; Hunter, Nora Nature, Vol. 336, Issue 6197 https://doi.org/10.1038/336390a0	journal	November 1988
Prion protein is necessary for normal synaptic function Collinge, John; Whittington, Miles A.; Sidle, Katie C. L. Nature, Vol. 370, Issue 6487 https://doi.org/10.1038/370295a0	journal	July 1994
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD Collinge, John; Sidle, Katie C. L.; Meads, Julie Nature, Vol. 383, Issue 6602 https://doi.org/10.1038/383685a0	journal	October 1996
Cellular prion protein is expressed in the human enteric nervous system Shmakov, Andrei N.; McLennan, Neil F.; McBride, Patricia Nature Medicine, Vol. 6, Issue 8 https://doi.org/10.1038/78558	journal	August 2000
The functional diversity of retinal ganglion cells in the mouse Baden, Tom; Berens, Philipp; Franke, Katrin Nature, Vol. 529, Issue 7586 https://doi.org/10.1038/nature16468	journal	January 2016
Evidence for zoonotic potential of ovine scrapie prions Cassard, Hervé; Torres, Juan-Maria; Lacroux, Caroline Nature Communications, Vol. 5, Issue 1 https://doi.org/10.1038/ncomms6821	journal	December 2014
Novel Strain of the Chronic Wasting Disease Agent Isolated From Experimentally Inoculated Elk With LL132 Prion Protein Moore, Jo; Tatum, Trudy; Hwang, Soyoun Scientific Reports, Vol. 10, Issue 1 https://doi.org/10.1038/s41598-020-59819-1	journal	February 2020
Transmission of scrapie prions to primate after an extended silent incubation period Comoy, Emmanuel E.; Mikol, Jacqueline; Luccantoni-Freire, Sophie Scientific Reports, Vol. 5, Issue 1 https://doi.org/10.1038/srep11573	journal	June 2015
Neuropathological study of cerebellar degeneration in prion disease Yang, Qing; Hashizume, Yoshio; Yoshida, Mari Neuropathology, Vol. 19, Issue 1 https://doi.org/10.1046/j.1440-1789.1999.00212.x	journal	January 1999
Epidemiological and genetical differences between classical and atypical scrapie cases Lühken, Gesine; Buschmann, Anne; Brandt, Horst Veterinary Research, Vol. 38, Issue 1 https://doi.org/10.1051/vetres:2006046	journal	January 2007
Atypical/Nor98 scrapie: properties of the agent, genetics, and epidemiology Benestad, Sylvie L.; Arsac, Jean-Noël; Goldmann, Wilfred Veterinary Research, Vol. 39, Issue 4 https://doi.org/10.1051/vetres:2007056	journal	January 2008
Rodent models for prion diseases Groschup, Martin H.; Buschmann, Anne Veterinary Research, Vol. 39, Issue 4 https://doi.org/10.1051/vetres:2008008	journal	February 2008
Prion agent diversity and species barrier Béringue, Vincent; Vilotte, Jean-Luc; Laude, Hubert Veterinary Research, Vol. 39, Issue 4 https://doi.org/10.1051/vetres:2008024	journal	June 2008
Scrapie-associated Prion Protein in the Gastro-intestinal Tract of Sheep with Natural Scrapie van Keulen, L. J. M.; Schreuder, B. E. C.; Vromans, M. E. W. Journal of Comparative Pathology, Vol. 121, Issue 1 https://doi.org/10.1053/jcpa.1998.0300	journal	July 1999
A Comparative Study of Immunohistochemical Methods for Detecting Abnormal Prion Protein with Monoclonal and Polyclonal Antibodies Hardt, M.; Baron, T.; Groschup, Mh Journal of Comparative Pathology, Vol. 122, Issue 1 https://doi.org/10.1053/jcpa.1999.0343	journal	January 2000
Onset and Distribution of Tissue PrP Accumulation in Scrapie-affected Suffolk Sheep as Demonstrated by Sequential Necropsies and Tonsillar Biopsies Jeffrey, M.; Martin, S.; Thomson, J. R. Journal of Comparative Pathology, Vol. 125, Issue 1 https://doi.org/10.1053/jcpa.2001.0476	journal	July 2001
Effects of Agent Strain and Host Genotype on PrP Accumulation in the Brain of Sheep Naturally and Experimentally Affected with Scrapie González, L.; Martin, S.; Begara-McGorum, I. Journal of Comparative Pathology, Vol. 126, Issue 1 https://doi.org/10.1053/jcpa.2001.0516	journal	January 2002
Fatal Familial Insomnia, a Prion Disease with a Mutation at Codon 178 of the Prion Protein Gene Medori, Rossella; Tritschler, Hans-Juergen; LeBlanc, Andréa New England Journal of Medicine, Vol. 326, Issue 7 https://doi.org/10.1056/NEJM199202133260704	journal	February 1992
Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease Casalone, Cristina; Zanusso, Gianluigi; Acutis, Pierluigi Proceedings of the National Academy of Sciences, Vol. 101, Issue 9 https://doi.org/10.1073/pnas.0305777101	journal	February 2004
Studies in bank voles reveal strain differences between chronic wasting disease prions from Norway and North America Nonno, Romolo; Di Bari, Michele A.; Pirisinu, Laura Proceedings of the National Academy of Sciences, Vol. 117, Issue 49 https://doi.org/10.1073/pnas.2013237117	journal	November 2020
Phenotype-genotype studies in kuru: Implications for new variant Creutzfeldt–Jakob disease Cervenáková, Larisa; Goldfarb, Lev G.; Garruto, Ralph Proceedings of the National Academy of Sciences, Vol. 95, Issue 22 https://doi.org/10.1073/pnas.95.22.13239	journal	October 1998
Genetic influence on the structural variations of the abnormal prion protein Parchi, P.; Zou, W.; Wang, W. Proceedings of the National Academy of Sciences, Vol. 97, Issue 18 https://doi.org/10.1073/pnas.97.18.10168	journal	August 2000
Scrapie pathogenesis in brain and retina: Effects of prion protein expression in neurons and astrocytes Chesebro, Bruce; Race, Richard; Kercher, Lisa Journal of Neurovirology, Vol. 11, Issue 5 https://doi.org/10.1080/13550280500187583	journal	January 2005
Deciphering the BSE-type specific cell and tissue tropisms of atypical (H and L) and classical BSE Balkema-Buschmann, Anne; Priemer, Grit; Ulrich, Reiner Prion, Vol. 13, Issue 1 https://doi.org/10.1080/19336896.2019.1651180	journal	January 2019
Experimental oral transmission of chronic wasting disease to sika deer (Cervus nippon) Sohn, Hyun-Joo; Mitchell, Gordon; Lee, Yoon Hee Prion, Vol. 14, Issue 1 https://doi.org/10.1080/19336896.2020.1857038	journal	January 2020
Other animal prion diseases Sigurdson, Christina J.; Miller, Michael W. British Medical Bulletin, Vol. 66, Issue 1 https://doi.org/10.1093/bmb/66.1.199	journal	June 2003
Sporadic and familial CJD: classification and characterisation Gambetti, Pierluigi; Kong, Qingzhong; Zou, Wenquan British Medical Bulletin, Vol. 66, Issue 1 https://doi.org/10.1093/bmb/66.1.213	journal	June 2003
Molecular classification of sporadic Creutzfeldt–Jakob disease Hill, Andrew F.; Joiner, Susan; Wadsworth, Jonathan D. F. Brain, Vol. 126, Issue 6 https://doi.org/10.1093/brain/awg125	journal	June 2003
Classification of sporadic Creutzfeldt-Jakob disease revisited Cali, I. Brain, Vol. 129, Issue 9 https://doi.org/10.1093/brain/awl224	journal	September 2006
The Transmissible Spongiform Encephalopathies of Livestock Greenlee, J. J.; Greenlee, M. H. W. ILAR Journal, Vol. 56, Issue 1 https://doi.org/10.1093/ilar/ilv008	journal	May 2015
North American and Norwegian Chronic Wasting Disease Prions Exhibit Different Potential for Interspecies Transmission and Zoonotic Risk Pritzkow, Sandra; Gorski, Damian; Ramirez, Frank The Journal of Infectious Diseases, Vol. 225, Issue 3 https://doi.org/10.1093/infdis/jiab385	journal	July 2021
Phenotypic Variability of Gerstmann-Straussler-Scheinker Disease is Associated with Prion Protein Heterogeneity Piccardo, Pedro; Dlouhy, Stephen R.; Lievens, Patricia M. J. Journal of Neuropathology and Experimental Neurology, Vol. 57, Issue 10 https://doi.org/10.1097/00005072-199810000-00010	journal	October 1998
Kuru Liberski, Pawel P.; Sikorska, Beata; Lindenbaum, Shirley Journal of Neuropathology & Experimental Neurology, Vol. 71, Issue 2 https://doi.org/10.1097/NEN.0b013e3182444efd	journal	February 2012
Implications of BSE infection screening data for the scale of the British BSE epidemic and current European infection levels Donnelly, Christl A.; Ferguson, Neil M.; Ghani, Azra C. Proceedings of the Royal Society of London. Series B: Biological Sciences, Vol. 269, Issue 1506 https://doi.org/10.1098/rspb.2002.2156	journal	November 2002
Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease Brandner, Sebastian; Whitfield, Jerome; Boone, Ken Philosophical Transactions of the Royal Society B: Biological Sciences, Vol. 363, Issue 1510 https://doi.org/10.1098/rstb.2008.0091	journal	November 2008
Early accumulation of PrPSc in gut-associated lymphoid and nervous tissues of susceptible sheep from a Romanov flock with natural scrapie Andréoletti, Olivier; Berthon, Patricia; Marc, Daniel Journal of General Virology, Vol. 81, Issue 12 https://doi.org/10.1099/0022-1317-81-12-3115	journal	December 2000
PrPCWD in the myenteric plexus, vagosympathetic trunk and endocrine glands of deer with chronic wasting disease Sigurdson, Christina J.; Spraker, Terry R.; Miller, Michael W. Journal of General Virology, Vol. 82, Issue 10 https://doi.org/10.1099/0022-1317-82-10-2327	journal	October 2001
Detection of PrPSc in peripheral tissues of clinically affected cattle after oral challenge with bovine spongiform encephalopathy Franz, Martin; Eiden, Martin; Balkema-Buschmann, Anne Journal of General Virology, Vol. 93, Issue 12 https://doi.org/10.1099/vir.0.044578-0	journal	December 2012
Disease-associated PrP in the enteric nervous system of scrapie-affected Suffolk sheep Heggebø, Ragna; González, Lorenzo; Press, Charles McL. Journal of General Virology, Vol. 84, Issue 5 https://doi.org/10.1099/vir.0.18874-0	journal	May 2003
Type 1 and type 2 human PrPSc have different aggregation sizes in methionine homozygotes with sporadic, iatrogenic and variant Creutzfeldt–Jakob disease Kobayashi, Atsushi; Satoh, Sakae; Ironside, James W. Journal of General Virology, Vol. 86, Issue 1 https://doi.org/10.1099/vir.0.80389-0	journal	January 2005
Progression of prion infectivity in asymptomatic cattle after oral bovine spongiform encephalopathy challenge Espinosa, Juan Carlos; Morales, Mónica; Castilla, Joaquín Journal of General Virology, Vol. 88, Issue 4 https://doi.org/10.1099/vir.0.82647-0	journal	April 2007
Enteroglial and neuronal involvement without apparent neuron loss in ileal enteric nervous system plexuses from scrapie-affected sheep Marruchella, Giuseppe; Ligios, Ciriaco; Albanese, Valeria Journal of General Virology, Vol. 88, Issue 10 https://doi.org/10.1099/vir.0.82907-0	journal	October 2007
A quantitative study of the pathological changes in the cerebellum in 15 cases of variant Creutzfeldt-Jakob disease (vCJD) Armstrong, R. A.; Ironside, J. W.; Lantos, P. L. Neuropathology and Applied Neurobiology, Vol. 35, Issue 1 https://doi.org/10.1111/j.1365-2990.2008.00979.x	journal	February 2009
Neuropathological Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD) and Other Human Spongiform Encephalopathies (Prion Diseases) Budka, Herbert; Aguzzi, Adriano; Brown, Paul Brain Pathology, Vol. 5, Issue 4 https://doi.org/10.1111/j.1750-3639.1995.tb00625.x	journal	October 1995
Pathology and Immunocytochemistry of a Kuru Brain Hainfellner, Johannes A.; Liberski, Pawel P.; Guiroy, Don C. Brain Pathology, Vol. 7, Issue 1 https://doi.org/10.1111/j.1750-3639.1997.tb01072.x	journal	January 1997
Dura mater graft-associated Creutzfeldt-Jakob disease with 30-year incubation period Shijo, Masahiro; Honda, Hiroyuki; Koyama, Sachiko Neuropathology, Vol. 37, Issue 3 https://doi.org/10.1111/neup.12359	journal	December 2016
Prion Strain Mutation Determined by Prion Protein Conformational Compatibility and Primary Structure Angers, Rachel C.; Kang, Hae-Eun; Napier, Dana Science, Vol. 328, Issue 5982 https://doi.org/10.1126/science.1187107	journal	May 2010
Unconventional Viruses and the Origin and Disappearance of Kuru Gajdusek, D. Carleton Science, Vol. 197, Issue 4307 https://doi.org/10.1126/science.142303	journal	September 1977
Molecular biology of prion diseases Prusiner, S. Science, Vol. 252, Issue 5012 https://doi.org/10.1126/science.1675487	journal	June 1991
Pathways of Prion Spread during Early Chronic Wasting Disease in Deer Hoover, Clare E.; Davenport, Kristen A.; Henderson, Davin M. Journal of Virology, Vol. 91, Issue 10 https://doi.org/10.1128/JVI.00077-17	journal	May 2017
Experimental Transmission of the Chronic Wasting Disease Agent to Swine after Oral or Intracranial Inoculation Moore, S. Jo; West Greenlee, M. Heather; Kondru, Naveen Journal of Virology, Vol. 91, Issue 19 https://doi.org/10.1128/JVI.00926-17	journal	October 2017
Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys ( Saimiri sciureus ) Marsh, Richard F.; Kincaid, Anthony E.; Bessen, Richard A. Journal of Virology, Vol. 79, Issue 21 https://doi.org/10.1128/JVI.79.21.13794-13796.2005	journal	November 2005
Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent Bessen, R. A.; Marsh, R. F. Journal of Virology, Vol. 66, Issue 4 https://doi.org/10.1128/jvi.66.4.2096-2101.1992	journal	April 1992
Prion Seeds Distribute throughout the Eyes of Sporadic Creutzfeldt-Jakob Disease Patients Orrù, Christina D.; Soldau, Katrin; Cordano, Christian mBio, Vol. 9, Issue 6 https://doi.org/10.1128/mBio.02095-18	journal	December 2018
Abnormal prion protein in the retina of the most commonly occurring subtype of sporadic Creutzfeldt-Jakob disease Head, M. W. British Journal of Ophthalmology, Vol. 89, Issue 9 https://doi.org/10.1136/bjo.2004.063495	journal	September 2005
Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey Gill, O. N.; Spencer, Y.; Richard-Loendt, A. BMJ, Vol. 347, Issue oct15 5 https://doi.org/10.1136/bmj.f5675	journal	October 2013
A novel progressive spongiform encephalopathy in cattle Wells, G.; Scott, A.; Johnson, C. Veterinary Record, Vol. 121, Issue 18 https://doi.org/10.1136/vr.121.18.419	journal	October 1987
Feline spongiform encephalopathy: fibril and PrP studies Pearson, G.; Wyatt, J.; Gruffydd-Jones, T. Veterinary Record, Vol. 131, Issue 14 https://doi.org/10.1136/vr.131.14.307	journal	October 1992
Clinical signs, histopathology and genetics of experimental transmission of BSE and natural scrapie to sheep and goats Foster, J. D.; Parnham, D.; Chong, A. Veterinary Record, Vol. 148, Issue 6 https://doi.org/10.1136/vr.148.6.165	journal	February 2001
Detection of disease-specific PrP in the distal ileum of cattle exposed orally to the agent of bovine spongiform encephalopathy Terry, L. A.; Marsh, S.; Ryder, S. J. Veterinary Record, Vol. 152, Issue 13 https://doi.org/10.1136/vr.152.13.387	journal	March 2003
Cases of scrapie with unusual features in Norway and designation of a new type, Nor98 Benestad, S. L.; Sarradin, P.; Thu, B. Veterinary Record, Vol. 153, Issue 7 https://doi.org/10.1136/vr.153.7.202	journal	August 2003
Distribution of PrP^res in the brains of BSE-affected cows detected by active surveillance in Catalonia, Spain Sisó, S.; Ordóñez, M.; Cordón, I. Veterinary Record, Vol. 155, Issue 17 https://doi.org/10.1136/vr.155.17.524	journal	October 2004
Pathogenesis of experimental bovine spongiform encephalopathy: preclinical infectivity in tonsil and observations on the distribution of lingual tonsil in slaughtered cattle Wells, G. A. H.; Spiropoulos, J.; Hawkins, S. A. C. Veterinary Record, Vol. 156, Issue 13 https://doi.org/10.1136/vr.156.13.401	journal	March 2005
Transmissible Spongiform Encephalopathies in Humans Belay, Ermias D. Annual Review of Microbiology, Vol. 53, Issue 1 https://doi.org/10.1146/annurev.micro.53.1.283	journal	October 1999
Prion Protein Accumulation in Eyes of Patients with Sporadic and Variant Creutzfeldt-Jakob Disease Head, Mark W.; Northcott, Victoria; Rennison, Kathleen Investigative Opthalmology & Visual Science, Vol. 44, Issue 1 https://doi.org/10.1167/iovs.01-1273	journal	January 2003
Electrophysiology and Optical Coherence Tomography to Evaluate Parkinson Disease Severity Garcia-Martin, Elena; Rodriguez-Mena, Diego; Satue, Maria Investigative Opthalmology & Visual Science, Vol. 55, Issue 2 https://doi.org/10.1167/iovs.13-13062	journal	February 2014
Detection of the Abnormal Isoform of the Prion Protein Associated With Chronic Wasting Disease in the Optic Pathways of the Brain and Retina of Rocky Mountain Elk (Cervus elaphus nelsoni) Spraker, T. R.; O’Rourke, K. I.; Gidlewski, T. Veterinary Pathology, Vol. 47, Issue 3 https://doi.org/10.1177/0300985810363702	journal	April 2010
Properties of L-Type Bovine Spongiform Encephalopathy in Intraspecies Passages Okada, H.; Iwamaru, Y.; Kakizaki, M. Veterinary Pathology, Vol. 49, Issue 5 https://doi.org/10.1177/0300985811427150	journal	November 2011
Comparison of Two US Sheep Scrapie Isolates Supports Identification as Separate Strains Moore, S. J.; Smith, J. D.; Greenlee, M. H. West Veterinary Pathology, Vol. 53, Issue 6 https://doi.org/10.1177/0300985816629712	journal	July 2016
Review: Update on Classical and Atypical Scrapie in Sheep and Goats Greenlee, Justin J. Veterinary Pathology, Vol. 56, Issue 1 https://doi.org/10.1177/0300985818794247	journal	September 2018
Sheep With the Homozygous Lysine-171 Prion Protein Genotype Are Resistant to Classical Scrapie After Experimental Oronasal Inoculation Cassmann, Eric D.; Moore, Sarah Jo; Smith, Jodi D. Veterinary Pathology, Vol. 56, Issue 3 https://doi.org/10.1177/0300985818817066	journal	December 2018
Immunohistochemical Detection and Distribution of Prion Protein in a Goat with Natural Scrapie Valdez, Reginald A.; Rock, Matthew J.; Anderson, Anne K. Journal of Veterinary Diagnostic Investigation, Vol. 15, Issue 2 https://doi.org/10.1177/104063870301500210	journal	March 2003
Identification and Characterization of two Bovine Spongiform Encephalopathy cases Diagnosed in the United States Richt, Jürgen A.; Kunkle, Robert A.; Alt, David Journal of Veterinary Diagnostic Investigation, Vol. 19, Issue 2 https://doi.org/10.1177/104063870701900202	journal	March 2007
Chronic Wasting Disease in a Wisconsin White-Tailed Deer Farm Keane, Delwyn P.; Barr, Daniel J.; Bochsler, Philip N. Journal of Veterinary Diagnostic Investigation, Vol. 20, Issue 5 https://doi.org/10.1177/104063870802000534	journal	September 2008
Antemortem Detection of PrP^CWD in Preclinical, Ranch-Raised Rocky Mountain Elk (Cevvus Elaphus Nelsoni) by Biopsy of the Rectal Mucosa Spraker, Terry R.; VerCauteren, Kurt C.; Gidlewski, Thomas Journal of Veterinary Diagnostic Investigation, Vol. 21, Issue 1 https://doi.org/10.1177/104063870902100103	journal	January 2009
Atypical Scrapie/Nor98 in a Sheep from New Zealand Kittelberger, Reinhold; Chaplin, Melanie J.; Simmons, Marion M. Journal of Veterinary Diagnostic Investigation, Vol. 22, Issue 6 https://doi.org/10.1177/104063871002200604	journal	November 2010
Second passage of chronic wasting disease of mule deer to sheep by intracranial inoculation compared to classical scrapie Cassmann, Eric D.; Frese, Rylie D.; Greenlee, Justin J. Journal of Veterinary Diagnostic Investigation, Vol. 33, Issue 4 https://doi.org/10.1177/10406387211017615	journal	May 2021
A Critical Review of Atypical Cerebellum-Type Creutzfeldt-Jakob Disease: Its Relationship to “New Variant” CJD and Bovine Spongiform Encephalopathy Narang, Harash K. Experimental Biology and Medicine, Vol. 226, Issue 7 https://doi.org/10.1177/153537020222600709	journal	July 2001
White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation Greenlee, Justin J.; Smith, Jodi D.; Kunkle, Robert A. Veterinary Research, Vol. 42, Issue 1 https://doi.org/10.1186/1297-9716-42-107	journal	January 2011
BSE infectivity in jejunum, ileum and ileocaecal junction of incubating cattle Hoffmann, Christine; Eiden, Martin; Kaatz, Martin Veterinary Research, Vol. 42, Issue 1 https://doi.org/10.1186/1297-9716-42-21	journal	February 2011
Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits Okada, Hiroyuki; Iwamaru, Yoshifumi; Imamura, Morikazu Veterinary Research, Vol. 42, Issue 1 https://doi.org/10.1186/1297-9716-42-79	journal	January 2011
The interpretation of disease phenotypes to identify TSE strains following murine bioassay: characterisation of classical scrapie Beck, Katy E.; Vickery, Christopher M.; Lockey, Richard Veterinary Research, Vol. 43, Issue 1 https://doi.org/10.1186/1297-9716-43-77	journal	November 2012
Comparative evidence for a link between Peyer's patch development and susceptibility to transmissible spongiform encephalopathies St Rose, Suzanne G.; Hunter, Nora; Matthews, Louise BMC Infectious Diseases, Vol. 6, Issue 1 https://doi.org/10.1186/1471-2334-6-5	journal	January 2006
Immunohistochemical and biochemical characteristics of BSE and CWD in experimentally infected European red deer (Cervus elaphus elaphus) Martin, Stuart; Jeffrey, Martin; González, Lorenzo BMC Veterinary Research, Vol. 5, Issue 1 https://doi.org/10.1186/1746-6148-5-26	journal	January 2009
Monitoring of clinical signs in goats with transmissible spongiform encephalopathies Konold, Timm; Bone, Gemma E.; Phelan, Laura J. BMC Veterinary Research, Vol. 6, Issue 1 https://doi.org/10.1186/1746-6148-6-13	journal	March 2010
Clinical, electroretinographic and histomorphometric evaluation of the retina in sheep with natural scrapie Regnier, Alain; Andreoletti, Olivier; Albaric, Olivier BMC Veterinary Research, Vol. 7, Issue 1 https://doi.org/10.1186/1746-6148-7-25	journal	January 2011
Experimental H-type and L-type bovine spongiform encephalopathy in cattle: observation of two clinical syndromes and diagnostic challenges Konold, Timm; Bone, Gemma E.; Clifford, Derek BMC Veterinary Research, Vol. 8, Issue 1 https://doi.org/10.1186/1746-6148-8-22	journal	March 2012
Stability properties of PrPScfrom cattle with experimental transmissible spongiform encephalopathies: use of a rapid whole homogenate, protease-free assay Vrentas, Catherine E.; Greenlee, Justin J.; Baron, Thierry BMC Veterinary Research, Vol. 9, Issue 1 https://doi.org/10.1186/1746-6148-9-167	journal	August 2013
Pathologic and biochemical characterization of PrPSc from elk with PRNP polymorphisms at codon 132 after experimental infection with the chronic wasting disease agent Moore, S. Jo; Vrentas, Catherine E.; Hwang, Soyoun BMC Veterinary Research, Vol. 14, Issue 1 https://doi.org/10.1186/s12917-018-1400-9	journal	March 2018
Prion protein polymorphisms associated with reduced CWD susceptibility limit peripheral PrPCWD deposition in orally infected white-tailed deer Otero, Alicia; Duque Velásquez, Camilo; Johnson, Chad BMC Veterinary Research, Vol. 15, Issue 1 https://doi.org/10.1186/s12917-019-1794-z	journal	February 2019
First case of chronic wasting disease in Europe in a Norwegian free-ranging reindeer Benestad, Sylvie L.; Mitchell, Gordon; Simmons, Marion Veterinary Research, Vol. 47, Issue 1 https://doi.org/10.1186/s13567-016-0375-4	journal	September 2016
The retina as an early biomarker of neurodegeneration in a rotenone-induced model of Parkinson’s disease: evidence for a neuroprotective effect of rosiglitazone in the eye and brain Normando, Eduardo Maria; Davis, Benjamin Michael; De Groef, Lies Acta Neuropathologica Communications, Vol. 4, Issue 1 https://doi.org/10.1186/s40478-016-0346-z	journal	August 2016
PrPres deposition in the retina is a common finding of sporadic, familial and iatrogenic Creutzfeldt-Jakob diseases (CJD) Takao, Masaki; Kimura, Hiroaki; Kitamoto, Tetsuyuki Acta Neuropathologica Communications, Vol. 6, Issue 1 https://doi.org/10.1186/s40478-018-0582-5	journal	August 2018
Creutzfeldt-Jakob disease de Seze, J.; Hache, J. C.; Vermersch, P. Neurology, Vol. 51, Issue 4 https://doi.org/10.1212/WNL.51.4.962	journal	October 1998
Neuroanatomical Distribution of Disease-Associated Prion Protein in Cases of Bovine Spongiform Encephalopathy Detected by Fallen Stock Surveillance in Japan Okada, Hiroyuki; Iwamaru, Yoshihumi; Imamura, Morikazu Journal of Veterinary Medical Science, Vol. 73, Issue 11 https://doi.org/10.1292/jvms.11-0291	journal	January 2011
Transmission of atypical scrapie to homozygous ARQ sheep Okada, Hiroyuki; Miyazawa, Kohtaro; Imamura, Morikazu Journal of Veterinary Medical Science, Vol. 78, Issue 10 https://doi.org/10.1292/jvms.16-0259	journal	January 2016
Retinal Function and Morphology Are Altered in Cattle Infected with the Prion Disease Transmissible Mink Encephalopathy Smith, J. D.; Greenlee, J. J.; Hamir, A. N. Veterinary Pathology, Vol. 46, Issue 5 https://doi.org/10.1354/vp.08-VP-0206-W-FL	journal	May 2009
Characterization of a US Sheep Scrapie Isolate with Short Incubation Time Hamir, A. N.; Richt, J. A.; Kunkle, R. A. Veterinary Pathology, Vol. 46, Issue 6 https://doi.org/10.1354/vp.08-VP-0258-H-FL	journal	July 2009
Comparison of Histological Lesions and Immunohistochemical Staining of Proteinase-resistant Prion Protein in a Naturally Occurring Spongiform Encephalopathy of Free-ranging Mule Deer (Odocoileus hemionus) with Those of Chronic Wasting Disease of Captive Mule Deer Spraker, T. R.; Zink, R. R.; Cummings, B. A. Veterinary Pathology, Vol. 39, Issue 1 https://doi.org/10.1354/vp.39-1-110	journal	January 2002
Chronic Wasting Disease Williams, E. S. Veterinary Pathology, Vol. 42, Issue 5 https://doi.org/10.1354/vp.42-5-530	journal	September 2005
Second Passage of Sheep Scrapie and Transmissible Mink Encephalopathy (TME) Agents in Raccoons (Procyon lotor) Hamir, A. N.; Kunkle, R. A.; Miller, J. M. Veterinary Pathology, Vol. 42, Issue 6 https://doi.org/10.1354/vp.42-6-844	journal	November 2005
Abnormal Prion Accumulation Associated with Retinal Pathology in Experimentally Inoculated Scrapie-Affected Sheep Greenlee, J. J.; Hamir, A. N.; Greenlee, M. H. West Veterinary Pathology, Vol. 43, Issue 5 https://doi.org/10.1354/vp.43-5-733	journal	September 2006
Experimental Transmission of Chronic Wasting Disease (CWD) of Elk (Cervus elaphus nelsoni), White-tailed Deer (Odocoileus virginianus), and Mule Deer (Odocoileus hemionus hemionus) to White-tailed Deer by Intracerebral Route Hamir, A. N.; Richt, J. A.; Miller, J. M. Veterinary Pathology, Vol. 45, Issue 3 https://doi.org/10.1354/vp.45-3-297	journal	May 2008
Serial Passage of Sheep Scrapie Inoculum in Suffolk Sheep Hamir, A. N.; Kunkle, R. A.; Richt, J. A. Veterinary Pathology, Vol. 46, Issue 1 https://doi.org/10.1354/vp.46-1-39	journal	January 2009
Identification of a Heritable Polymorphism in Bovine PRNP Associated with Genetic Transmissible Spongiform Encephalopathy: Evidence of Heritable BSE Nicholson, Eric M.; Brunelle, Brian W.; Richt, Juergen A. PLoS ONE, Vol. 3, Issue 8 https://doi.org/10.1371/journal.pone.0002912	journal	August 2008
Prion Protein Polymorphisms Affect Chronic Wasting Disease Progression Johnson, Chad J.; Herbst, Allen; Duque-Velasquez, Camilo PLoS ONE, Vol. 6, Issue 3 https://doi.org/10.1371/journal.pone.0017450	journal	March 2011
Clinical and Pathologic Features of H-Type Bovine Spongiform Encephalopathy Associated with E211K Prion Protein Polymorphism Greenlee, Justin J.; Smith, Jodi D.; West Greenlee, M. Heather PLoS ONE, Vol. 7, Issue 6 https://doi.org/10.1371/journal.pone.0038678	journal	June 2012
Experimental Oral Transmission of Chronic Wasting Disease to Reindeer (Rangifer tarandus tarandus) Mitchell, Gordon B.; Sigurdson, Christina J.; O’Rourke, Katherine I. PLoS ONE, Vol. 7, Issue 6 https://doi.org/10.1371/journal.pone.0039055	journal	June 2012
Incidence of Infection in Prnp ARR/ARR Sheep following Experimental Inoculation with or Natural Exposure to Classical Scrapie Jeffrey, Martin; Martin, Stuart; Chianini, Francesca PLoS ONE, Vol. 9, Issue 3 https://doi.org/10.1371/journal.pone.0091026	journal	March 2014
Changes in Retinal Function and Morphology Are Early Clinical Signs of Disease in Cattle with Bovine Spongiform Encephalopathy West Greenlee, M. Heather; Smith, Jodi D.; Platt, Ekundayo M. PLOS ONE, Vol. 10, Issue 3 https://doi.org/10.1371/journal.pone.0119431	journal	March 2015
Transmission of the atypical/Nor98 scrapie agent to Suffolk sheep with VRQ/ARQ, ARQ/ARQ, and ARQ/ARR genotypes Cassmann, Eric D.; Mammadova, Najiba; Moore, S. Jo PLOS ONE, Vol. 16, Issue 2 https://doi.org/10.1371/journal.pone.0246503	journal	February 2021
Diversity in Neuroanatomical Distribution of Abnormal Prion Protein in Atypical Scrapie Nentwig, Alice; Oevermann, Anna; Heim, Dagmar PLoS Pathogens, Vol. 3, Issue 6 https://doi.org/10.1371/journal.ppat.0030082	journal	June 2007
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues Andréoletti, Olivier; Orge, Leonor; Benestad, Sylvie L. PLoS Pathogens, Vol. 7, Issue 2 https://doi.org/10.1371/journal.ppat.1001285	journal	February 2011
The Strain-Encoded Relationship between PrPSc Replication, Stability and Processing in Neurons is Predictive of the Incubation Period of Disease Ayers, Jacob I.; Schutt, Charles R.; Shikiya, Ronald A. PLoS Pathogens, Vol. 7, Issue 3 https://doi.org/10.1371/journal.ppat.1001317	journal	March 2011
PrPSc formation and clearance as determinants of prion tropism Shikiya, Ronald A.; Langenfeld, Katie A.; Eckland, Thomas E. PLOS Pathogens, Vol. 13, Issue 3 https://doi.org/10.1371/journal.ppat.1006298	journal	March 2017
Host prion protein expression levels impact prion tropism for the spleen Béringue, Vincent; Tixador, Philippe; Andréoletti, Olivier PLOS Pathogens, Vol. 16, Issue 7 https://doi.org/10.1371/journal.ppat.1008283	journal	July 2020
A family with hereditary cerebellar ataxia finally confirmed as Gerstmann-Sträussler-Scheinker syndrome with P102L mutation in PRNP gene Long, Ling; Cai, Xiaodong; Shu, Yaqing Neurosciences, Vol. 22, Issue 2 https://doi.org/10.17712/nsj.2017.2.20160522	journal	April 2017
Chronic wasting disease in deer and elk in North America Williams, E. S.; Miller, M. W. Revue Scientifique et Technique de l'OIE, Vol. 21, Issue 2 https://doi.org/10.20506/rst.21.2.1340	journal	August 2002
Detection of misfolded prion protein in retina samples of sheep and cattle by use of a commercially available enzyme immunoassay Smith, Jodi D.; Greenlee, Justin J. American Journal of Veterinary Research, Vol. 75, Issue 3 https://doi.org/10.2460/ajvr.75.3.268	journal	March 2014
Pathogenesis, detection, and control of scrapie in sheep Cassmann, Eric D.; Greenlee, Justin J. American Journal of Veterinary Research, Vol. 81, Issue 7 https://doi.org/10.2460/ajvr.81.7.600	journal	July 2020
Sheep With the Homozygous Lysine-171 Prion Protein Genotype Are Resistant to Classical Scrapie After Experimental Oronasal Inoculation Cassmann, Eric D.; Moore, Sarah Jo; Smith, Jodi D. Figshare https://doi.org/10.25384/sage.c.4339769.v1	collection	January 2018
Transmission of Atypical Bovine Prions to Mice Transgenic for Human Prion Protein Béringue, Vincent; Herzog, Laëtitia; Reine, Fabienne Emerging Infectious Diseases, Vol. 14, Issue 12 https://doi.org/10.3201/eid1412.080941	journal	December 2008
Experimental Oral Transmission of Atypical Scrapie to Sheep Simmons, Marion M.; Moore, S. Jo; Konold, Timm Emerging Infectious Diseases, Vol. 17, Issue 5 https://doi.org/10.3201/eid1705.101654	journal	May 2011
Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment Brown, Paul; Brandel, Jean-Philippe; Sato, Takeshi Emerging Infectious Diseases, Vol. 18, Issue 6 https://doi.org/10.3201/eid1806.120116	journal	June 2012
Atypical Scrapie Prions from Sheep and Lack of Disease in Transgenic Mice Overexpressing Human Prion Protein Wadsworth, Jonathan D. F.; Joiner, Susan; Linehan, Jacqueline M. Emerging Infectious Diseases, Vol. 19, Issue 11 https://doi.org/10.3201/eid1911.121341	journal	November 2013
Horizontal Transmission of Chronic Wasting Disease in Reindeer Moore, S. Jo; Kunkle, Robert; Greenlee, M. Heather West Emerging Infectious Diseases, Vol. 22, Issue 12 https://doi.org/10.3201/eid2212.160635	journal	December 2016
Oral Transmission of L-Type Bovine Spongiform Encephalopathy Agent among Cattle Okada, Hiroyuki; Iwamaru, Yoshifumi; Imamura, Morikazu Emerging Infectious Diseases, Vol. 23, Issue 2 https://doi.org/10.3201/eid2302.161416	journal	February 2017
Prion Disease in Dromedary Camels, Algeria Babelhadj, Baaissa; Di Bari, Michele Angelo; Pirisinu, Laura Emerging Infectious Diseases, Vol. 24, Issue 6 https://doi.org/10.3201/eid2406.172007	journal	June 2018
Novel Type of Chronic Wasting Disease Detected in Moose ( Alces alces ), Norway Pirisinu, Laura; Tran, Linh; Chiappini, Barbara Emerging Infectious Diseases, Vol. 24, Issue 12 https://doi.org/10.3201/eid2412.180702	journal	December 2018
Revisiting the Heidenhain Variant of Creutzfeldt-Jakob Disease: Evidence for Prion Type Variability Influencing Clinical Course and Laboratory Findings Baiardi, Simone; Capellari, Sabina; Ladogana, Anna Journal of Alzheimer's Disease, Vol. 50, Issue 2 https://doi.org/10.3233/JAD-150668	journal	January 2016
Investigating Multimodal Diagnostic Eye Biomarkers of Cognitive Impairment by Measuring Vascular and Neurogenic Changes in the Retina Cabrera DeBuc, Delia; Somfai, Gabor Mark; Arthur, Edmund Frontiers in Physiology, Vol. 9 https://doi.org/10.3389/fphys.2018.01721	journal	December 2018
A Comparison of Classical and H-Type Bovine Spongiform Encephalopathy Associated with E211K Prion Protein Polymorphism in Wild-Type and EK211 Cattle Following Intracranial Inoculation Moore, S. Jo; West Greenlee, M. Heather; Smith, Jodi D. Frontiers in Veterinary Science, Vol. 3 https://doi.org/10.3389/fvets.2016.00078	journal	September 2016
Evaluation of Antemortem Diagnostic Techniques in Goats Naturally Infected With Scrapie Mammadova, Najiba; West Greenlee, M. Heather; Moore, S. Jo Frontiers in Veterinary Science, Vol. 7 https://doi.org/10.3389/fvets.2020.517862	journal	November 2020
Astrocyte Reactivity: A Biomarker for Retinal Ganglion Cell Health in Retinal Neurodegeneration R. Formichella, Cathryn Journal of Clinical & Cellular Immunology, Vol. 05, Issue 01 https://doi.org/10.4172/2155-9899.1000188	journal	January 2014
Reflections on the Utility of the Retina as a Biomarker for Alzheimer’s Disease: A Literature Review Ngolab, Jennifer; Homna, Patrick; Rissman, Robert A. Adis Journals https://doi.org/10.6084/m9.figshare.10008494	image	January 2019
Deciphering the BSE-type specific cell and tissue tropisms of atypical (H and L) and classical BSE Balkema-Buschmann, Anne; Priemer, Grit; Ulrich, Reiner Taylor & Francis https://doi.org/10.6084/m9.figshare.9761495	dataset	January 2019
First case of chronic wasting disease in Europe in a Norwegian free-ranging reindeer Benestad, Sylvie; Mitchell, Gordon; Simmons, Marion Figshare https://doi.org/10.6084/m9.figshare.c.3640160.v1	collection	January 2016
Chronic Wasting Disease of Captive mule Deer: a Spongiform Encephalopathy Williams, E. S.; Young, S. Journal of Wildlife Diseases, Vol. 16, Issue 1 https://doi.org/10.7589/0090-3558-16.1.89	journal	January 1980
Spongiform Encephalopathy of Rocky Mountain elk Williams, Elizabeth S.; Young, Stuart Journal of Wildlife Diseases, Vol. 18, Issue 4 https://doi.org/10.7589/0090-3558-18.4.465	journal	October 1982
A Natural Case of Chronic Wasting Disease in a Free-ranging Moose (Alces alces shirasi) Baeten, Laurie A.; Powers, Barbara E.; Jewell, Jean E. Journal of Wildlife Diseases, Vol. 43, Issue 2 https://doi.org/10.7589/0090-3558-43.2.309	journal	April 2007
Transmissible mink Encephalopathy in Carnivores: Clinical, Light and Electron Microscopic Studies in Raccons, Skunks and Ferrets Eckroade, Robert J.; ZuRHEIN, Gabriele M.; Hanson, Robert P. Journal of Wildlife Diseases, Vol. 9, Issue 3 https://doi.org/10.7589/0090-3558-9.3.229	journal	July 1973

Similar Records

FTIR-Microspectroscopy of Prion-Infected Nervous Tissue

Journal Article · Sat Dec 31 23:00:00 EST 2005 · Biochim Biophys Acta · OSTI ID:914383

Magnetic microparticle-based multimer detection system for the detection of prion oligomers in sheep

Journal Article · Tue Sep 01 00:00:00 EDT 2015 · International Journal of Nanomedicine · OSTI ID:1628001

Transmission of the atypical/Nor98 scrapie agent to Suffolk sheep with VRQ/ARQ, ARQ/ARQ, and ARQ/ARR genotypes

Journal Article · Wed Feb 10 23:00:00 EST 2021 · PLoS ONE · OSTI ID:1905052

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
Creutzfeldt–Jakob disease
Gerstmann–Straussler–Scheinker disease
Kuru
bovine spongiform encephalopathy
cerebellar cortex
chronic wasting disease
enteric nervous system
prion neuroinvasion
retinal ganglion cells
scrapie

Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases

Citation Formats

References (194)

Similar Records

Related Subjects