Chronic Wasting Disease

Bibliography

Diagnostics

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Cheng YC, Hannaoui S, John TR, et al. Early and non-invasive detection of chronic wasting disease prions in elk feces by real-time quaking induced conversion. PLoS One 2016 Nov 9;11(11):e0166187

Cheng YC, Hannaoui S, John TR, et al. Real-time quaking-induced conversion assay for detection of CWD prions in fecal material. J Vis Exp 2017 Sep 29;(127)

Cooke CM, Rodger J, Smith A, et al. Fate of prions in soil: detergent extraction of PrP from soils. Environ Sci Technol 2007 Feb 1;41(3):811–17

Davenport KA, Hoover CE, Denkers ND, et al. Modified protein misfolding cyclic amplification overcomes real-time quaking-induced conversion assay inhibitors in deer saliva to detect chronic wasting disease prions. J Clin Microbiol 2018 Aug 27;56(9)

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Denkers ND, Henderson DM, Mathiason CK, et al. Enhanced prion detection in biological samples by magnetic particle extraction and real-time quaking-induced conversion. J Gen Virol 2016 Aug;97(8):2023–29

Ellis CK, Volker SF, Griffin DL, et al. Use of faecal volatile organic compound analysis for ante-mortem discrimination between CWD-positive, -negative exposed, and -known negative white-tailed deer (Odocoileus virginianus). Prion 2019 Jan;13(1):94–105

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Furr A, Knudsen D, Hildreth MB, et al. Enhancement of immunohistochemical staining of scrapie proteins and immune cells within lymph nodes of early scrapie-infected sheep. J Immunol Methods 2011;371(1):1–7

Genovesi S, Leita L, Sequi P, et al. Direct detection of soil-bound prions. PloS One 2007 Oct 24;2(10):e1069

Geremia C, Hoeting JA, Wolfe LL, et al. Age and repeated biopsy influence antemortem PrPCWD testing in mule deer (Odocoileus hemionus) in Colorado, USA. J Wildl Dis 2015 Oct;51(4):801–10

Gonzalez L, Horton R, Ramsay D, et al. Adaptation and evaluation of a rapid test for the diagnosis of sheep scrapie in samples of rectal mucosa. J Vet Diagn Invest 2008 Mar;20(2):203–8

Gray JG, Graham C, Dudas S, et al. Defining and assessing analytical performance criteria for transmissible spongiform encephalopathy–detecting amyloid seeding assays. J Mol Diagn 2016 May;18(3):454–67

Haley NJ, Carver S, Hoon-Hanks LL, et al. Detection of chronic wasting disease in the lymph nodes of free-ranging cervids by real-time quaking-induced conversion. J Clin Microbiol 2014 Sep 52(9):3237–43

Haley NJ, Hoover EA. Chronic wasting disease of cervids: current knowledge and future perspectives. Annu Rev Anim Biosci 2015 3(1):305–25

Haley NJ, Mathiason CK, Carver S, et al. Detection of chronic wasting disease prions in salivary, urinary, and intestinal tissues of deer: potential mechanisms of prion shedding and transmission. J Virol 2011 Jul 1;85(13):6309–18

Haley NJ, Mathiason CK, Carver S, et al. Sensitivity of protein misfolding cyclic amplification versus immunohistochemistry in ante-mortem detection of chronic wasting disease. J Gen Virol 2012 May;93(Pt 5):1141–50

Haley NJ, Mathiason CK, Zabel MD, et al. Detection of sub-clinical CWD infection in conventional test-negative deer long after oral exposure to urine and feces from CWD+ deer. PLoS One 2009 Nov 24;4(11):e7990

Haley NJ, Richt JA. Evolution of diagnostic tests for chronic wasting disease, a naturally occurring prion disease of cervids. Pathogens 2017 Aug 5;6(3)

Haley NJ, Richt JA, Davenport KA, et al. Design, implementation, and interpretation of amplification studies for prion detection. Prion 2018 Mar 4;12(2):73–82

Haley NJ, Siepker C, Hoon-Hanks LL, et al. Seeded amplification of chronic wasting disease prions in nasal brushings and recto-anal mucosa-associated lymphoid tissues from elk by real-time quaking-induced conversion. J Clin Microbiol 2016 Apr;54(4):1117–26

Haley NJ, Siepker C, Walter WD, et al. Antemortem detection of chronic wasting disease prions in nasal brush collections and rectal biopsy specimens from white-tailed deer by real-time quaking-induced conversion. J Clin Microbiol 2016 Apr;54(4):1108–16

Haley NJ, Van de Motter A, Carver S, et al. Prion-seeding activity in cerebrospinal fluid of deer with chronic wasting disease. PloS One 2013 Nov 25;8(11):e81488

Hamir AN, Miller JM, Cutlip RC. Failure to detect prion protein (PrPres) by immunohistochemistry in striated muscle tissues of animals experimentally inoculated with agents of transmissible spongiform encephalopathy. Vet Pathol 2004 Jan;41(1):78–81

Hamir AN, Palmer MV, Kunkle RA. Wasting and neurologic signs in a white-tailed deer (Odocoileus virginianus) not associated with abnormal prion protein. J Wildl Dis 2008 Oct;44(4):1045–50

Hannaoui S, Schatzl HM, Gilch S. Chronic wasting disease: emerging prions and their potential risk. PLoS Pathog 2017 Nov 2;13(11):e1006619

Hedlin P, Taschuk R, Potter A, et al. Detection and control of prion diseases in food animals. Int Sch Res Notices 2012

Henderson DM, Davenport KA, Haley NJ, et al. Quantitative assessment of prion infectivity in tissues and body fluids by real-time quaking-induced conversion. J Gen Virol 2015 Jan;96(1):210–9

Henderson DM, Denkers ND, Hoover CE, et al. Longitudinal detection of prion shedding in saliva and urine by chronic wasting disease-infected deer by real-time quaking-induced conversion. J Virol 2015 Sep;89(18):9338–47

Henderson DM, Manca M, Haley NJ, et al. Rapid antemortem detection of CWD prions in deer saliva. PloS One 2013 Sep 11;8(9):e74377

Hibler CP, Wilson KL, Spraker TR, et al. Field validation and assessment of an enzyme-linked immunosorbent assay for detecting chronic wasting disease in mule deer (Odocoileus hemionus), white-tailed deer (Odocoileus virginianus), and Rocky Mountain elk (Cervus elaphus nelsoni). J Vet Diagn Invest 2003 Jul;15(4):311–9

Holcomb KM, Galloway NL, Mathiason CK, et al. Intra-host mathematical model of chronic wasting disease dynamics in deer (Odocoileus). Prion 2016 Feb;10(5):377–90

Hoover CE, Davenport KA, Henderson DM, et al. Detection and quantification of CWD prions in fixed paraffin embedded tissues by real-time quaking-induced conversion. Sci Rep 2016 May 9;6:25098

Huang H, Rendulich J, Stevenson D, et al. Evaluation of western blotting methods using samples with or without sodium phosphotungstic acid precipitation for diagnosis of scrapie and chronic wasting disease. Can J Vet Res 2005 Jul;69(3):193–9

Huang H, Soutyrine A, Rendulich J, et al. Investigation of the effects of experimental autolysis on the detection of abnormal prion protein in lymphoid and central nervous system tissues from elk and sheep using the western blotting method. Can J Vet Res 2011 Jan;75(1):69–72

Jeffrey M, Wells GA. Spongiform encephalopathy in a Nyala (Tragelaphus angasi). Vet Pathol 1988 Sep 1;25(5):398–9

Jeong HJ, Lee NH, Lee JB, et al. Development of monoclonal antibodies against the abnormal prion protein isoform (PrPres) associated with chronic wasting disease (CWD). J Vet Sci 2012 Dec;13(4):429–32

John TR, Schatzl HM, Gilch S. Early detection of chronic wasting disease prions in urine of pre-symptomatic deer by real-time quaking-induced conversion assay. Prion 2013 Jun 7(3):253–8

Johnson CJ, Carlson CM, Morawski AR, et al. Assessing transmissible spongiform encephalopathy species barriers with an in vitro prion protein conversion assay. J Vis Exp 2015 Mar 10;(97):e52522

Kang HE, Mo Y, Rahim RA, et al. Prion diagnosis: application of real-time quaking-induced conversion. Biomed Res Int 2017 May 17;2017

Keane DP, Barr DJ, Bochsler PN, et al. Chronic wasting disease in a Wisconsin white-tailed deer farm. J Vet Diagn Invest 2008 Sep;20(5):698–703

Keane DP, Barr DJ, Keller JE, et al. Comparison of retropharyngeal lymph node and obex region of the brainstem in detection of chronic wasting disease in white-tailed deer (Odocoileus virginianus). J Vet Diagn Invest 2008 Jan;20(1):58–60

Keane D, Barr D, Osborn R, et al. Validation of use of rectoanal mucosa-associated lymphoid tissue for immunohistochemical diagnosis of chronic wasting disease in white-tailed deer (Odocoileus virginianus). J Clin Microbiol 2009 May;47(5):1412–7

Kramm C, Pritzkow S, Lyon A, et al. Detection of prions in blood of cervids at the asymptomatic stage of chronic wasting disease. Sci Rep 2017 Dec 8;7(1):17241

Kurt TD, Perrott MR, Wilusz CJ, et al. Efficient in vitro amplification of chronic wasting disease PrPres. J Virol 2007 Aug;81(17):9605–8

Lehto MT, Peery HE, Cashman NR. Current and future molecular diagnostics for prion diseases. Expert Rev Mol Diagn 2006 Jul;6(4):597–611

Martin S, Jeffrey M, González L, et al. Immunohistochemical and biochemical characteristics of BSE and CWD in experimentally infected European red deer (Cervus elaphus elaphus). BMC Vet Res 2009 Jul 27;5(1):26

Masujin K, Shimada K, Kimura KM, et al. Applicability of current bovine spongiform encephalopathy (BSE) diagnostic procedures for chronic wasting disease (CWD). Microbiol Immunol 2007;51(10):1039–43

Mathiason CK, Hays SA, Powers J, et al. Infectious prions in pre-clinical deer and transmission of chronic wasting disease solely by environmental exposure. PLoS One 2009 Jun 16;4(6):e5916

Monello RJ, Powers JG, Hobbs NT, et al. Efficacy of antemortem rectal biopsies to diagnose and estimate prevalence of chronic wasting disease in free-ranging cow elk (Cervus elaphus nelson). J Wildl Dis 2013 Apr;49(2):270–8

Morales R, Duran-Aniotz C, Diaz-Espinoza R, et al. Protein misfolding cyclic amplification of infectious prions. Nat Protoc 2012 Jun 28;7(7):1397–409

Nagaoka K, Yoshioka M, Shimozaki N, et al. Sensitive detection of scrapie prion protein in soil. Biochem Biophys Res Commun 2010 Jul 2;397(3):626–30

Nichols TA, Pulford B, Wyckoff AC, et al. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area. Prion 2009 Jul 1;3(3):171–83

Nichols TA, Spraker TR, Gidlewski T, et al. Detection of prion protein in the cerebrospinal fluid of elk (Cervus canadensis nelsoni) with chronic wasting disease using protein misfolding cyclic amplification. J Vet Diagn Invest 2012 Jul 1;24(4):746–9

O’Rourke KI, Baszler TV, Miller JM, et al. Monoclonal antibody F89/160.1.5 defines a conserved epitope on the ruminant prion protein. J Clin Microbiol 1998 Jun;36(6):1750–5

O’Rourke KI, Zhuang D, Lyda A, et al. Abundant PrP(CWD) in tonsil from mule deer with preclinical chronic wasting disease. J Vet Diagn Invest 2003 Jul;15(4):320–3

Orru CD, Groveman BR, Raymond LD, et al. Bank vole prion protein as an apparently universal substrate for RT-QuIC-based detection and discrimination of prion strains. PLoS Pathog 2015 Jun 18;11(6):e1004983

Orru CD, Hughson AG, Groveman BR, et al. Factors that improve RT-QuIC detection of prion seeding activity. Viruses 2016 May 23;8(5):E140

Peters J, Miller JM, Jenny AL, et al. Immunohistochemical diagnosis of chronic wasting disease in preclinically affected elk from a captive herd. J Vet Diagn Invest 2000 Nov;12(6):579–82

Pulford B, Spraker TR, Wyckoff AC, et al. Detection of PrPCWD in feces from naturally exposed Rocky Mountain elk (Cervus elaphus nelson) using protein misfolding cyclic amplification. J Wildl Dis 2012 Apr;48(2):425–34

Pushie MJ, Shaykhutdinov R, Nazyrova A, et al. An NMR metabolomics study of elk inoculated with chronic wasting disease. J Toxicol Environ Health A 2011 Nov 1;74(22–24):1476–92

Rivera NA, Novakofski J, Weng HY, et al. Metals in obex and retropharyngeal lymph nodes of Illinois white-tailed deer and their variations associated with CWD status. Prion 2015 May 6;9(1):48–58

Rubenstein R, Chang B, Gray P, et al. A novel method for preclinical detection of PrPSc in blood. J Gen Virol 2010 Jul 1;91(7):1883–92

Rubenstein R, Chang B, Gray P, et al. Prion disease detection, PMCA kinetics, and IgG in urine from sheep naturally/experimentally infected with scrapie and deer with preclinical/clinical chronic wasting disease. J Virol 2011 Sep;85(17):9031–8

Safar JG, Lessard P, Tamguney G, et al. Transmission and detection of prions in feces. J Infect Dis 2008 Jul 1;198(1):81–9

Safar JG, Scott M, Monaghan J, et al. Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice. Nat Biotechnol Nov 2002;20(11):1147–50

Schmerr MJ, Jenny AL, Bulgin MS, et al. Use of capillary electrophoresis and fluorescent labeled peptides to detect the abnormal prion protein in the blood of animals that are infected with a transmissible spongiform encephalopathy. J Chromatogr A 1999 Aug 20;853(1):207–14

Schuler KL, Jenks JA, DePerno CS, et al. Tonsillar biopsy test for chronic wasting disease: two sampling approaches in mule deer and white-tailed deer. J Wildl Dis 2005 Oct;41(4):820–4

Schuler KL, Jenks JA, Klaver RW, et al. Chronic wasting disease detection and mortality sources in semi-protected deer population. Wildlife Biol 2018 Sep;2018(1)

Sobrova P, Ryvolova M, Adam V, et al. Capillary electromigration based techniques in diagnostics of prion protein caused diseases. Electrophoresis 2012 Dec;33(24):3644–52

Spraker TR, Gidlewski TL, Balachandran A, et al. Detection of PrP(CWD) in postmortem rectal lymphoid tissues in Rocky Mountain elk (Cervus elaphus nelsoni) infected with chronic wasting disease. J Vet Diagn Invest 2006 Nov;18(6):553–7

Spraker TR, Gidlewski T, Powers JG, et al. Progressive accumulation of the abnormal conformer of the prion protein and spongiform encephalopathy in the obex of nonsymptomatic and symptomatic Rocky Mountain elk (Cervus elaphus nelsoni) with chronic wasting disease. J Vet Diagn Invest 2015 Jul 16;27(4):431-441

Spraker TR, Miller MW, Williams ES, et al. Spongiform encephalopathy in free-ranging mule deer (Odocoileus hemionus), white-tailed deer (Odocoileus virginianus) and Rocky Mountain elk (Cervus elaphus nelsoni) in northcentral Colorado. J Wildl Dis 1997 Jan;33(1):1–6

Spraker TR, O’Rourke KI, Balachandran A, et al. Validation of monoclonal antibody F99/97.6.1 for immunohistochemical staining of brain and tonsil in mule deer (Odocoileus hemionus) with chronic wasting disease. J Vet Diagn Invest 2002 Jan;14(1):3–7

Spraker TR, VerCauteren KC, Gidlewski T, et al. Antemortem detection of PrPCWD in preclinical, ranch-raised Rocky Mountain elk (Cevvus elaphus nelsoni) by biopsy of the rectal mucosa. J Vet Diagn Invest 2009 Jan;21(1):15–24

Spraker TR, VerCauteren KC, Gidlewski TL, et al. Impact of age and sex of Rocky Mountain elk (Cervus elaphus nelsoni) on follicle counts from rectal mucosal biopsies for preclinical detection of chronic wasting disease. J Vet Diagn Invest 2009 Nov;21(6):868–70

Spraker TR, Zink RR, Cummings BA, et al. 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. Vet Pathol 2002 Jan;39(1):110–9 

Spraker TR, Zink RR, Cummings BA, et al. Distribution of protease-resistant prion protein and spongiform encephalopathy in free-ranging mule deer (Odocoileus hemionus) with chronic wasting disease. Vet Pathol 2002 Sep;39(5):546–56

Thomsen BV, Schneider DA, O’Rourke KI, et al. Diagnostic accuracy of rectal mucosa biopsy testing for chronic wasting disease within white-tailed deer (Odocoileus virginianus) herds in North America: effects of age, sex, polymorphism at PRNP codon 96, and disease progression. J Vet Diagn Invest 2012 Sep;24(5):878-87

Triantis J, Dennis MM, Salman MD, et al. Effect of time and temperature on PrPCWD immunoreactivity as evidenced by western blot. J Vet Diagn Invest 2007 Jul;19(4):389–91

Watts JC, Balachandran A, Westaway D. The expanding universe of prion diseases. PLoS Pathog 2006 Mar 31;2(3):e26

Wild MA, Spraker TR, Sigurdson CJ, et al. Preclinical diagnosis of chronic wasting disease in captive mule deer (Odocoileus hemionus) and white-tailed deer (Odocoileus virginianus) using tonsillar biopsy. J Gen Virol 2002 Oct 1;83(10):2629–34

Wilham JM, Orru CD, Bessen RA, et al. Rapid end-point quantitation of prion seeding activity with sensitivity comparable to bioassays. PLoS Pathog 2010 Dec 2;6(12):e1001217

Williams ES. Chronic wasting disease. Vet Pathol 2005;20

Williams ES, Miller MW, Kreeger TJ, et al. Chronic wasting disease of deer and elk: a review with recommendations for management. J Wildl Manage 2002 Jul;66(3):551–63

Williams ES, Young S. Spongiform encephalopathy of Rocky Mountain elk. J Wildl Dis 1982 Oct;18(4):465–71

Wolfe LL, Conner MM, Baker TH, et al. Evaluation of antemortem sampling to estimate chronic wasting disease prevalence in free-ranging mule deer. J Wildl Manage 2002 Jul;66(3):564–73

Wyckoff AC, Galloway N, Meyerett-Reid C, et al. Prion amplification and hierarchical Bayesian modeling refine detection of prion infection. Sci Rep 2015 Feb 10;5(1):1–8

Genetics of CWD

Angers R, Christiansen J, Nalls AV, et al. Structural effects of PrP polymorphisms on intra- and interspecies prion transmission. Proc Natl Acad Sci U S A 2014 Jul 29;111(30):11169–74

Basu U, Almeida LM, Dudas S, et al. Gene expression alterations in Rocky Mountain elk infected with chronic wasting disease. Prion 2012 Jul 1;6(3):282–301

Benestad SL, Telling GC. Chronic wasting disease: an evolving prion disease of cervids. Handb Clin Neurol 2018;153:135–51

Bett C, Fernandez-Borges N, Kurt TD, et al. Structure of the β2-α2 loop and interspecies prion transmission. FASEB J 2012 Jul;26(7):2868–76 (published online Apr 9)

Bian J, Christiansen JR, Moreno JA, et al. Primary structural differences at residue 226 of deer and elk PrP dictate selection of distinct CWD prion strains in gene-targeted mice. Proc Natl Acad Sci 2019 May 30;201903947

Blanchong JA, Heisey DM, Scribner KT, et al. Genetic susceptibility to chronic wasting disease in free-ranging white-tailed deer: complement component C1q and Prnp polymorphisms. Infect Genet Evol 2009 Dec;9(6):1329–35

Blanchong JA, Samuel MD, Scribner KT, et al. Landscape genetics and the spatial distribution of chronic wasting disease. Biol Lett 2008 Feb 23;4(1):130–3

Brandt AL, Green ML, Ishida Y, et al. Influence of the geographic distribution of prion protein gene sequence variation on patterns of chronic wasting disease spread in white-tailed deer (Odocoileus virginianus). Prion 2018;12(3–4):204–15

Brandt AL, Kelly AC, Green ML, et al. Prion protein gene sequence and chronic wasting disease susceptibility in white-tailed deer (Odocoileus virginianus). Prion 2015;9(6):449–62

Brayton KA, O’Rourke KI, Lyda AK, et al. A processed pseudogene contributes to apparent mule deer prion gene heterogeneity. Gene 2004 Feb;326:167–73

Cheng YC, Musiani M, Cavedon M, et al. High prevalence of prion protein genotype associated with resistance to chronic wasting disease in one Alberta woodland caribou population. Prion 2017 Mar 4;11(2):136–42

Cortez LM, Sim VL. Implications of prion polymorphisms. Prion 2013 Jul-Aug;7(4):276–9

Cullingham CI, Merrill EH, Pybus MJ, et al. Broad and fine-scale genetic analysis of white-tailed deer populations: estimating the relative risk of chronic wasting disease spread. Evol Appl 2011 Jan;4(1):116–31

Cullingham CI, Nakada SM, Merrill EH, et al. Multiscale population genetic analysis of mule deer (Odocoileus hemionus hemionus) in western Canada sheds new light on the spread of chronic wasting disease. Can J Zoo 2011 Jan 28;89(2):134–47

Davenport KA, Mosher BA, Brost BM, et al. Assessment of chronic wasting disease prion shedding in deer saliva with occupancy modeling. J Clin Microbiol 2017 Dec 26;56(1):e01243-17

DeVivo MT, Edmunds DR, Kauffman MJ, et al. Endemic chronic wasting disease causes mule deer population decline in Wyoming. PLoS One 2017 Oct 19;12(10):e0186512

Duque Velasquez C, Kim C, Herbst A, et al. Deer prion proteins modulate the emergence and adaptation of chronic wasting disease strains. J Virol 2015 Dec;89(24):12362–73

Ernest HB, Hoar BR, Well JA, et al. Molecular genealogy tools for white-tailed deer with chronic wasting disease. Can J Vet Res 2010 Apr;74(2):153–6

Fox KA, Jewell JE, Williams ES, et al. Patterns of PrPCWD accumulation during the course of chronic wasting disease infection in orally inoculated mule deer (Odocoileus hemionus). J Gen Virol 2006 Nov;87(Pt 11):3451–61

Geremia C, Hoeting JA, Wolfe LL, et al. Age and repeated biopsy influence antemortem PrP(CWD) testing in mule deer (Odocoileus hemionus) in Colorado, USA. J Wildl Dis 2015 Oct;51(4):801–10

Goldmann, W. PrP genetics in ruminant transmissible spongiform encephalopathies. Vet Res 2008 Jul-Aug;39(4):30

Gossert AD, Bonjour S, Lysek DA, et al. Prion protein NMR structures of elk and of mouse/elk hybrids. Proc Natl Acad Sci U S A 2005 Jan 18;102(3):646–50

Grear DA, Samuel MD, Scribner KT, et al. Influence of genetic relatedness and spatial proximity on chronic wasting disease infection among female white-tailed deer. J Appl Ecol 2010 Apr 28;47(3):532–40

Green KM, Browning SR, Seward TS, et al. The elk PRNP codon 132 polymorphism controls cervid and scrapie prion propagation. J Gen Virol 2008 Feb;89(Pt 2):598–608

Green ML, Manjerovic MB, Mateus-Pinilla N, et al. Genetic assignment tests reveal dispersal of white-tailed deer: implications for chronic wasting disease. J Mammal 2014 Jun 26;95(3):646–54

Haley NJ, Henderson DM, Wycoff S, et al. Chronic wasting disease management in ranched elk using rectal biopsy testing. Prion 2018 Mar 4;12(2):93–108

Haley NJ, Hoover EA. Chronic wasting disease of cervids: current knowledge and future perspectives. Annu Rev Anim Biosci 2015;3(1):305–25

Haley NJ, Richt JA. Evolution of diagnostic tests for chronic wasting disease, a naturally occurring prion disease of cervids. Pathogens 2017 Aug 5;6(3):E35

Haley NJ, Rielinger R, Davenport KA, et al. Estimating chronic wasting disease susceptibility in cervids using real-time quaking-induced conversion. J Gen Virol 2017 Nov;98(11):2882–92

Haley NJ, Siepker C, Walter WD, et al. Antemortem detection of chronic wasting disease prions in nasal brush collections and rectal biopsy specimens from white-tailed deer by real-time quaking-induced conversion. J Clin Microbiol 2016 Apr 1;54(4):1108–16

Hamir AN, Gidlewski T, Spraker TR, et al. Preliminary observations of genetic susceptibility of elk (Cervus elaphus nelsoni) to chronic wasting disease by experimental oral inoculation. J Vet Diagn Invest 2006 Jan;18(1):110–4

Hannaoui S, Amidian S, Cheng YC, et al. Destabilizing polymorphism in cervid prion protein hydrophobic core determines prion conformation and conversion efficiency. PLoS Pathog 2017 Aug 11;13(8):e1006553

Hannaoui S, Schatzl HM, Gilch S. Chronic wasting disease: emerging prions and their potential risk. PLoS Pathog 2017 Nov 2;13(11):e1006619

Happ GM, Huson HJ, Beckmen KB, et al. Prion protein genes in caribou from Alaska. J Wildl Dis 2007 Apr;43(2):224–8

Heaton MP, Leymaster KA, Freking BA, et al. Prion gene sequence variation within diverse groups of US sheep, beef cattle, and deer. Mamm Genome 2003 Nov;14(11):765–77

Henderson DM, Denkers ND, Hoover CE, et al. Longitudinal detection of prion shedding in saliva and urine by chronic wasting disease-infected deer by real-time quaking-induced conversion. J Virol 2015 Sep;89(18):9338–47

Huson HJ, Happ GM. Polymorphisms of the prion protein gene (PRNP) in Alaskan moose (Alces alces gigas). Anim Genet 2006 Aug;37(4):425–6

Jeong HJ, Lee JB, Park SY, et al. Identification of single-nucleotide polymorphisms of the prion protein gene in sika deer (Cervus nippon laiouanus). J Vet Sci 2007 Sep;8(3):299–301

Jewell JE, Conner MM, Wolfe LL, et al. Low frequency of PrP genotype 225SF among free-ranging mule deer (Odocoileus hemionus) with chronic wasting disease. J Gen Virol 2005 Aug;86(Pt 8):2127–34

Johnson CJ, Herbst A, Duque-Velasquez C, et al. Prion protein polymorphisms affect chronic wasting disease progression. PloS One 2011 Mar 18;6(3):e17450

Johnson C, Johnson J, Clayton M, et al. Prion protein gene heterogeneity in free-ranging white-tailed deer within the chronic wasting disease affected region of Wisconsin. J Wildl Dis 2003 Jul;39(3):576–81

Johnson C, Johnson J, Vanderloo JP, et al. Prion protein polymorphisms in white-tailed deer influence susceptibility to chronic wasting disease. J Gen Virol 2006 Jul 1;87(7):2109–14

Keane DP, Barr DJ, Bochsler PN, et al. Chronic wasting disease in a Wisconsin white-tailed deer farm. J Vet Diagn Invest 2008 Sep;20(5):698–703

Keeler SP, Bernarsky NL, Huffman JE, et al. A survey of the prion protein gene heterogeneity in New Jersey white-tailed deer (Odocoileus virginianus). J Pa Acad Sci 2011 Dec;85(4):183–7

Kelly AC, Mateus-Pinilla NE, Brown W, et al. Genetic assessment of environmental features that influence deer dispersal: implications for prion-infected populations. Popul Ecol 2014 Jan 8;56(2):327–40

Kelly AC, Mateus-Pinilla NE, Diffendorfer J, et al. Prion sequence polymorphisms and chronic wasting disease resistance in Illinois white-tailed deer (Odocoileus virginianus). Prion 2(1):28–36

Kelly AC, Mateus‐Pinilla NE, Douglas M, et al. Utilizing disease surveillance to examine gene flow and dispersal in white-tailed deer. J Appl Ecol 2010 Sep 14;47(6):1189–98

Kim HJ, Lee WY, Kim MJ, et al. Gene expression profile of a persistently chronic wasting disease (CWD) prion-infected RK13 cell line. Prev Vet Med 2012;36(4):186-195

Kramm C, Pritzkow S, Lyon A, et al. Detection of prions in blood of cervids at the asymptomatic stage of chronic wasting disease. Sci Rep 2017 Dec 8;7(1):17241 

Kurt TD, Telling GC, Zabel MD, et al. Trans-species amplification of PrPCWD and correlation with rigid loop 170N. Virology 2009 Apr 25;387(1):235–43

Kyle LM, John TR, Schaetzl HM, et al. Introducing a rigid loop structure from deer into mouse prion protein increases its propensity for misfolding in vitro. PLoS One 2013 Jun 25;8(6):e66715

Lang KR, Blanchong JA. Population genetic structure of white-tailed deer: understanding risk of chronic wasting disease spread. J Wildl Manage 2011 Nov 18;76(4):832–40

Li L, Napper S, Cashman NR. Immunotherapy for prion diseases: opportunities and obstacles. Immunotherapy 2010 Mar;2(2):269–82

Lloyd S, Mead S, Collinge J. Genetics of prion disease. Top Curr Chem 2011;305:1–22

Magle SB, Samuel MD, Van Deelen TR, et al. Evaluating spatial overlap and relatedness of white-tailed deer in a chronic wasting disease management zone. PLoS One 2013 Feb 20;8(2): e56568

Matsumoto T, Samuel MD, Bollinger T, et al. Association mapping of genetic risk factors for chronic wasting disease in wild deer. Evol Appl 2013 Feb;6(2):340–52

Meade-White K, Race B, Trifilo M, et al. Resistance to chronic wasting disease in transgenic mice expressing a naturally occurring allelic variant of deer prion protein. J Virol 2007 May;81(9):4533–9

Michel B, Ferguson A, Johnson T, et al. Complement protein C3 exacerbates prion disease in a mouse model of chronic wasting disease. Int Immunol 2013 Dec;25(12):697–702

Miller MW, Wolfe LL, Sirochman TM, et al. Survival patterns in white-tailed and mule deer after oral inoculation with a standardized, conspecific prion dose. J Wildl Dis 2012 Apr;48(2):526–9

Miller WL, Walter WD. Spatial heterogeneity of prion gene polymorphisms in an area recently infected by chronic wasting disease. Prion 2019 Jan;13(1):65–76

Mitchell GB, Sigurdson CJ, O’Rourke KI, et al. Experimental oral transmission of chronic wasting disease to reindeer (Rangifer tarandus tarandus). PloS One 2012;7(6):e39055

Monello RJ, Galloway NL, Powers JG, et al. Pathogen-mediated selection in free-ranging elk populations infected by chronic wasting disease. Proc Natl Acad U S A 2017 Nov 14;114(46):12208–12

Moore SJ, Vrentas CE, Hwang S, et al. Pathologic and biochemical characterization of PrPSc from elk with PRNP polymorphisms at codon 132 after experimental infection with the chronic wasting disease agent. BMC Vet Res 2018 Mar 9;14(1):80

O’Rourke KI, Besser TE, Miller MW, et al. PrP genotypes of captive and free-ranging Rocky Mountain elk (Cervus elaphus nelsoni) with chronic wasting disease. J Gen Virol 1999 Oct 1;80(10):2765–2679

O’Rourke KI, Spraker TR, Hamburg LK, et al. Polymorphisms in the prion precursor functional gene but not the pseudogene are associated with susceptibility to chronic wasting disease in white-tailed deer. J Gen Virol 2004 May;85(Pt 5):1339–46

O’Rourke KI, Spraker TR, Zhuang D, et al. Elk with a long incubation prion disease phenotype have a unique PrPd profile. Neuroreport 2007 Dec 3;18(18):1935–8

Otero A, Duque Velasquez C, Johnson C, et al. Prion protein polymorphisms associated with reduced CWD susceptibility limit peripheral PrPCWD deposition in orally infected white-tailed deer. BMC Vet Res 2019 Feb 4;15(1):50

Peletto S, Perucchini M, Acin C, et al. Genetic variability of the prion protein gene (PRNP) in wild ruminants from Italy and Scotland. J Vet Sci 2009 Jun;10(2):115–20

Perucchini M, Griffin K, Miller MW, et al. PrP genotypes of free-ranging wapiti (Cervus elaphus nelsoni) with chronic wasting disease. J Gen Virol 2008 May;89(Pt 5):1324–8

Pitarch JL, Raksa HC, Arnal MC, et al. Low sequence diversity of the prion protein gene (PRNP) in wild deer and goat species from Spain. Vet Res 2018 Apr 10;49(1):33

Race B, Meade-White K, Miller MW, et al. In vivo comparison of chronic wasting disease infectivity from deer with variation at prion protein residue 96. J Virol 2011 Sep;85(17):9235–8

Robinson SJ, Samuel MD, Johnson CJ, et al. Emerging prion disease drives host selection in a wildlife population. Ecol App 2012 Apr;22(3):1050–9

Robinson SJ, Samuel MD, O’Rourke KI, et al. The role of genetics in chronic wasting disease of North American cervids. Prion 2012 Jun;6(2):153–62

Robinson SJ, Samuel MD, Rolley RE, et al. Using landscape epidemiological models to understand the distribution of chronic wasting disease in the midwestern USA. Landsc Ecol 2013 Dec;28(10):1923–35

Rogers KG, Robinson SJ, Samuel MD, et al. Diversity and distribution of white-tailed deer MtDNA lineages in chronic wasting disease (CWD) outbreak areas in southern Wisconsin, USA. J Toxicol Environ Health A2011;74(22–24):1521–35

Seabury CM, Gill CA, Templeton JW, et al. Molecular characterization of the Rocky Mountain elk (Cervus elaphus nelsoni) PRNP putative promoter. J Hered 2007 Nov;98(7):678–86

Sigurdson CJ. A prion disease of cervids: chronic wasting disease. Vet Res 2008 Jul-Aug;39(4):41

Sigurdson CJ, Aguzzi A. Chronic wasting disease. Biochim Biophys Acta 2007 Jun;1772(6):610–8

Sigurdson CJ, Miller MW. Other animal prion diseases. Br Med Bull 2003 Jun;66:199-212

Sigurdson CJ, Nilsson KPR, Hornemann S, et al. A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest 2010 Jul;120(7):2590–9

Spraker TR, O’Rourke KI, Gidlewski T, et al. 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). Vet Pathol 2010 May;47(3):536–46

Tamguney G, Giles K, Oehler A, et al. Chimeric elk/mouse prion proteins in transgenic mice. J Gen Virol 2013 Feb 1;94(2):443–52

Thomsen BV, Schneider DA, O’Rourke KI, et al. Diagnostic accuracy of rectal mucosa biopsy testing for chronic wasting disease within white-tailed deer (Odocoileus virginianus) herds in North America: effects of age, sex, polymorphism at PRNP codon 96, and disease progression. J Vet Diagn Invest 2012 Sep;24(5):878–87

White SN, Spraker TR, Reynolds JO, et al. Association analysis of PRNP gene region with chronic wasting disease in Rocky Mountain elk. BMC Res Notes 2010 Nov 18;3:314

Williams AL, Kreeger TJ, Schumaker BA. Chronic wasting disease model of genetic selection favoring prolonged survival in Rocky Mountain elk (Cervus elaphus). Ecosphere 2014 May 22;5(5)

Williams ES. Chronic wasting disease. Vet Pathol 2005;20

Williams ES, Miller MW. Transmissible spongiform encephalopathies in non-domestic animals: origin, transmission and risk factors. Rev Sci Tech 2003 Apr;22(1):145–56

Wilson GA, Nakada SM, Bollinger TK, et al. Polymorphisms at the PRNP gene influence susceptibility to chronic wasting disease in two species of deer (Odocoileus spp.) in western Canada. J Toxicol Environ Health A 2009;72(17–18):1025–9

Wolfe LL, Fox KA, Miller MW. “Atypical” chronic wasting disease in PRNP genotype 225FF mule deer. J Wildl Dis 2014 Jul;50(3):660–5

Wolfe LL, Spraker TR, Gonzalez L, et al. PrPCWD in rectal lymphoid tissue of deer (Odocoileus spp.). J Gen Virol 2007 Jul;88(7):2078–82

Wrathall AE, Holyoak GR, Parsonson IM, et al. Risks of transmitting ruminant spongiform encephalopathies (prion diseases) by semen and embryo transfer techniques. Theriogenology 2008 Sep 15;70(5):725–45 (published online Jun 30)

Human susceptibility

Abrams JY, Maddox RA, Harvey AR, et al. Travel history, hunting, and venison consumption related to prion disease exposure, 2006-2007 FoodNet population survey. J Am Diet Assoc 2011 Jun;111(6):858–63

Anderson CA, Bosque P, Filley CM, et al. Colorado surveillance program for chronic wasting disease transmission to humans: lessons from 2 highly suspicious but negative cases. Arch Neurol 2007 Mar;64(3):439–41

Angers RC, Browning SR, Seward TS, et al. Prions in skeletal muscles of deer with chronic wasting disease. Science 2006 Feb 24;311(5764):1117

Angers RC, Seward TS, Napier D, et al. Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis 2009 May;15(5):696–703

Apostol MI, Wiltzius JJW, Sawaya MR, et al. Atomic structures suggest determinants of transmission barriers in mammalian prion disease. Biochemistry 2011 Feb 16;50(13):2456–63

Barria MA, Balachandran A, Morita M, et al. Molecular barriers to zoonotic transmission of prions. Emerg Infect Dis 2014 Jan;20(1):88–97

Barria MA, Ironside JW, Head MW. Exploring the zoonotic potential of animal prion diseases. Prion 2014 Feb 18;8(1):85–91

Barria MA, Libori A, Mitchell G, et al. Susceptibility of human prion protein to conversion by chronic wasting disease prions. Emerg Infect Dis 2018 Aug;24(8):1482–9

Barria MA, Telling GC, Gambetti P, et al. Generation of a new form of human PrP(Sc) in vitro by interspecies transmission from cervid prions. J Biol Chem 2011 Mar 4;286(9):7490–5

Belay ED, Bartz JC. Prion diseases. Viral infections of humans. Boston, MA: Springer US; 2014 Jun 28:1165–86

Belay ED, Gambetti P, Schonberger LB, et al. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol 2001 Oct;58(10):1673–8

Belay ED, Maddox RA, Williams ES, et al. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis 2004 Jun;10(6):977–84

Belay ED, Schonberger LB. The public health impact of prion diseases. Annu Rev Public Health 2004 Dec;26:191–212

Benestad SL, Telling GC. Chronic wasting disease: an evolving prion disease of cervids. Handb Clin Neurol 2018;153:135–51

Beringue V, Herzog L, Jaumain E, et al. Facilitated cross-species transmission of prions in extraneural tissue. Science 2012 Jan 27;335(6067):472–5

Bett C, Fernandez-Borges N, Kurt TD, et al. Structure of the β2-α2 loop and interspecies prion transmission. FASEB J 2012 Jul;26(7):2868–76 (published online Apr 9)

Bian J, Christiansen JR, Moreno JA, et al. Primary structural differences at residue 226 of deer and elk PrP dictate selection of distinct CWD prion strains in gene-targeted mice. Proc Natl Acad Sci 2019 May 30;201903947

Bosque PJ. Bovine spongiform encephalopathy, chronic wasting disease, scrapie, and the threat to humans from prion disease epizootics. Curr Neurol Neurosci Rep 2002 Nov;2(6):488–95

CDC. Fatal degenerative neurologic illnesses in men who participated in wild game feasts – Wisconsin, 2002. MMWR 2003 Feb 21;52(7):125-7

Collinge J. The risk of prion zoonoses. Science 2012 Jan 27;335(6067):411–3

Comoy EE, Mikol J, Luccantoni-Freire S, et al. Transmission of scrapie prions to primate after an extended silent incubation period. Sci Rep 2015 Jun 30;5(1):1–11

Cervenakova L, Sigurdson C, Piccardo P, et al. Successful transmission of chronic wasting disease (CWD) into mice over-expressing bovine prion protein (TgSB3985). Prion 2014 Apr;8

Daus ML, Breyer J, Wagenfuehr K, et al. Presence and seeding activity of pathological prion protein (PrPTSE) in skeletal muscles of white-tailed deer infected with chronic wasting disease. PLoS One 2011 Apr 1;6(4):e18345

Davenport KA, Henderson DM, Bian J, et al. Insights into chronic wasting disease and bovine spongiform encephalopathy species barriers by use of real-time conversion. J Virol 2015 Sep;89(18):9524–31

European Food Safety Authority. Joint scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans. EFSA Journal 2011;9(1):1945

Garruto RM, Reiber C, Alfonso MP, et al. Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease. Environ Health 2008 Jun 24;7(1):31

Haley NJ, Hoover EA. Chronic wasting disease of cervids: current knowledge and future perspectives. Annu Rev Anim Biosci 2015;3(1):305–25

Hannaoui S, Schatzl HM, Gilch S. Chronic wasting disease: emerging prions and their potential risk. PLoS Pathog 2017 Nov 2;13(11):e1006619

Jewell JE, Brown J, Kreeger T, et al. Prion protein in cardiac muscle of elk (Cervus elaphus nelsoni) and white-tailed deer (Odocoileus virginianus) infected with chronic wasting disease. J Gen Virol 2006 Nov;87(Pt 11):3443–50

Johnson CJ, Carlson CM, Morawski AR, et al. Assessing transmissible spongiform encephalopathy species barriers with an in vitro prion protein conversion assay. J Vis Exp 2015 Mar 10;(97):e52522

Kong Q, Huang S, Zou W, et al. Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. J Neurosci 2005 Aug 31;25(35):7944–9

Kurt TD, Jiang L, Fernandez-Borges N, et al. Human prion protein sequence elements impede cross-species chronic wasting disease transmission. J Clin Invest 2015 Apr;125(4):1485–96

Kurt TD, Sigurdson CJ. Cross-species transmission of CWD prions. Prion 2016;10(1):83–91

Kurt TD, Telling GC, Zabel MD, et al. Trans-species amplification of PrPCWD and correlation with rigid loop 170N. Virology 2009 Apr 25;387(1):235–43

Li L, Coulthart MB, Balachandran A, et al. Species barriers for chronic wasting disease by in vitro conversion of prion protein. Biochem Biophys Res Commun 2007 Dec 28;364(4):796–800

Luers L, Bannach O, Stohr J, et al. Seeded fibrillation as molecular basis of the species barrier in human prion diseases. PLoS One 2013 Aug 20;8(8):e72623

Marsh RF, Kincaid AE, Bessen RA, et al. Interspecies transmission of chronic wasting disease prions to squirrel monkeys (Saimiri sciureus). J Virol 2005 Nov;79(21):13794–6

Moreno JA, Telling GC. Molecular mechanisms of chronic wasting disease prion propagation. Cold Spring Harb Perspect Med 2018 Jun 1;8(6):a024448

Olszowy KM, Lavelle J, Rachfal K, et al. Six-year follow-up of a point-source exposure to CWD contaminated venison in an upstate New York community: risk behaviours and health outcomes 2005-2011. Public Health 2014 Sep;128(9):860–8

Osterholm MT, Anderson CJ, Zabel MD, et al. Chronic wasting disease in cervids: implications for prion transmission to humans and other animal species. MBio 2019 Jul;10(4):e01091-19

Pape WJ, Forster JE, Anderson CA, et al. Human prion disease and relative risk associated with chronic wasting disease. Emerg Infect Dis 2006 Oct;12(10):1527–35

Prcina M, Bardon J, Kontsekova E. Chronic wasting disease. Acta Virol 2008;52(4):209–18

Race B, Meade-White KD, Miller MW, et al. Susceptibilities of nonhuman primates to chronic wasting disease. Emerg Infect Dis 2009 Sep;15(9):1366–76

Race B, Meade-White KD, Phillips K, et al. Chronic wasting disease agents in nonhuman primates. Emerg Infect Dis 2014 May;20(5):833–7

Race B, Williams K, Chesebro B. Transmission studies of chronic wasting disease to transgenic mice overexpressing human prion protein using the RT-QuIC assay. Vet Res 2019 Jan 22;50(1):6

Race B, Williams K, Orru CD, et al. Lack of transmission of chronic wasting disease to cynomolgus macaques. J Virol 2018 Jul 15;92(14)

Raymond GJ, Bossers A, Raymond LD, et al. Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease. EMBO J 2000 Sep 1;19(17):4425–30

Salman MD. Chronic wasting disease in deer and elk: scientific facts and findings. J Vet Med Sci 2003 Jul;65(7):761–8

Sandberg MK, Al-Doujaily H, Sigurdson CJ, et al. Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. J Gen Virol 2010 Oct;91(Pt 10):2651-7

Saunders SE, Bartelt-Hunt SL, Bartz JC. Occurrence, transmission, and zoonotic potential of chronic wasting disease. Emerg Infect Dis 2012 Mar;18(3):369-76

Schmaedicke AC. Estimating the risk of CWD transmission to humans—an interim report of a comprehensive study in non-human primates. Prion 2012 Apr;6:67–8

Sejvar JJ, Schonberger LB, Belay ED. Transmissible spongiform encephalopathies. J Am Vet Med Associ 2008 Dec 1;233(11):1705–12

Sigurdson CJ, Aguzzi A. Chronic wasting disease. Biochim Biophys Acta 2007 Jun;1772(6):610–8 

Sigurdson CJ, Miller MW. Other animal prion diseases. Br Med Bull 2003 Jun;66:199-212

Sigurdson CJ, Nilsson KPR, Hornemann S, et al. A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest 2010 Jul;120(7):2590–9

Tamguney G, Giles K, Bouzamondo-Bernstein E, et al. Transmission of elk and deer prions to transgenic mice. J Virol 2006 Sep;80(18):9104-14

Telling G. Chronic wasting disease and the development of research models. Prions and Diseases 2012 Oct 15:45–57

Waddell L, Greig J, Mascarenhas M, et al. Current evidence on the transmissibility of chronic wasting disease prions to humans—a systematic review. Transbound Emerg Dis 2018 Feb;65(1):37–49

Ward HJT, Knight RSG. Transmissible spongiform encephalopathy and meat safety. Safety of Meat and Processed Meat. Food Microbiology and Safety. New York, NY: Springer New York; 2009 Feb 28:125–46

Wilson R, Plinston C, Hunter N, et al. Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein. J Gen Virol 2012 Jul;93(Pt 7):1624-9

Management

Al-Arydah M, Smith RJ, Lutscher F. Modeling gender-structured wildlife diseases with harvesting: chronic wasting disease as an example. Int Sch Res Notices 2012;2012

Almberg ES, Cross PC, Johnson CJ, et al. Modeling routes of chronic wasting disease transmission: environmental prion persistence promotes deer population decline and extinction. PLoS One 2011 May 13;6(5):e19896

Amick K, Clark D, Brook RK. Stakeholder perspectives on chronic wasting disease risk and management on the Canadian prairies. Hum Dimens Wildl 2015 Aug 31;20(5):408–24

Argue CK, Ribble C, Lees VW, et al. Epidemiology of an outbreak of chronic wasting disease on elk farms in Saskatchewan. Can Vet J 2007 Dec;48(12):1241–8

Arnot C, Laate E, Unterschultz J, et al. Chronic wasting disease (CWD) potential economic impact on cervid farming in Alberta. J Toxicol Environ Health A 2009 Aug 20;72(17–18):1014–7

Baeten LA, Powers BE, Jewell JE, et al. A natural case of chronic wasting disease in a free-ranging moose (Alces alces shirasi). J Wildl Dis 2007;43(2):309–14

Basu U, Almeida LM, Dudas S, et al. Gene expression alterations in Rocky Mountain elk infected with chronic wasting disease. Prion 2012 Jul 1;6(3):282–301

Ball K. Chronic wasting disease in a Rocky Mountain elk. Can Vet J;43(11):880–2

Beringer J, Hansen LP, Millspaugh JJ, et al. A statewide surveillance effort for detecting chronic wasting disease in wild white-tailed deer in Missouri. Wildl Soc Bull 2003;31(3):873–81

Bishop RC. The economic impacts of chronic wasting disease (CWD) in Wisconsin. Hum Dimens Wildl 2004;9(3):181–92

Blanchong JA, Grear DA, Weckworth BV, et al. Effects of chronic wasting disease on reproduction and fawn harvest vulnerability in Wisconsin white-tailed deer. J Wildl Dis 2012 Apr;48(2):361–70

Blanchong JA, Joly DO, Samuel MD, et al. White-tailed deer harvest from the chronic wasting disease eradication zone in south-central Wisconsin. Wildl Soc Bull 2006 Oct;34(3):725–31

Blanchong JA, Samuel MD, Scribner KT, et al. Landscape genetics and the spatial distribution of chronic wasting disease. Biol Lett 2008 Feb 23;4(1):130–3

Booth CJ, Johnson CJ, Pedersen JA. Microbial and enzymatic inactivation of prions in soil environments. Soil Biol Biochem 2013;59:1–15

Bosschere HD, Saegerman C, Neukermans A, et al. First chronic wasting disease (CWD) surveillance of roe deer (Capreolus capreolus) in the northern part of Belgium. Vet Q 2006;28(2):54–60

Brown TL, Decker DJ, Major JT, et al. Hunters’ and other citizens’ reactions to discovery of CWD in central New York. Hum Dimens Wildl 2006;11(3):203–14

Clements GM, Hygnstrom SE, Gilsdorf JM, et al. Movements of white-tailed deer in riparian habitat: implications for infectious diseases. J Wildl Manage 2011 Jul 22;75(6):1436–42

Conner MM, Ebinger MR, Blanchong JA, et al. Infectious disease in cervids of North America. Ann N Y Acad Sci 2008;1134:146–72

Conner MM, McCarty CW, Miller MW. Detection of bias in harvest-based estimates of chronic wasting disease prevalence in mule deer. J Wildl Dis 2000 Oct;36(4):691–9

Conner MM, Miller MW. Movement patterns and spatial epidemiology of a prion disease in mule deer population units. Ecol Appl 2004 Dec;14(6):1870–81

Conner MM, Miller MW, Ebinger MR, et al. A meta-baci approach for evaluating management intervention on chronic wasting disease in mule deer. Ecol Appl 2007 Jan;17(1):140–53

Cooney EE, Holsman RH. Influences on hunter support for deer herd reduction as a chronic wasting disease (CWD) management strategy. Hum Dimens Wildl 2010 May 18;15(3):194–207

Cullingham CI, Merrill EH, Pybus MJ, et al. Broad and fine-scale genetic analysis of white-tailed deer populations: estimating the relative risk of chronic wasting disease spread. Evol Appl 2011 Jan;4(1):116–31

Cullingham CI, Nakada SM, Merrill EH, et al. Multiscale population genetic analysis of mule deer (Odocoileus hemionus hemionus) in western Canada sheds new light on the spread of chronic wasting disease. Can J Zoo 2011 Jan 28;89(2):134–47

Diefenbach DR, Rosenberry CS, Boyd RC. From the field: efficacy of detecting chronic wasting disease via sampling hunter-killed white-tailed deer. Wildl Soc Bull 2004;32(1):267-72 

Dulberger J, Hobbs NT, Swanson HM, et al. Estimating chronic wasting disease effects on mule deer recruitment and population growth. J Wildl Dis 2010;46(4):1086–95

Eschenfelder KR. What information should state wildlife agencies provide on their CWD websites? Hum Dimens Wildl 2006;11(3):221–3

Eschenfelder KR, Miller CA. Examining the role of web site information in facilitating different citizen–government relationships: a case study of state chronic wasting disease web sites. Gov Inf Q 2007 Jan;24(1):64–88

Evans TS, Kirchgessner MS, Eyler B, et al. Habitat influences distribution of chronic wasting disease in white-tailed deer. J Wildl Manage 2015 Nov 26;80(2):284–91 

Evans TS, Schuler KL, Walter WD. Surveillance and monitoring of white-tailed deer for chronic wasting disease in the northeastern United States. J Fish Wildl Manage 2014 Jun;5(2):387–93

Farnsworth ML, Hoeting JA, Hobbs NT, et al. Linking chronic wasting disease to mule deer movement scales: a hierarchical Bayesian approach. Ecol Appl 2006 Jun;16(3):1026–36

Farnsworth ML, Wolfe LL, Hobbs NT, et al. Human land use influences chronic wasting disease prevalence in mule deer. Ecol App 2005 Feb;15(1):119–26

Fischer JW, Phillips GE, Baasch DM, et al. Modifying elk (Cervus elaphus) behavior with electric fencing at established fence-lines to reduce disease transmission potential. Wildl Soc Bull 2011 Mar 30;35(1):9–14

Frost CJ, Hygnstrom SE, Tyre AJ, et al. Probabilistic movement model with emigration simulates movements of deer in Nebraska, 1990–2006. Ecol Modell 2009 Oct 10;220(19):2481–90

Garlick MJ, Powell JA, Hooten MB, et al. Homogenization, sex, and differential motility predict spread of chronic wasting disease in mule deer in southern Utah. J Math Biol 2013 Jul 12;69(2):369–99

Geremia C, Miller MW, Hoeting JA, et al. Bayesian modeling of prion disease dynamics in mule deer using population monitoring and capture-recapture data. PLoS One 2015 Oct 28;10(10):e0140687

Gigliotti LM. Hunters’ concerns about chronic wasting disease in South Dakota. Hum Dimens Wildl 2004;9(3):233–5

Gillin CM, Mawdsley JR. AFWA technical report on best management practices for surveillance, management and control of chronic wasting disease. AFWA. 2018

Grear DA, Samuel MD, Langenberg JA, et al. Demographic patterns and harvest vulnerability of chronic wasting disease infected white-tailed deer in Wisconsin. J Wildl Manage 2006 Apr;70(2):546–53

Green ML, Manjerovic MB, Mateus-Pinilla N, et al. Genetic assignment tests reveal dispersal of white-tailed deer: implications for chronic wasting disease. J Mammal 2014 Jun 26;95(3):646–54

Gross JE, Miller MW. Chronic wasting disease in mule deer: disease dynamics and control. J Wildl Manage 2001;65(2):205–15

Habib TJ, Merrill EH, Pybus MJ, et al. Modelling landscape effects on density–contact rate relationships of deer in eastern Alberta: implications for chronic wasting disease. Ecol Modell 2011 Aug 10;222(15):2722–32

Haigh JC, Mackintosh C, Griffin F. Viral, parasitic and prion diseases of farmed deer and bison. Rev Sci Tech 2002 Aug;21(2):219–48

Haley NJ, Hoover EA. Chronic wasting disease of cervids: current knowledge and future perspectives. Annu Rev Anim Biosci 2015;3(1):305–25

Haley NJ, Henderson DM, Wycoff S, et al. Chronic wasting disease management in ranched elk using rectal biopsy testing. Prion 2018 Mar 4;12(2):93–108

Hannaoui S, Schatzl HM, Gilch S. Chronic wasting disease: emerging prions and their potential risk. PLoS Pathog 2017 Nov 2;13(11):e1006619

Harper EE, Miller CA, Vaske JJ. Hunter perceptions of risk, social trust, and management of chronic wasting disease in Illinois. Hum Dimens Wildl 2015 Jul 13;20(5):394–407

Heberlein TA. ‘Fire in the Sistine Chapel’: how Wisconsin responded to chronic wasting disease. Hum Dimens Wildl 2004 Jul;9(3):165–79

Heberlein TA, Stedman RC. Socially amplified risk: attitude and behavior change in response to CWD in Wisconsin deer. Hum Dimens Wildl 2009 Nov 6;14(5):326–40

Hedlin P, Taschuk R, Potter A, et al. Detection and control of prion diseases in food animals. Int Sch Res Notices 2012

Heisey DM, Osnas EE, Cross PC, et al. Linking process to pattern: estimating spatiotemporal dynamics of a wildlife epidemic from cross-sectional data. Ecol Monogr 2010 May 1;80(2):221–40

Hinckley GT, Johnson CJ, Jacobson KH, et al. Persistence of pathogenic prion protein during simulated wastewater treatment processes. Environ Sci Technol 2008 Jun 10;42(14):5254–9

Holsman RH, Petchenik J. Predicting deer hunter harvest behavior in Wisconsin’s chronic wasting disease eradication zone. Hum Dimens Wildl 2006 Jul;11(3):177–89

Holsman RH, Petchenik J, Cooney EE. CWD after ‘the fire’: six reasons why hunters resisted Wisconsin’s eradication effort. Hum Dimens Wildl 2010 May 21;15(3):180–93

Imran M, Mahmood S. An overview of animal prion diseases. Virol J 2011 Nov;8(1):493

Jacques CN, Jenks JA, Jenny AL, et al. Prevalence of chronic wasting disease and bovine tuberculosis in free-ranging deer and elk in South Dakota. J Wildl Dis 2003 Jan;39(1):29–34

Jennelle CS, Henaux V, Wasserberg G, et al. Transmission of chronic wasting disease in Wisconsin white-tailed deer: implications for disease spread and management. PLoS One 2014 Mar 21;9(3):e91043

Jennelle CS, Samuel MD, Nolden CA, et al. Deer carcass decomposition and potential scavenger exposure to chronic wasting disease. J Wildl Dis 2009 Jul 1;73(5):655–62 

Jennelle CS, Samuel MD, Nolden CA, et al. Surveillance for transmissible spongiform encephalopathy in scavengers of white-tailed deer carcasses in the chronic wasting disease area of Wisconsin. J Toxicol Environ Health A 2009 Aug 20;72(17–18):1018–24

Johnson CJ, Bennett JP, Biro SM, et al. Degradation of the disease-associated prion protein by a serine protease from lichens. PLoS One 2011 May 11;6(5):e19836

Johnson C, Johnson J, Vanderloo JP, et al. Prion protein polymorphisms in white-tailed deer influence susceptibility to chronic wasting disease. J Gen Virol 2006 Jul 1;87(7):2109–14

Joly DO, Ribic CA, Langenberg JA, et al. Chronic wasting disease in free-ranging Wisconsin white-tailed deer. Emerg Infect Dis 2003 May;9(5):599–601

Joly DO, Samuel MD, Langenberg JA, et al. Spatial epidemiology of chronic wasting disease in Wisconsin white-tailed deer. J Wildl Dis 2006 Jul;42(3):578–88

Joly DO, Samuel MD, Langenberg JA, et al. Surveillance to detect chronic wasting disease in white-tailed deer in Wisconsin. J Wildl Dis 2009 Oct;45(4):989–97

Kahn S, Dube C, Bates L, et al. Chronic wasting disease in Canada: part 1. Can Vet J 2004 May;45(5):397–404

Kataoka N, Nishimura M, Horiuchi M, et al. Surveillance of chronic wasting disease in sika deer, Cervus nippon, from Tokachi District in Hokkaido. J Vet Med Sci 2005;67(3):349–51

Kellar JA, Lees VW. Risk management of the transmissible spongiform encephalopathies in North America. Rev Sci Tech 2003 Apr;22(1):201–25

Kelly AC, Mateus-Pinilla NE, Brown W, et al. Genetic assessment of environmental features that influence deer dispersal: implications for prion-infected populations. Popul Ecol 2014 Jan 8;56(2):327–40 

Kelly AC, Mateus‐Pinilla NE, Douglas M, et al. Utilizing disease surveillance to examine gene flow and dispersal in white-tailed deer. J Appl Ecol 2010 Sep 14;47(6):1189–98

Krumm CE, Conner MM, Hobbs NT, et al. Mountain lions prey selectively on prion-infected mule deer. Biol Lett 2010;6(2):209–11

Krumm CE, Conner MM, Miller MW. Relative vulnerability of chronic wasting disease infected mule deer to vehicle collisions. J Wildl Dis 2005 Jul;41(3):503–11 

Kuznetsova A, McKenzie D, Banser P, et al. Potential role of soil properties in the spread of CWD in western Canada. Prion 2014 Mar 11;8(1):92–9

Lang KR, Blanchong JA. Population genetic structure of white-tailed deer: understanding risk of chronic wasting disease spread. J Wildl Manage 2011 Nov 18;76(4):832–40

Lapointe JM, Leclair D, Mesher C, et al. Quebec: screening for chronic wasting disease in caribou in northern Quebec. Can Vet J 2002 Nov;43(11):886–7

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Leach SP, Salman MD, Hamar D. Trace elements and prion diseases: a review of the interactions of copper, manganese and zinc with the prion protein. Anim Health Res Rev 2006 June;7(1–2):97–105

Lischka SA, Shelton P, Buhnerkempe J. Support for chronic wasting disease management among residents of the infected area in Illinois. Hum Dimens Wildl 2010 May 21;15(3):229–32

Lupi O. Risk analysis of ectoparasites acting as vectors for chronic wasting disease. Med Hypotheses 2005;65(1):47–54

Lyon KM, Vaske JJ. Predicting hunting participation in response to chronic wasting disease in four states. Hum Dimens Wildl 2010 May 18;15(3):208–20

Magle SB. Movements and habitat interactions of white-tailed deer: implications for chronic wasting disease management. Am Midl Nat 2015 Apr;173(2):267–82

Magle SB, Chamberlin JC, Mathews NE. Survival of white-tailed deer in Wisconsin’s chronic wasting disease zone. Northeast Nat 2012 Mar 1;19(1):67–76

Magle SB, Samuel MD, Van Deelen TR, et al. Evaluating spatial overlap and relatedness of white-tailed deer in a chronic wasting disease management zone. PLoS One 2013 Feb 20;8(2): e56568

Manjerovic MB, Green ML, Mateus-Pinilla N, et al. The importance of localized culling in stabilizing chronic wasting disease prevalence in white-tailed deer populations. Prev Vet Med 2014;113(1):139–45

Mateus-Pinilla N, Weng HY, Ruiz MO, et al. Evaluation of a wild white-tailed deer population management program for controlling chronic wasting disease in Illinois, 2003–2008. Prev Vet Med 2013 Jul;110(3):541–8

Miller CA. Deer hunter participation and chronic wasting disease in Illinois: an assessment at time zero. Hum Dimens Wildl 2004;9(3):237–9

Miller CA. Hunter perceptions and behaviors related to chronic wasting disease in northern Illinois. Hum Dimens Wildl 2003 Jun;8(3):229–30

Miller CA, Shelby LB. Hunters’ general disease risk sensitivity and behaviors associated with chronic wasting disease. Hum Dimens Wildl 2009 Mar 24;14(2):133–41

Miller MW, Conner MM. Epidemiology of chronic wasting disease in free-ranging mule deer: spatial, temporal, and demographic influences on observed prevalence patterns. J Wildl Dis 2005;41(2):275–90

Miller MW, Hobbs NT, Tavener SJ. Dynamics of prion disease transmission in mule deer. Ecol Appl 2006 Dec;16(6):2208–14 

Miller MW, Swanson HM, Wolfe LL, et al. Lions and prions and deer demise. PLoS One 2008 Dec 24;3(12):e4019

Miller MW, Wild MA. Epidemiology of chronic wasting disease in captive white-tailed and mule deer. J Wildl Dis 2004 Apr;40(2):320–7

Miller MW, Wild MA, Williams ES. Epidemiology of chronic wasting disease in captive Rocky Mountain elk. J Wildl Dis 1998 Jul;34(3):532–8

Miller MW, Williams ES, Hobbs NT, et al. Environmental sources of prion transmission in mule deer. Emerg Infect Dis 2004 Jun;10(6)

Miller MW, Williams ES, McCarty CW, et al. Epizootiology of chronic wasting disease in free-ranging cervids in Colorado and Wyoming. J Wildl Dis 2000 Oct;36(4):676–90 

Miller MW, Wolfe LL, Sirochman TM, et al. Survival patterns in white-tailed and mule deer after oral inoculation with a standardized, conspecific prion dose. J Wildl Dis 2012 Apr;48(2):526–9

Monello RJ, Powers JG, Hobbs NT, et al. Survival and population growth of a free-ranging elk population with a long history of exposure to chronic wasting disease. J Wildl Manage 2014 Feb 6;78(2):214–23

Needham MD, Vaske JJ. Beliefs about chronic wasting disease risks across multiple states, years, and interest groups. Hum Dimens Wildl 2006 May;11(3):215–20 

Needham MD, Vaske JJ. Hunter perceptions of similarity and trust in wildlife agencies and personal risk associated with chronic wasting disease. Soc Nat Resour 2008 Feb 15;21(3):197–214

Needham MD, Vaske JJ, Donnelly MP, et al. Hunting specialization and its relationship to participation in response to chronic wasting disease. J Leis Res 2007;39(3):413–37

Needham MD, Vaske JJ, Manfredo MJ. Hunters’ behavior and acceptance of management actions related to chronic wasting disease in eight states. Hum Dimens Wildl 2004 Jul;9(3):211–31 

Needham MD, Vaske JJ, Manfredo MJ. State and residency differences in hunters’ responses to chronic wasting disease. Hum Dimens Wildl 2006 Jul;11(3):159–76

Nobert BR, Merrill EH, Pybus MJ, et al. Landscape connectivity predicts chronic wasting disease risk in Canada. J Appl Ecol 2016 Apr 12;53(5):1450–9

Nusser SM, Clark WR, Otis DL, et al. Sampling considerations for disease surveillance in wildlife populations. J Wildl Manage 2008 Jan 1;72(1):52–60

O’Hara Ruiz M, Kelly AC, Brown WM, et al. Influence of landscape factors and management decisions on spatial and temporal patterns of the transmission of chronic wasting disease transmission in white-tailed deer. Geospat Health 2013 Nov;8(1):215–27

Oraby T, Vasilyeva O, Krewski D, et al. Modeling seasonal behavior changes and disease transmission with application to chronic wasting disease. J Theor Biol 2014 Jan 7;340:50–9

Osnas EE, Heisey DM, Rolley RE, et al. Spatial and temporal patterns of chronic wasting disease: fine-scale mapping of a wildlife epidemic in Wisconsin. Ecol Appl 2009 Jul 1;19(5):1311–22

Oyer AM, Mathews NE, Skuldt LH. Long-distance movement of a white-tailed deer away from a chronic wasting disease area. J Wildl Manage 2007;71(5):1635–8

Pedersen JA, McMahon KD, Benson CH. Prions: novel pathogens of environmental concern? J Environ Eng (New York) 2006 Sep;132(9):967–9

Petchenik J. Landowner responses to harvest incentives in Wisconsin’s southwest chronic wasting disease eradication zone. Hum Dimens Wildl 2006;11(3):225–6

Pilon JL, Nash PB, Arver T, et al. Feasibility of infectious prion digestion using mild conditions and commercial subtilisin. J Virol Methods 2009 Oct;161(1):168–72 

Potapov A, Merrill E, Lewis MA. Wildlife disease elimination and density dependence. Proc R Soc Lond B Biol Sci 2012 May 16;279(1741):3139–45

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Potapov A, Merrill E, Pybus M, et al. Chronic wasting disease: transmission mechanisms and the possibility of harvest management. PLoS One 2016 Mar 10;11(3):e0151039

Potapov A, Merrill E, Pybus M, et al. Empirical estimation of R0 for unknown transmission functions: the case of chronic wasting disease in Alberta. PLoS One 2015 Oct 9;10(10):e0140024

Raymond GJ, Olsen EA, Lee KS, et al. Inhibition of protease-resistant prion protein formation in a transformed deer cell line infected with chronic wasting disease. J Virol 2006 Dec;80(2):596–604

Rees EE, Merrill EH, Bollinger TK, et al. Targeting the detection of chronic wasting disease using the hunter harvest during early phases of an outbreak in Saskatchewan, Canada. Prev Vet Med 2012 Apr 1;104(1):149–59

Robinson SJ, Samuel MD, Rolley RE, et al. Using landscape epidemiological models to understand the distribution of chronic wasting disease in the midwestern USA. Landsc Ecol 2013 Dec;28(10):1923–35

Rodriguez CM, Bennett JP, Johnson CJ. Lichens: unexpected anti-prion agents? Prion 2012 Jan;6(1):11–6

Roels S, Saegerman C, Bosschere HD, et al. First results of chronic wasting disease (CWD) surveillance in the south‐eastern part of Belgium. Vet Q 2006 Sep;27(3):98–104 

Rogers KG, Robinson SJ, Samuel MD, et al. Diversity and distribution of white-tailed deer MtDNA lineages in chronic wasting disease (CWD) outbreak areas in southern Wisconsin, USA. J Toxicol Environ Health A 2011;74(22–24):1521–35

Russell RE, Gude JA, Anderson NJ, et al. Identifying priority chronic wasting disease surveillance areas for mule deer in Montana. J Wildl Manage 2015;79(6):989–97

Russo F, Johnson CJ, Johnson CJ, et al. Pathogenic prion protein is degraded by a manganese oxide mineral found in soils. J Gen Virol 2009 Jan;90(1):275–80

Sargeant GA, Weber DC, Roddy DE. Implications of chronic wasting disease, cougar predation, and reduced recruitment for elk management. J Wildl Manage 2011 Jan 31;75(1):171–7

Saunders SE, Bartz JC, Bartelt-Hunt SL. Soil-mediated prion transmission: is local soil-type a key determinant of prion disease incidence? Chemosphere 2012 May;87(7):661–7 

Saunders SE, Bartz JC, Vercauteren KC, et al. Enzymatic digestion of chronic wasting disease prions bound to soil. Environ Sci Technol 2010;44(11):4129–35

Saunders SE, Yuan Q, Bartz JC, et al. Effects of solution chemistry and aging time on prion protein adsorption and replication of soil-bound prions. PLoS One 2011 Apr 19;6(4):e18752

Schauber E, Woolf A. Chronic wasting disease in deer and elk: a critique of current models and their application. Wildl Soc Bull 2003;31(3):610-6 

Schettler E, Steinbach F, Eschenbacher-Kaps I, et al. Surveillance for prion disease in cervids, Germany. Emerg Infect Dis 2006;12(2):319–22

Seidl AF, Koontz SR. Potential economic impacts of chronic wasting disease in Colorado. Hum Dimens Wildl 2004;9(3):241–5

Sharp A, Pastor J. Stable limit cycles and the paradox of enrichment in a model of chronic wasting disease. Ecol Appl 2011 Jun 1;21(4):1024–30

Sigurdson CJ, Aguzzi A. Chronic wasting disease. Biochim Biophys Acta 2007 Jun;1772(6):610–8

Sigurdson CJ, Miller MW. Other animal prion diseases. Br Med Bull 2003 Jun;66:199-212

Silbernagel ER, Skelton NK, Waldner CL, et al. Interaction among deer in a chronic wasting disease endemic zone. J Wildl Manage 2011 Jun 9;75(6):1453–61

Skuldt LH, Mathews NE, Oyer AM. White-tailed deer movements in a chronic wasting disease area in south-central Wisconsin. J Wildl Manage 2008;72(5):1156–60 

Song HR, Lawson A. Space-time Bayesian survival modeling of chronic wasting disease in deer. Prev Vet Med 2009 Sep;91(1):46–54

Sohn HJ, Kim JH, Choi KS, et al. A case of chronic wasting disease in an elk imported to Korea from Canada. J Vet Med Sci 2002;64(9):855–8

Sorensen A, van Beest FM, Brook RK. Impacts of wildlife baiting and supplemental feeding on infectious disease transmission risk: a synthesis of knowledge. Prev Vet Med 2014;113(4):356–63

Stafford NT, Needham MD, Vaske JJ, et al. Hunter and nonhunter beliefs about chronic wasting disease in Wisconsin. J Wildl Manage 2007 Jul 1;71(5):1739–44

Storm DJ, Samuel MD, Rolley RE, et al. Deer density and disease prevalence influence transmission of chronic wasting disease in white-tailed deer. Ecosphere 2013 Jan 17;4(1):10

Thompson AK, Samuel MD, Deelen TRV. Alternative feeding strategies and potential disease transmission in Wisconsin white-tailed deer. J Wildl Manage 2008 Feb 1;72(2):416–21

Travis D, Miller M. A short review of transmissible spongiform encephalopathies, and guidelines for managing risks associated with chronic wasting disease in captive cervids in zoos. J Zoo Wildl Med 2003 Jun;34(2):125–33

Trifilo MJ, Ying G, Teng C, et al. Chronic wasting disease of deer and elk in transgenic mice: oral transmission and pathobiology. Virology 2007;365(1):136–43

Uehlinger FD, Johnston AC, Bollinger TK, et al. Systematic review of management strategies to control chronic wasting disease in wild deer populations in North America. BMC Vet Res 2016 Aug 22;12(1):173

Vaske JJ. Lessons learned from human dimensions of chronic wasting disease research. Hum Dimens Wildl 2010 May 21;15(3):165–79

Vaske JJ, Lyon KM. CWD prevalence, perceived human health risks, and state influences on deer hunting participation. Risk Anal 2010 Oct 6;31(3):488–96

Vaske JJ, Needham MD, Newman P, et al. Potential for conflict index: hunters’ responses to chronic wasting disease. Wildl Soc Bull 2006;34(1):44–50

Vaske JJ, Needham MD, Stafford NT, et al. Information sources and knowledge about chronic wasting disease in Colorado and Wisconsin. Hum Dimens Wildl 2006;11(3):191–202

Vaske JJ, Timmons NR, Beaman J, et al. Chronic wasting disease in Wisconsin: hunter behavior, perceived risk, and agency trust. Hum Dimens Wildl 2004;9(3):193–209 (published online 2010 Aug 17)

VerCauteren KC, Burke PW, Phillips GE, et al. Elk use of wallows and potential chronic wasting disease transmission. J Wildl Dis 2007 Oct;43(4):784–8

Vercauteren KC, Lavelle MJ, Seward NW, et al. Fence-line contact between wild and farmed cervids in Colorado: potential for disease transmission. J Wildl Manage 2007 Jul 1;71(5):1594–602

VerCauteren KC, Lavelle MJ, Seward NW, et al. Fence-line contact between wild and farmed white-tailed deer in Michigan: potential for disease transmission. J Wildl Manage 2007 Jul 1;71(5):1603–6

Walsh DP, Miller MW. A weighted surveillance approach for detecting chronic wasting disease foci. J Wildl Dis 2010 Jan;46(1):118–35

Walter WD, Baasch DM, Hygnstrom SE, et al. Space use of sympatric deer in a riparian ecosystem in an area where chronic wasting disease is endemic. Wildlife Biol 2011 Jun 1;17(2):191–209

Wasserberg G, Osnas EE, Rolley RE, et al. Host culling as an adaptive management tool for chronic wasting disease in white-tailed deer: a modelling study. J Appl Ecol 2009 Apr;46(2):457–66

Watts JC, Balachandran A, Westaway D. The expanding universe of prion diseases. PLoS Pathog 2006 Mar 31;2(3):e26

Wiggins RC. Prion stability and infectivity in the environment. Neurochem Res 2009 Jan;34(1):158 (published online 2008 May 16)

Wild MA, Hobbs NT, Graham MS, et al. The role of predation in disease control: a comparison of selective and nonselective removal on prion disease dynamics in deer. J Wildl Dis 2011 Jan;47(1):78–93

Williams ES. Chronic wasting disease. Vet Pathol 2005;20

Williams AL, Kreeger TJ, Schumaker BA. Chronic wasting disease model of genetic selection favoring prolonged survival in Rocky Mountain elk (Cervus elaphus). Ecosphere 2014 May 22;5(5)

Williams ES, Miller MW. Transmissible spongiform encephalopathies in non-domestic animals: origin, transmission and risk factors. Rev Sci Tech 2003 Apr;22(1):145–56

Williams ES, Miller MW, Kreeger TJ, et al. Chronic wasting disease of deer and elk: a review with recommendations for management. J Wildl Manage 2002 Jul;66(3):551–63

Wolfe LL, Miller MW, Williams ES. Feasibility of ‘test-and-cull’ for managing chronic wasting disease in urban mule deer. Wildl Soc Bull 2004 Jun;32(2):500–505

Wyckoff AC, Galloway N, Meyerett-Reid C, et al. Prion amplification and hierarchical Bayesian modeling refine detection of prion infection. Sci Rep 2015 Feb 10;5(1):1–8

Xu S, Reuter T, Gilroyed BH, et al. Biodegradation of prions in compost. Environ Sci Technol 2014;48(12):6909–18

Yuan Q, Eckland T, Telling G, et al. Mitigation of prion infectivity and conversion capacity by a simulated natural process—repeated cycles of drying and wetting. PLoS Pathog 2015 Feb 9;11(2):e1004638

Zimmer N, Boxall PC, Adamowicz WL. The impact of chronic wasting disease and its management on hunter perceptions, opinions, and behaviors in Alberta, Canada. J Toxicol Environ Health A 2011 Nov 1;74(22–24):1621–35 

Zimmer NMP, Boxall PC, Adamowicz WL. The impacts of chronic wasting disease and its management on recreational hunters. Can J Ag Econ 2012 Oct 11;60(1):71–92

Non-human interspecies transmission

Aguilar-Calvo P, Garcia C, Espinosa JC, et al. Prion and prion-like diseases in animals. Virus Res 2015 Sep 2;207:82–93

Apostol MI, Wiltzius JJW, Sawaya MR, et al. Atomic structures suggest determinants of transmission barriers in mammalian prion disease. Biochemistry 2011 Feb 16;50(13):2456–63

Bari MAD, Nonno R, Castilla J, et al. Chronic wasting disease in bank voles: characterisation of the shortest incubation time model for prion diseases. PLoS Pathog 2013 Mar 7;9(3):e1003219

Bartz JC, Marsh RF, McKenzie DI, et al. The host range of chronic wasting disease is altered on passage in ferrets. Virology 1998 Nov 25;251(2):297–301

Belay ED, Bartz JC. Prion diseases. Viral infections of humans. Boston, MA: Springer US; 2014 Jun 28:1165–86

Bett C, Fernandez-Borges N, Kurt TD, et al. Structure of the β2-α2 loop and interspecies prion transmission. FASEB J 2012 Jul;26(7):2868–76 (published online Apr 9)

Bian J, Khaychuk V, Angers RC, et al. Prion replication without host adaptation during interspecies transmissions. Proc Natl Acad Sci U S A 2017 Jan 31;114(5):1141–6

Chesebro B. Introduction to the transmissible spongiform encephalopathies or prion diseases. Br Med Bull 2003;66:1–20 

Chianini F, Fernandez-Borges N, Vidal E, et al. Rabbits are not resistant to prion infection. Proc Natl Acad Sci U S A 2012 Mar 27;109(13):5080–5

Comoy EE, Mikol J, Luccantoni-Freire S, et al. Transmission of scrapie prions to primate after an extended silent incubation period. Sci Rep 2015 Jun 30;5(1):1–11

Dagleish MP, Martin S, Steele P, et al. Experimental transmission of bovine spongiform encephalopathy to European red deer (Cervus elaphus elaphus). BMC Vet Res 2008 May 28;4:17

Gould DH, Voss JL, Miller MW, et al. Survey of cattle in northeast Colorado for evidence of chronic wasting disease: geographical and high-risk targeted sample. J Vet Diagn Invest 2003;15(3):274–7

Greenlee JJ, Greenlee MHW. The transmissible spongiform encephalopathies of livestock. ILAR J 2015 May 19;56(1):7–25

Greenlee JJ, Nicholson EM, Smith JD, et al. Susceptibility of cattle to the agent of chronic wasting disease from elk after intracranial inoculation. J Vet Diagn Invest 2012 Nov;24(6):1087–93

Greenlee JJ, Smith JD, Kunkle RA. White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation. Vet Res 2011 Oct 11;42(1):107

Haley NJ, Siepker C, Greenlee JJ, et al. Limited amplification of chronic wasting disease prions in the peripheral tissues of intracerebrally inoculated cattle. J Gen Virol 2016 Jul;97(7):1720–4

Hamir AN, Cutlip RC, Miller JM, et al. Preliminary findings on the experimental transmission of chronic wasting disease agent of mule deer to cattle. J Vet Diagn Invest 2001 Jan;13(1):91–6

Hamir AN, Greenlee JJ, Nicholson EM, et al. Experimental transmission of chronic wasting disease (CWD) from elk and white-tailed deer to fallow deer by intracerebral route: final report. Can J Vet Res 2011 Apr;75(2):152–6

Hamir AN, Kehrli ME, Kunkle RA, et al. Experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle. J Vet Diagn Invest 2011 May;23(3):407–20

Hamir AN, Kunkle RA, Cutlip RC, et al. Experimental transmission of chronic wasting disease agent from mule deer to cattle by the intracerebral route. J Vet Diagn Invest 2005;17(3):276–81 

Hamir AN, Kunkle RA, Cutlip RC, et al. Transmission of chronic wasting disease of mule deer to Suffolk sheep following intracerebral inoculation. J Vet Diagn Invest 2006 Nov;18(6):558–65

Hamir AN, Kunkle RA, Miller JM, et al. Age-related lesions in laboratory-confined raccoons (Procyon lotor) inoculated with the agent of chronic wasting disease of mule deer. J Vet Diagn Invest 2007 Nov;19(6):680–6 

Hamir AN, Kunkle RA, Miller JM, et al. Experimental second passage of chronic wasting disease (CWDmule deer) agent to cattle. J Comp Pathol 2006 Jan;134(1):63–9

Hamir AN, Miller JM, Cutlip RC, et al. Experimental inoculation of scrapie and chronic wasting disease agents in raccoons (Procyon lotor). Vet Rec 2003;153(4):121–3 

Hamir AN, Miller JM, Cutlip RC, et al. Preliminary observations on the experimental transmission of scrapie to elk (Cervus elaphus nelsoni) by intracerebral inoculation. Vet Pathol 2003 Jan;40(1):81–5

Hamir AN, Miller JM, Cutlip RC, et al. Transmission of sheep scrapie to elk (Cervus elaphus nelsoni) by intracerebral inoculation: final outcome of the experiment. J Vet Diagn Invest 2004 Jul;16(4):316–21

Hamir AN, Miller JM, Kunkle RA, et al. Susceptibility of cattle to first-passage intracerebral inoculation with chronic wasting disease agent from white-tailed deer. Vet Pathol 2007 Jul;44(4):487–93

Harrington RD, Baszler TV, O’Rourke KI, et al. A species barrier limits transmission of chronic wasting disease to mink (Mustela vison). J Gen Virol 2008 Apr;89(4):1086–96

Heisey DM, Mickelsen NA, Schneider JR, et al. Chronic wasting disease (CWD) susceptibility of several North American rodents that are sympatric with cervid CWD epidemics. J Virol 2010 Jan;84(1):210–5 (published online 2009 Dec 9)

Jennelle CS, Samuel MD, Nolden CA, et al. Surveillance for transmissible spongiform encephalopathy in scavengers of white-tailed deer carcasses in the chronic wasting disease area of Wisconsin. J Toxicol Environ Health A 2009 Aug 20;72(17–18):1018–24

Johnson CJ, Carlson CM, Morawski AR, et al. Assessing transmissible spongiform encephalopathy species barriers with an in vitro prion protein conversion assay. J Vis Exp 2015 Mar 10;(97):e52522

Kurt TD, Seelig DM, Schneider JR, et al. Alteration of the chronic wasting disease species barrier by in vitro prion amplification. J Virol 2011 Sep;85(17):8528–37

Kurt TD, Telling GC, Zabel MD, et al. Trans-species amplification of PrPCWD and correlation with rigid loop 170N. Virology 2009 Apr 25;387(1):235–43

Kyle LM, John TR, Schaetzl HM, et al. Introducing a rigid loop structure from deer into mouse prion protein increases its propensity for misfolding in vitro. PLoS One 2013 Jun 25;8(6):e66715

Lee YH, Sohn HJ, Kim MJ, et al. Experimental chronic wasting disease in wild type VM mice. J Vet Med Sci 2013;75(8):1107–10

Mathiason CK, Nalls AV, Seelig DM, et al. Susceptibility of domestic cats to chronic wasting disease. J Virol 2013 Jan 24;87(4):1947–56

Morawski AR, Carlson CM, Chang H, et al. In vitro prion protein conversion suggests risk of bighorn sheep (Ovis canadensis) to transmissible spongiform encephalopathies. BMC Vet Res 2013 Aug 9;9(1):157

Perrott MR, Sigurdson CJ, Mason GL, et al. Mucosal transmission and pathogenesis of chronic wasting disease in ferrets. J Gen Virol 2013 Feb;94(2):432–42

Sigurdson CJ, Mathiason CK, Perrott MR, et al. Experimental chronic wasting disease (CWD) in the ferret. J Comp Pathol 2008 May;138(4):189–96

Sigurdson CJ, Nilsson KPR, Hornemann S, et al. A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest 2010 Jul;120(7):2590–9

Sigurdson CJ, Miller MW. Other animal prion diseases. Br Med Bull 2003 Jun;66:199-212

Vidal E, Fernandez-Borges N, Pintado B, et al. Transgenic mouse bioassay: evidence that rabbits are susceptible to a variety of prion isolates. PLoS Pathog 2015 Aug 6;11(8):e1004977

Pathogenesis & transmission

Almberg ES, Cross PC, Johnson CJ, et al. Modeling routes of chronic wasting disease transmission: environmental prion persistence promotes deer population decline and extinction. PLoS One 2011 May 13;6(5):e19896

Aguilar-Calvo P, Garcia C, Espinosa JC, et al. Prion and prion-like diseases in animals. Virus Res 2015 Sep 2;207:82–93

Aguzzi A, Heikenwalder M, Polymenidou M. Insights into prion strains and neurotoxicity. Nat Rev Mol Cell Biol 2007 Jul;8(7):552–61

Aguzzi A, Nuvolone M, Zhu C. The immunobiology of prion diseases. Nat Rev Immunol 2013 Nov 5;13(12):888–902

Aguzzi A, Sigurdson C, Heikenwaelder M. Molecular mechanisms of prion pathogenesis. Annu Rev Pathol Mech Dis 2008;3(1):11–40 (published online 2007 Aug 8)

Angers RC, Browning SR, Seward TS, et al. Prions in skeletal muscles of deer with chronic wasting disease. Science 2006 Feb 24;311(5764):1117

Angers RC, Seward TS, Napier D, et al. Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis 2009 May;15(5):696–703

Bahmanyar S, Williams ES, Johnson FB, et al. Amyloid plaques in spongiform encephalopathy of mule deer. J Comp Pathol 1985 Jan;95(1):1–5

Balachandran A, Harrington NP, Algire J, et al. Experimental oral transmission of chronic wasting disease to red deer (Cervus elaphus elaphus): early detection and late stage distribution of protease-resistant prion protein. Can Vet J 2010 Feb;51(2):169–78

Bartelt-Hunt SL, Bartz JC. Behavior of prions in the environment: implications for prion biology. PLoS Pathog 2013 Feb 7;9(2):e1003113

Bartelt-Hunt SL, Saunders SE, Bartz JC. Soil binds prions and influences their biologic properties. Proteins at Interfaces III State of the Art 2012 Dec 12;20:433–52

Bastian FO. Spiroplasma as a candidate agent for the transmissible spongiform encephalopathies. J Neuropathol Exp Neurol 2005 Oct 1;64(10):833–8

Bastian FO, Dash S, Garry RF. Linking chronic wasting disease to scrapie by comparison of Spiroplasma mirum ribosomal DNA sequences. Exp Mol Pathol 2004 Aug;77(1):49–56

Bastian FO, Fermin CD. Slow virus disease: deciphering conflicting data on the transmissible spongiform encephalopathies (TSE) also called prion diseases. Microsc Res Tech 2005 Nov;68(3–4):239–46

Basu U, Almeida LM, Dudas S, et al. Gene expression alterations in Rocky Mountain elk infected with chronic wasting disease. Prion 2012 Jul 1;6(3):282–301

Beekes M, McBride PA. The spread of prions through the body in naturally acquired transmissible spongiform encephalopathies. FEBS J 2007 Jan 8;274(3):588–605

Belay ED, Bartz JC. Prion diseases. Viral infections of humans. Boston, MA: Springer US; 2014 Jun 28:1165–86

Benestad SL, Mitchell G, Simmons M, et al. First case of chronic wasting disease in Europe in a Norwegian free-ranging reindeer. Vet Res 2016 Sep 15;47:88

Bhupanapadu Sunkesula SR, Luo X, Das D, et al. Iron content of ferritin modulates its uptake by intestinal epithelium: implications for co-transport of prions. Mol Brain 2010 Apr 29;3(1):14

Bian J, Christiansen JR, Moreno JA, et al. Primary structural differences at residue 226 of deer and elk PrP dictate selection of distinct CWD prion strains in gene-targeted mice. Proc Natl Acad Sci 2019 May 30;201903947 

Bourne DC. Chronic wasting disease of cervids. Small Rumin Res 2015 Jul;128:72–8

Conner MM, Miller MW. Movement patterns and spatial epidemiology of a prion disease in mule deer population units. Ecol Appl 2004 Dec;14(6):1870–81

Cullingham CI, Nakada SM, Merrill EH, et al. Multiscale population genetic analysis of mule deer (Odocoileus hemionus hemionus) in western Canada sheds new light on the spread of chronic wasting disease. Can J Zoo 2011 Jan 28;89(2):134–47

Daus ML, Breyer J, Wagenfuehr K, et al. Presence and seeding activity of pathological prion protein (PrPTSE) in skeletal muscles of white-tailed deer infected with chronic wasting disease. PLoS One 2011 Apr 1;6(4):e18345 

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Chakraborty C, Nandi S, Jana S. Prion disease: a deadly disease for protein misfolding. Curr Pharm Biotechnol 2005 Apr;6(2):167–77

Chesebro B. Introduction to the transmissible or prion diseases. Br Med Bull 2003;66:1–20

Collinge J. Prion strain mutation and selection. Science 2010 May 28;328(5982):1111–2

Collinge J. The risk of prion zoonoses. Science 2012 Jan 27;335(6067):411–3

Cortez LM, Sim VL. Implications of prion polymorphisms. Prion 2013 Jul-Aug;7(4):276–9

Crowell J, Hughson A, Caughey B, et al. Host determinants of prion strain diversity independent of prion protein genotype. J Virol 2015 Oct;89(20):10427–41

Daus ML, Beekes M. Chronic wasting disease: fingerprinting the culprit in risk assessments. Prion 2012 Jan-Mar;6(1):17–22

Daus ML, Breyer J, Wagenfuehr K, et al. Presence and seeding activity of pathological prion protein (PrPTSE) in skeletal muscles of white-tailed deer infected with chronic wasting disease. PLoS One 2011 Apr 1;6(4):e18345

Di Bari MA, Nonno R, Castilla J, et al. Chronic wasting disease in bank voles: characterisation of the shortest incubation time model for prion diseases. PLoS Pathog 2013 Mar;9(3):e1003219

Duque Velasquez C, Kim C, Herbst A, et al. Deer prion proteins modulate the emergence and adaptation of chronic wasting disease strains. J Virol 2015 Dec;89(24):12362–73

Fernandez-Borges N, Di Bari MA, Erana H, et al. Cofactors influence the biological properties of infectious recombinant prions. Acta Neuropathol 2018 Feb 1;135(2):179–99

Fernandez-Borges N, Erana H, Venegas V, et al. Animal models for prion-like diseases. Virus Res 2015 Sep 2;207:5–24

Gavier-Widen D, Stack MJ, Baron T, et al. Diagnosis of transmissible spongiform encephalopathies in animals: a review. J Vet Diagn Invest 2005 Nov;17(6):509–27

Gough KC, Maddison BC. Prion transmission. Prion 2010;4(4):275–82

Gough KC, Rees HC, Ives SE, et al. Methods for differentiating prion types in food-producing animals. Biology (Basel) 2015 Nov 13;4(4):785–813

Green KM, Browning SR, Seward TS, et al. The elk PRNP codon 132 polymorphism controls cervid and scrapie prion propagation. J Gen Virol 2008 Feb;89(Pt 2):598–608

Green KM, Castilla J, Seward TS, et al. Accelerated high fidelity prion amplification within and across prion species barriers. PLoS Pathog 2008 Aug 29;4(8):e1000139

Haley NJ, Carver S, Hoon-Hanks LL, et al. Detection of chronic wasting disease in the lymph nodes of free-ranging cervids by real-time quaking-induced conversion. J Clin Microbiol 2014 Sep;52(9):3237–43

Haley NJ, Hoover EA. Chronic wasting disease of cervids: current knowledge and future perspectives. Annu Rev Anim Biosci 2015;3:305–25

Hannaoui S, Schatzl HM, Gilch S. Chronic wasting disease: emerging prions and their potential risk. PLoS Pathog 2017 Nov 2;13(11):e1006619

Harrathi C, Fernandez-Borges N, Erana H, et al. Insights into the bidirectional properties of the sheep-deer prion transmission barrier. Mol Neurobiol 2019 Aug;56(8):5287–5303 (published online 2018 Dec 27)

Hedlin P, Taschuk R, Potter A, et al. Detection and control of prion diseases in food animals. Int Sch Res Notices 2012

Herbst A, Duque Velasquez C, Triscott E, et al. Chronic wasting disease prion strain emergence and host range expansion. Emerg Infect Dis 2017 Sep;23(9):1598–1600

Houston F, Andreoletti O. Animal prion diseases: the risks to human health. Brain Pathol 2019;29(2):248–62

Imran M, Mahmood S. An overview of animal prion diseases. Virol J 2011 Nov;8(1):493

Johnson CJ, Aiken JM, McKenzie D, et al. Highly efficient amplification of chronic wasting disease agent by protein misfolding cyclic amplification with beads (PMCAb). PLoS One 2012 Apr 13;7(4):e35383

Johnson C, Johnson J, Vanderloo JP, et al. Prion protein polymorphisms in white-tailed deer influence susceptibility to chronic wasting disease. J Gen Virol 2006 Jul 1;87(7):2109–14

Johnson CJ, Pedersen JA, Chappell RJ, et al. Oral transmissibility of prion disease Is enhanced by binding to soil particles. PLoS Pathog 2007 Jul;3(7):e93

Johnson RT. Prion diseases. Lancet Neurol 2005 Oct;4(10):635–42

Kurt TD, Bett C, Fernandez-Borges N, et al. Prion transmission prevented by modifying the b2-a2 loop structure of host PrPC. J Neurosci 2014 Jan 15;34(3):1022–7

LaFauci G, Carp RI, Meeker HC, et al. Passage of chronic wasting disease prion into transgenic mice expressing Rocky Mountain elk (Cervus elaphus nelsoni). PrPC. J Gen Virol 2006 Dec;87(Pt 12):3773–80

Lee YH, Sohn HJ, Kim MJ, et al. Strain characterization of the Korean CWD cases in 2001 and 2004. J Vet Med Sci 2013 Jan 31;75(1):95–8

Maddison BC, Owen JP, Bishop K, et al. The interaction of ruminant PrPsc with soils is influenced by prion source and soil type. Environ Sci Technol 2010 Oct 22;44(22):8503–8

Meyerett C, Michel B, Pulford B, et al. In vitro strain adaptation of CWD prions by serial protein misfolding cyclic amplification. Virology 2008 Dec 20;382(2):267–76

Meyerett-Reid C, Wyckoff AC, Spraker T, et al. De novo generation of a unique cervid prion strain using protein misfolding cyclic amplification. mSphere 2017 Jan 25;2(1)

Michel B, Ferguson A, Johnson T, et al. Genetic depletion of complement receptors CD21/35 prevents terminal prion disease in a mouse model of chronic wasting disease. J Immunol 2012 Nov 1;189(9):4520–7

Monello RJ, Galloway NL, Powers JG, et al. Pathogen-mediated selection in free-ranging elk populations infected by chronic wasting disease. Proc Natl Acad Sci U S A 2017 Nov 14;114(46):12208–12

Moreno JA, Telling GC. Insights into mechanisms of transmission and pathogenesis from transgenic mouse models of prion diseases. Methods Mol Biol 2017;1658:219–52

Novakofski J, Brewer MS, Mateus-Pinilla N, et al. Prion biology relevant to bovine spongiform encephalopathy. J Anim Sci 2005 Jun;83(6):1455–76

Orru CD, Groveman BR, Raymond LD, et al. Bank vole prion protein as an apparently universal substrate for RT-QuIC-based detection and discrimination of prion strains. PLoS Pathog 2015 Jun 18;11(6):e1004983

Otero A, Duque Velasquez C, Johnson C, et al. Prion protein polymorphisms associated with reduced CWD susceptibility limit peripheral PrPCWD deposition in orally infected white-tailed deer. BMC Vet Res 2019 Feb 4;15(1):50

Perrott MR, Sigurdson CJ, Mason GL, et al. Evidence for distinct chronic wasting disease (CWD) strains in experimental CWD in ferrets. J Gen Virol 2012 Jan;93(Pt 1):212–21

Race B, Williams K, Chesebro B. Transmission studies of chronic wasting disease to transgenic mice overexpressing human prion protein using the RT-QuIC assay. Vet Res 2019 Jan 22;50(1):6

Race B, Williams K, Orru CD, et al. Lack of transmission of chronic wasting disease to cynomolgus macaques. J Virol 2018 Jun 29;92(14)

Race RE, Raines A, Baron TGM, et al. Comparison of abnormal prion protein glycoform patterns from transmissible spongiform encephalopathy agent-infected deer, elk, sheep, and cattle. J Virol 2002 Dec;76(23):12365–8

Raymond GJ, Raymond LD, Meade-White KD, et al. Transmission and adaptation of chronic wasting disease to hamsters and transgenic mice: evidence for strains. J Virol 2007 Apr;81(8):4305–14 (published online 2007 Feb 7)

Sandberg MK, Al-Doujaily H, Sigurdson CJ, et al. Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. J Gen Virol 2010 Oct;91(Pt 10):2651–7

Saunders SE, Bartelt-Hunt SL, Bartz JC. Occurrence, transmission, and zoonotic potential of chronic wasting disease. Emerg Infect Dis 2012 Mar;18(3):369–76

Saunders SE, Bartz JC, Bartelt-Hunt SL. Influence of prion strain on prion protein adsorption to soil in a competitive matrix. Environ Sci Technol 2009 Jul 15;43(14):5242–8

Saunders SE, Bartelt-Hunt SL, Bartz JC. Prions in the environment. Prion 2008 Nov 25;2(4):162–9

Saunders SE, Bartz JC, Telling GC, et al. Environmentally-relevant forms of the prion protein. Environ Sci Technol 2008 Jul;42(17):6573–9

Schutt CR, Bartz JC. Prion interference with multiple prion isolates. Prion 2008;2(2):61–3

Seelig DM, Nalls AV, Flasik M, et al. Lesion profiling and subcellular prion localization of cervid chronic wasting disease in domestic cats. Vet Pathol 2015 Jan;52(1):107–19

Shikiya RA, Bartz JC. In vitro generation of high-titer prions. J Virol 2011 Dec;85(24):13439–42 (published online Nov 21)

Sigurdson CJ, Nilsson KPR, Hornemann S, et al. A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest 2010 Jul;120(7):2590–9

Sigurdson CJ, Bartz JC, Glatzel M. Cellular and molecular mechanisms of prion disease. Annu Rev Pathol 2019 Jan 24;14:497–516

Sigurdson CJ, Manco G, Schwarz P, et al. Strain fidelity of chronic wasting disease upon murine adaptation. J Virol 2006 Dec;80(24):12303–11

Sigurdson CJ, Nilsson KPR, Hornemann S, et al. Prion strain discrimination using luminescent conjugated polymers. Nat Methods 2007 Dec;4(12):1023–30

Stewart P, Campbell L, Skogtvedt S, et al. Genetic predictions of prion disease susceptibility in carnivore species based on variability of the prion gene coding region. PLoS One 2012 Dec 7;7(12):e50623

Telling GC. Nucleic acid-free mutation of prion strains. Prion 2010 Oct;4(4):252–5

Telling GC. Transgenic mouse models and prion strains. In Prion Proteins, edited by Jorg Tatzelt, 79–99. Topics in Current Chemistry. Berlin, Heidelberg: Springer Berlin Heidelberg, 2011

VerCauteren KC, Pilon JL, Nash PB, et al. Prion remains infectious after passage through digestive system of American crows (Corvus brachyrhynchos). PLoS One 2012 Oct 17;7(10):e45774

Vickery CM, Lockey R, Holder TM, et al. Assessing the susceptibility of transgenic mice overexpressing deer prion protein to bovine spongiform encephalopathy. J Virol 2014 Feb;88(3):1830–3

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Watts JC, Giles K, Patel S, et al. Evidence that bank vole PrP is a universal acceptor for prions. PLoS Pathog 2014 Apr 3;10(4):e1003990

Wisniewski T, Goni F. Immunomodulation for prion and prion-related diseases. Expert Rev Vaccines 2010 Dec;9(12):1441–52

Wrathall AE, Holyoak GR, Parsonson IM, et al. Risks of transmitting ruminant spongiform encephalopathies (prion diseases) by semen and embryo transfer techniques. Theriogenology 2008 Sep 15;70(5):725–45

Yuan Q, Telling G, Bartelt-Hunt SL, et al. Dehydration of prions on environmentally relevant surfaces protects them from inactivation by freezing and thawing. J Virol 2018 Mar 28;92(8)

Therapies & vaccines

Abdelaziz DH, Thapa S, Abdulrahman B, et al. Immunization of cervidized transgenic mice with multimeric deer prion protein induces self-antibodies that antagonize chronic wasting disease infectivity in vitro. Sci Rep 2017 May;7(1):10538

Abdelaziz DH, Thapa S, Brandon J, et al. Recombinant prion protein vaccination of transgenic elk PrP mice and reindeer overcomes self-tolerance and protects mice against chronic wasting disease. J Biol Chem 2018 Dec 21;293(51):19812–22

Aguzzi A, Heikenwalder M, Miele G. Progress and problems in the biology, diagnostics, and therapeutics of prion diseases. J Clin Invest 2004 Jul;114(2):153–60 

Berry DB, Lu D, Geva M, et al. Drug resistance confounding prion therapeutics. Proc Natl Acad Sci U S A 2013 Sep 11;110(44):4160–9

Cashman NR. Propagated protein misfolding: new opportunities for therapeutics, new public health risk. Can Commun Dis Rep 2015 Aug 6;41(8):196–9

Caughey B, Orru CD, Groveman BR, et al. Chapter seventeen - amplified detection of prions and other amyloids by RT-QuIC in diagnostics and the evaluation of therapeutics and disinfectants. Progress in Molecular Biology and Translational Science 2017;150:375–88

Chesney AR, Booth CJ, Lietz CB, et al. Peroxymonosulfate rapidly inactivates the disease-associated prion protein. Environ Sci Technol 2016 Jun;50(13):7095–105

Goni F, Mathiason CK, Yim L, et al. Mucosal immunization with an attenuated Salmonella vaccine partially protects white-tailed deer from chronic wasting disease. Vaccine 2015 Jan 29 33(5):726–33

Li L, Napper S, Cashman NR. Immunotherapy for prion diseases: opportunities and obstacles. Immunotherapy 2010 Mar;2(2):269–82

Michel B, Ferguson A, Johnson T, et al. Complement protein C3 exacerbates prion disease in a mouse model of chronic wasting disease. Int Immunol 2013 Dec;25(12):697–702

Pilon J, Loiacono C, Okeson D, et al. Anti-prion activity generated by a novel vaccine formulation. Neurosci Lett 2007 Dec 18;429(2):161–64

Pilon JL, Rhyan JC, Wolfe LL, et al. Immunization with a synthetic peptide vaccine fails to protect mule deer (Odocoileus hemionus) from chronic wasting disease. J Wildl Dis 2013 Jul;49(3):694–98

Taschuk R, Marciniuk K, Maattanen P, et al. Safety, specificity and immunogenicity of a PrPSc-specific prion vaccine based on the YYR disease specific epitope. Prion 8(1) 2014 Jan-Feb;8(1):51–59

Taschuk R, Scruten E, Woodbury M, et al. Induction of PrPSc-specific systemic and mucosal immune responses in white-tailed deer with an oral vaccine for chronic wasting disease. Prion 2017 Oct 2;11(5):368–80

Wisniewski T, Goni F. Vaccination strategies. Handb Clin Neurol 2018;153:419–30

Wolfe LL, Kocisko DA, Caughey B, et al. Assessment of prospective preventive therapies for chronic wasting disease in mule deer. J Wildl Dis 2012 Apr;48(2):530–3

Wood ME, Griebel P, Huizenga ML, et al. Accelerated onset of chronic wasting disease in elk (Cervus canadensis) vaccinated with a PrPSc-specific vaccine and housed in a prion contaminated environment. Vaccine 2018 Nov 29;36(50):7737–43

Recent News

Jun 10, 2020

News Scan for Jun 10, 2020

CWD detection in Wisconsin
H5N8 avian flu in Hungarian poultry
Mar 16, 2020

CWD Scan for Mar 16, 2020

CWD spreads in Minnesota
Taxpayers pay for CWD testing
Pennsylvania CWD plan endorsed
Feb 12, 2020

CWD Scan for Feb 12, 2020

CWD expansion in Iowa
South Dakota CWD
In-state Montana testing
Feb 11, 2020

CWD Scan for Feb 11, 2020

More CWD in Montana
New Tennessee CWD counties
Feb 04, 2020

News Scan for Feb 04, 2020

Global flu levels high
CWD in captive Montana elk
CWD risk in non-cervid animals
Jan 23, 2020
Two bucks in snow

Minnesota finds CWD in wild deer in just 1 area; new Wisconsin county affected

The 27 detections from the 2019 hunting season were confined to southeastern Minnesota.

Jan 17, 2020
Buck and doe on deer farm

Study raises possibility of sexual spread of CWD in deer

"We know almost nothing about sexual transmission and prion diseases," one expert says.

Jan 13, 2020

News Scan for Jan 13, 2020

New DRC Ebola cases
More CWD in Minnesota
Mandatory vaccination in Europe
Avian flu in 3 nations
Jan 09, 2020

News Scan for Jan 09, 2020

CWD on Wisconsin deer farms
Minnesota CWD deer ban defier
Autism caregivers' vaccine views
Dec 26, 2019
hungry doe

CWD detection prompts Minnesota ban on farmed deer movement

Earlier sites of CWD prion transmission in Minnesota have links to cervid farms.

Online Resources

Recent Literature

Kramm C, Gomez-Gutierrez R, Soto C, et al. In vitro detection of chronic wasting disease (CWD) prions in semen and reproductive tissues of white tailed deer bucks (Odocoileus virginianus). PLOS One 2019 Dec 30;14(12):e0226560