Volume 31, Number 2—February 2025
Research
Prions in Muscles of Cervids with Chronic Wasting Disease, Norway
Table 1
Information for animals included in a study of prions in muscles of cervids with CWD, Norway*
Species | ID number | Code | CWD genotype | Animal sex | Area detected | Diagnostic status† |
|
---|---|---|---|---|---|---|---|
Brain | Lymph node | ||||||
Rangifer tarandus |
17-CD20830 | Reindeer A | A/A‡ | M | Nordfjella | Positive | Positive |
18-CD3207 |
Reindeer B |
A/B‡ |
F |
Nordfjella |
Negative |
Positive |
|
Alces alces |
17-CD11399 | Moose A | KK109 | F | Lierne | Positive | Negative |
21-CD41 | Moose B | KK109 | M | Telemark | Positive | Negative | |
19-CD24854 | Moose C | QQ109 | F | Sigdal | Positive | Negative | |
22-CD29 |
Moose D |
QQ109 |
F |
Tynset |
Positive |
Negative |
|
Cervus elaphus | 17-CD14051 | Red deer | QQ226 | F | Gjemnes | Positive | Negative |
*Animals detected through Norway’s CWD surveillance program. CWD, chronic wasting disease; ID, identification. †PrPSc detected by traditional diagnostic methods: ELISA, Western blot, and immunohistochemistry. ‡Nomenclature according to Güere et al. (26).
References
- Otero A, Velásquez CD, Aiken J, McKenzie D. Chronic wasting disease: a cervid prion infection looming to spillover. Vet Res. 2021;52:115. DOIPubMedGoogle Scholar
- Sejvar JJ, Schonberger LB, Belay ED. Transmissible spongiform encephalopathies. J Am Vet Med Assoc. 2008;233:1705–12. DOIPubMedGoogle Scholar
- Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science. 1982;216:136–44. DOIPubMedGoogle Scholar
- US Geological Survey National Wildlife Health Center. Distribution of chronic wasting disease in North America [cited 2024 Jun 20] https://www.usgs.gov/centers/nwhc/science/expanding-distribution-chronic-wasting-disease
- Tranulis MA, Gavier-Widén D, Våge J, Nöremark M, Korpenfelt S-L, Hautaniemi M, et al. Chronic wasting disease in Europe: new strains on the horizon. Acta Vet Scand. 2021;63:48. DOIPubMedGoogle Scholar
- Sun JL, Kim S, Crowell J, Webster BK, Raisley EK, Lowe DC, et al. Novel prion strain as cause of chronic wasting disease in a moose, Finland. Emerg Infect Dis. 2023;29:323–32. DOIPubMedGoogle Scholar
- Sola D, Tran L, Våge J, Madslien K, Vuong TT, Korpenfelt SL, et al. Heterogeneity of pathological prion protein accumulation in the brain of moose (Alces alces) from Norway, Sweden and Finland with chronic wasting disease. Vet Res. 2023;54:74. DOIPubMedGoogle Scholar
- Bian J, Kim S, Kane SJ, Crowell J, Sun JL, Christiansen J, et al. Adaptive selection of a prion strain conformer corresponding to established North American CWD during propagation of novel emergent Norwegian strains in mice expressing elk or deer prion protein. PLoS Pathog. 2021;17:
e1009748 . DOIPubMedGoogle Scholar - Nonno R, Di Bari MA, Pirisinu L, D’Agostino C, Vanni I, Chiappini B, et al. Studies in bank voles reveal strain differences between chronic wasting disease prions from Norway and North America. Proc Natl Acad Sci U S A. 2020;117:31417–26. DOIPubMedGoogle Scholar
- Hopp P, Rolandsen CM, Korpenfelt SL, Våge J, Sörén K, Solberg EJ, et al. Sporadic cases of chronic wasting disease in old moose - an epidemiological study. J Gen Virol. 2024;105:105. DOIPubMedGoogle Scholar
- Di Bari MA, Nonno R, Castilla J, D’Agostino C, Pirisinu L, Riccardi G, et al. Chronic wasting disease in bank voles: characterisation of the shortest incubation time model for prion diseases. PLoS Pathog. 2013;9:
e1003219 . DOIPubMedGoogle Scholar - Pirisinu L, Tran L, Chiappini B, Vanni I, Di Bari MA, Vaccari G, et al. Novel type of chronic wasting disease detected in moose (Alces alces), Norway. Emerg Infect Dis. 2018;24:2210–8. DOIPubMedGoogle Scholar
- Koutsoumanis K, Allende A, Alvarez-Ordoňez A, Bolton D, Bover-Cid S, Chemaly M, et al.; EFSA Panel on Biological Hazards (BIOHAZ). Update on chronic wasting disease (CWD) III. EFSA J. 2019;17:
e05863 .PubMedGoogle Scholar - Vikøren T, Våge J, Madslien KI, Røed KH, Rolandsen CM, Tran L, et al. First detection of chronic wasting disease in a wild red deer (Cervus elaphus) in Europe. J Wildl Dis. 2019;55:970–2. DOIPubMedGoogle Scholar
- Pritzkow S, Gorski D, Ramirez F, Telling GC, Benestad SL, Soto C. North American and Norwegian chronic wasting disease prions exhibit different potential for interspecies transmission and zoonotic risk. J Infect Dis. 2022;225:542–51. DOIPubMedGoogle Scholar
- Harpaz E, Vuong TT, Tran L, Tranulis MA, Benestad SL, Ersdal C. Inter- and intra-species conversion efficacies of Norwegian prion isolates estimated by serial protein misfolding cyclic amplification. Vet Res. 2023;54:84. DOIPubMedGoogle Scholar
- Marín-Moreno A, Benestad SL, Barrio T, Pirisinu L, Espinosa JC, Tran L, et al. Classical BSE dismissed as the cause of CWD in Norwegian red deer despite strain similarities between both prion agents. Vet Res. 2024;55:62. DOIPubMedGoogle Scholar
- Race BL, Meade-White KD, Ward A, Jewell J, Miller MW, Williams ES, et al. Levels of abnormal prion protein in deer and elk with chronic wasting disease. Emerg Infect Dis. 2007;13:824–30. DOIPubMedGoogle Scholar
- Mathiason CK, Hays SA, Powers J, Hayes-Klug J, Langenberg J, Dahmes SJ, et al. Infectious prions in pre-clinical deer and transmission of chronic wasting disease solely by environmental exposure. PLoS One. 2009;4:
e5916 . DOIPubMedGoogle Scholar - John TR, Schätzl 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;7:253–8. DOIPubMedGoogle Scholar
- Henderson DM, Manca M, Haley NJ, Denkers ND, Nalls AV, Mathiason CK, et al. Rapid antemortem detection of CWD prions in deer saliva. PLoS One. 2013;8:
e74377 . DOIPubMedGoogle Scholar - Koutsoumanis K, Allende A, Alvarez-Ordoñez A, Bolton D, Bover-Cid S, Chemaly M, et al.; EFSA Panel on Biological Hazards (BIOHAZ). Monitoring of chronic wasting disease (CWD) (IV). EFSA J. 2023;21:
e07936 .PubMedGoogle Scholar - Lambert ZJ, Greenlee JJ, Cassmann ED, West Greenlee MH. Differential accumulation of misfolded prion strains in natural hosts of prion diseases. Viruses. 2021;13:2453. DOIPubMedGoogle Scholar
- Simmons SM, Bartz JC. Strain-specific targeting and destruction of cells by prions. Biology (Basel). 2024;13:57. DOIPubMedGoogle Scholar
- Güere ME, Våge J, Tharaldsen H, Benestad SL, Vikøren T, Madslien K, et al. Chronic wasting disease associated with prion protein gene (PRNP) variation in Norwegian wild reindeer (Rangifer tarandus). Prion. 2020;14:1–10. DOIPubMedGoogle Scholar
- Beekes M, McBride PA. The spread of prions through the body in naturally acquired transmissible spongiform encephalopathies. FEBS J. 2007;274:588–605. DOIPubMedGoogle Scholar
- Fox KA, Jewell JE, Williams ES, Miller MW. Patterns of PrPCWD accumulation during the course of chronic wasting disease infection in orally inoculated mule deer (Odocoileus hemionus). J Gen Virol. 2006;87:3451–61. DOIPubMedGoogle Scholar
- Hoover CE, Davenport KA, Henderson DM, Denkers ND, Mathiason CK, Soto C, et al. Pathways of prion spread during early chronic wasting disease in deer. J Virol. 2017;91:e00077–17. DOIPubMedGoogle Scholar
- Sigurdson CJ, Williams ES, Miller MW, Spraker TR, O’Rourke KI, Hoover EA. Oral transmission and early lymphoid tropism of chronic wasting disease PrPres in mule deer fawns (Odocoileus hemionus). J Gen Virol. 1999;80:2757–64. DOIPubMedGoogle Scholar
- Benavente R, Reed JH, Lockwood M, Morales R. PMCA screening of retropharyngeal lymph nodes in white-tailed deer and comparisons with ELISA and IHC. Sci Rep. 2023;13:20171. DOIPubMedGoogle Scholar
- Haley NJ, Mathiason CK, Carver S, Zabel M, Telling GC, Hoover EA. Detection of chronic wasting disease prions in salivary, urinary, and intestinal tissues of deer: potential mechanisms of prion shedding and transmission. J Virol. 2011;85:6309–18. DOIPubMedGoogle Scholar
- Ferreira NC, Charco JM, Plagenz J, Orru CD, Denkers ND, Metrick MA II, et al. Detection of chronic wasting disease in mule and white-tailed deer by RT-QuIC analysis of outer ear. Sci Rep. 2021;11:7702. DOIPubMedGoogle Scholar
- Cooper SK, Hoover CE, Henderson DM, Haley NJ, Mathiason CK, Hoover EA. Detection of CWD in cervids by RT-QuIC assay of third eyelids. PLoS One. 2019;14:
e0221654 . DOIPubMedGoogle Scholar - Angers RC, Browning SR, Seward TS, Sigurdson CJ, Miller MW, Hoover EA, et al. Prions in skeletal muscles of deer with chronic wasting disease. Science. 2006;311:1117. DOIPubMedGoogle Scholar
- Henderson DM, Denkers ND, Hoover CE, McNulty EE, Cooper SK, Bracchi LA, et al. Progression of chronic wasting disease in white-tailed deer analyzed by serial biopsy RT-QuIC and immunohistochemistry. PLoS One. 2020;15:
e0228327 . DOIPubMedGoogle Scholar - Kramm C, Soto P, Nichols TA, Morales R. Chronic wasting disease (CWD) prion detection in blood from pre-symptomatic white-tailed deer harboring PRNP polymorphic variants. Sci Rep. 2020;10:19763. DOIPubMedGoogle Scholar
- Henderson DM, Tennant JM, Haley NJ, Denkers ND, Mathiason CK, Hoover EA. Detection of chronic wasting disease prion seeding activity in deer and elk feces by real-time quaking-induced conversion. J Gen Virol. 2017;98:1953–62. DOIPubMedGoogle Scholar
- Chianini F, Cosseddu GM, Steele P, Hamilton S, Hawthorn J, Síso S, et al. Correlation between infectivity and disease associated prion protein in the nervous system and selected edible tissues of naturally affected scrapie sheep. PLoS One. 2015;10:
e0122785 . DOIPubMedGoogle Scholar - Andréoletti O, Orge L, Benestad SL, Beringue V, Litaise C, Simon S, et al. Atypical/Nor98 scrapie infectivity in sheep peripheral tissues. PLoS Pathog. 2011;7:
e1001285 . DOIPubMedGoogle Scholar - Kumagai S, Daikai T, Onodera T. Bovine spongiform encephalopathy—a review from the perspective of food safety. Food Saf (Tokyo). 2019;7:21–47. DOIPubMedGoogle Scholar
- Peden AH, Ritchie DL, Head MW, Ironside JW. Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease. Am J Pathol. 2006;168:927–35. DOIPubMedGoogle Scholar
- Thomzig A, Cardone F, Krüger D, Pocchiari M, Brown P, Beekes M. Pathological prion protein in muscles of hamsters and mice infected with rodent-adapted BSE or vCJD. J Gen Virol. 2006;87:251–4. DOIPubMedGoogle Scholar
- Bosque PJ, Ryou C, Telling G, Peretz D, Legname G, DeArmond SJ, et al. Prions in skeletal muscle. Proc Natl Acad Sci U S A. 2002;99:3812–7. DOIPubMedGoogle Scholar
- Daus ML, Breyer J, Wagenfuehr K, Wemheuer WM, Thomzig A, Schulz-Schaeffer WJ, et al. Presence and seeding activity of pathological prion protein (PrP(TSE)) in skeletal muscles of white-tailed deer infected with chronic wasting disease. PLoS One. 2011;6:
e18345 . DOIPubMedGoogle Scholar - Jewell JE, Brown J, Kreeger T, Williams ES. 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;87:3443–50. DOIPubMedGoogle Scholar
- Li M, Schwabenlander MD, Rowden GR, Schefers JM, Jennelle CS, Carstensen M, et al. RT-QuIC detection of CWD prion seeding activity in white-tailed deer muscle tissues. Sci Rep. 2021;11:16759. DOIPubMedGoogle Scholar
- Tranulis MA, Tryland M. The zoonotic potential of chronic wasting disease—a review. Foods. 2023;12:824. DOIPubMedGoogle Scholar
- Wadsworth JDF, Joiner S, Linehan JM, Jack K, Al-Doujaily H, Costa H, et al. Humanized transgenic mice are resistant to chronic wasting disease prions from Norwegian reindeer and moose. J Infect Dis. 2022;226:933–7. DOIPubMedGoogle Scholar
1These first authors contributed equally to this article.
2These authors are co–senior authors.
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