Volume 29, Number 2—February 2023
Research
Novel Prion Strain as Cause of Chronic Wasting Disease in a Moose, Finland
Table 2
Inoculum | GtQ | GtE |
---|---|---|
GtQ (M-F1) brain 1 | 228 +3 (10/10) | >469 +17 (4/7) |
GtQ (M-F1) brain 2 | 223 +4 (9/9) | >507 (0/5) |
GtQ (M-NO1) | 281 +24 (5/5) | |
GtQ (M-NO2) | 541 + 5 (5/7) |
*Time to disease onset (incubation time) is expressed as the mean number of days after which inoculated mice first showed ultimately progressive signs of neurologic disease +SD. Parentheses include the numbers of diseased mice/number of inoculated mice. Incubation times of Norway moose CWD prions have been described previously (27). CWD, chronic wasting disease; GtE, CWD-susceptible gene-targeted mice in which the prion protein coding sequence was replaced with one encoding glutamate at codon 226; GtQ, CWD-susceptible gene-targeted mice in which the prion protein coding sequence was replaced with one encoding glutamine at codon 226; M-F1, Finland moose 1; M-NO1, Norway moose 1; M-NO2, Norway moose 2.
References
- Riek R, Hornemann S, Wider G, Billeter M, Glockshuber R, Wüthrich K. NMR structure of the mouse prion protein domain PrP(121-231). Nature. 1996;382:180–2. DOIPubMedGoogle Scholar
- Kraus A, Hoyt F, Schwartz CL, Hansen B, Artikis E, Hughson AG, et al. High-resolution structure and strain comparison of infectious mammalian prions. Mol Cell. 2021;81:4540–4551.e6. DOIPubMedGoogle Scholar
- Kang HE, Bian J, Kane SJ, Kim S, Selwyn V, Crowell J, et al. Incomplete glycosylation during prion infection unmasks a prion protein epitope that facilitates prion detection and strain discrimination. J Biol Chem. 2020;295:10420–33. DOIPubMedGoogle Scholar
- Basler K, Oesch B, Scott M, Westaway D, Wälchli M, Groth DF, et al. Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell. 1986;46:417–28. DOIPubMedGoogle Scholar
- Hoyt F, Standke HG, Artikis E, Schwartz CL, Hansen B, Li K, et al. Cryo-EM structure of anchorless RML prion reveals variations in shared motifs between distinct strains. Nat Commun. 2022;13:4005. DOIPubMedGoogle Scholar
- Manka SW, Zhang W, Wenborn A, Betts J, Joiner S, Saibil HR, et al. 2.7 Å cryo-EM structure of ex vivo RML prion fibrils. Nat Commun. 2022;13:4004. DOIPubMedGoogle Scholar
- Telling GC. The shape of things to come: structural insights into how prion proteins encipher heritable information. Nat Commun. 2022;13:4003. DOIPubMedGoogle Scholar
- Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science. 1982;216:136–44. DOIPubMedGoogle Scholar
- Büeler H, Aguzzi A, Sailer A, Greiner R-A, Autenried P, Aguet M, et al. Mice devoid of PrP are resistant to scrapie. Cell. 1993;73:1339–47. DOIPubMedGoogle Scholar
- Castilla J, Saá P, Hetz C, Soto C. In vitro generation of infectious scrapie prions. Cell. 2005;121:195–206. DOIPubMedGoogle Scholar
- Legname G, Baskakov IV, Nguyen HO, Riesner D, Cohen FE, DeArmond SJ, et al. Synthetic mammalian prions. Science. 2004;305:673–6. DOIPubMedGoogle Scholar
- Bartz JC. Prion strain diversity. Cold Spring Harb Perspect Med. 2016;6:
a024349 . DOIPubMedGoogle Scholar - Will RG, Cousens SN, Farrington CP, Smith PG, Knight RSG, Ironside JW. Deaths from variant Creutzfeldt-Jakob disease. Lancet. 1999;353:979. DOIPubMedGoogle Scholar
- Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, et al. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science. 1996;274:2079–82. DOIPubMedGoogle Scholar
- Bessen RA, Marsh RF. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J Virol. 1994;68:7859–68. DOIPubMedGoogle Scholar
- Benestad SL, Telling GC. Chronic wasting disease: an evolving prion disease of cervids. Handb Clin Neurol. 2018;153:135–51. DOIPubMedGoogle Scholar
- Williams ES, Young S. Chronic wasting disease of captive mule deer: a spongiform encephalopathy. J Wildl Dis. 1980;16:89–98. DOIPubMedGoogle Scholar
- US Geological Survey. Expanding distribution of chronic wasting disease. 2022 Sep 26 [cited 2022 Dec 22]. https://www.usgs.gov/centers/nwhc/science/expanding-distribution-chronic-wasting-disease
- Sohn HJ, Kim JH, Choi KS, Nah JJ, Joo YS, Jean YH, et al. A case of chronic wasting disease in an elk imported to Korea from Canada. J Vet Med Sci. 2002;64:855–8. DOIPubMedGoogle Scholar
- Kim TY, Shon HJ, Joo YS, Mun UK, Kang KS, Lee YS. Additional cases of Chronic Wasting Disease in imported deer in Korea. J Vet Med Sci. 2005;67:753–9. DOIPubMedGoogle Scholar
- Lee YH, Sohn HJ, Kim MJ, Kim HJ, Park KJ, Lee WY, et al. Experimental chronic wasting disease in wild type VM mice. J Vet Med Sci. 2013;75:1107–10. DOIPubMedGoogle Scholar
- Benestad SL, Mitchell G, Simmons M, Ytrehus B, Vikøren T. First case of chronic wasting disease in Europe in a Norwegian free-ranging reindeer. Vet Res (Faisalabad). 2016;47:88. DOIPubMedGoogle Scholar
- Tranulis MA, Gavier-Widén D, Våge J, Nöremark M, Korpenfelt SL, Hautaniemi M, et al. Chronic wasting disease in Europe: new strains on the horizon. Acta Vet Scand. 2021;63:48. DOIPubMedGoogle Scholar
- Angers RC, Seward TS, Napier D, Green M, Hoover E, Spraker T, et al. Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis. 2009;15:696–703. DOIPubMedGoogle Scholar
- Angers RC, Kang HE, Napier D, Browning S, Seward T, Mathiason C, et al. Prion strain mutation determined by prion protein conformational compatibility and primary structure. Science. 2010;328:1154–8. DOIPubMedGoogle Scholar
- Angers R, Christiansen J, Nalls AV, Kang HE, Hunter N, Hoover E, et al. Structural effects of PrP polymorphisms on intra- and interspecies prion transmission. Proc Natl Acad Sci U S A. 2014;111:11169–74. DOIPubMedGoogle Scholar
- Bian J, Christiansen JR, Moreno JA, Kane SJ, Khaychuk V, Gallegos J, 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 U S A. 2019;116:12478–87. 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 - Browning SR, Mason GL, Seward T, Green M, Eliason GA, Mathiason C, et al. Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP. J Virol. 2004;78:13345–50. DOIPubMedGoogle Scholar
- Kang HE, Weng CC, Saijo E, Saylor V, Bian J, Kim S, et al. Characterization of conformation-dependent prion protein epitopes. J Biol Chem. 2012;287:37219–32. DOIPubMedGoogle Scholar
- Taraboulos A, Jendroska K, Serban D, Yang S-L, DeArmond SJ, Prusiner SB. Regional mapping of prion proteins in brain. Proc Natl Acad Sci U S A. 1992;89:7620–4. DOIPubMedGoogle Scholar
- Fraser H, Dickinson AG. The sequential development of the brain lesion of scrapie in three strains of mice. J Comp Pathol. 1968;78:301–11. DOIPubMedGoogle Scholar
- Peretz D, Williamson RA, Legname G, Matsunaga Y, Vergara J, Burton DR, et al. A change in the conformation of prions accompanies the emergence of a new prion strain. Neuron. 2002;34:921–32. DOIPubMedGoogle Scholar
- Polymenidou M, Stoeck K, Glatzel M, Vey M, Bellon A, Aguzzi A. Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease. Lancet Neurol. 2005;4:805–14. 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
- Davenport KA, Christiansen JR, Bian J, Young M, Gallegos J, Kim S, et al. Comparative analysis of prions in nervous and lymphoid tissues of chronic wasting disease-infected cervids. J Gen Virol. 2018;99:753–8. DOIPubMedGoogle Scholar
- Prusiner SB, Scott M, Foster D, Pan K-M, Groth D, Mirenda C, et al. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell. 1990;63:673–86. DOIPubMedGoogle Scholar
- Scott M, Groth D, Foster D, Torchia M, Yang S-L, DeArmond SJ, et al. Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell. 1993;73:979–88. DOIPubMedGoogle Scholar
- Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen FE, et al. Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell. 1995;83:79–90. DOIPubMedGoogle Scholar
- Bian J, Khaychuk V, Angers RC, Fernández-Borges N, Vidal E, Meyerett-Reid C, et al. Prion replication without host adaptation during interspecies transmissions. Proc Natl Acad Sci U S A. 2017;114:1141–6. DOIPubMedGoogle Scholar
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