Classical and Atypical Scrapie in Sheep and Goats. Review on the Etiology, Genetic Factors, Pathogenesis, Diagnosis, and Control Measures of Both Diseases

Overview

Journal Animals (Basel)

Date 2021 Apr 3

PMID 33806658

Citations 9

Authors

Cristina Acin

Rosa Bolea

Marta Monzon

Eva Monleon

Bernardino Moreno

Hicham Filali

Belen Marin

Diego Sola

Marina Betancor

Isabel M Guijarro

Mirta Garcia

Antonia Vargas

Juan Jose Badiola

Affiliations

Soon will be listed here.

Abstract

Prion diseases, such as scrapie, are neurodegenerative diseases with a fatal outcome, caused by a conformational change of the cellular prion protein (PrP), originating with the pathogenic form (PrP). Classical scrapie in small ruminants is the paradigm of prion diseases, as it was the first transmissible spongiform encephalopathy (TSE) described and is the most studied. It is necessary to understand the etiological properties, the relevance of the transmission pathways, the infectivity of the tissues, and how we can improve the detection of the prion protein to encourage detection of the disease. The aim of this review is to perform an overview of classical and atypical scrapie disease in sheep and goats, detailing those special issues of the disease, such as genetic factors, diagnostic procedures, and surveillance approaches carried out in the European Union with the objective of controlling the dissemination of scrapie disease.

Citing Articles

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References

Baron T, Vulin J, Biacabe A, Lakhdar L, Verchere J, Torres J . Emergence of classical BSE strain properties during serial passages of H-BSE in wild-type mice. PLoS One. 2011; 6(1):e15839. PMC: 3021503. DOI: 10.1371/journal.pone.0015839. View

Matsuura Y, Miyazawa K, Imamura M, Yokoyama T, Iwamaru Y . First case of atypical scrapie in a goat in Japan. J Vet Med Sci. 2019; 81(7):986-989. PMC: 6656802. DOI: 10.1292/jvms.18-0710. View

Zou W, Capellari S, Parchi P, Sy M, Gambetti P, Chen S . Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease. J Biol Chem. 2003; 278(42):40429-36. DOI: 10.1074/jbc.M308550200. View

Debeer S, Baron T, Bencsik A . Immunohistochemistry of PrPsc within bovine spongiform encephalopathy brain samples with graded autolysis. J Histochem Cytochem. 2001; 49(12):1519-24. DOI: 10.1177/002215540104901205. View

Garza M, Erana H, Castilla J, Acin C, Vargas A, Badiola J . Protein misfolding cyclic amplification corroborates the absence of PrP accumulation in placenta from foetuses with the ARR/ARQ genotype in natural scrapie. Vet Microbiol. 2017; 203:294-300. DOI: 10.1016/j.vetmic.2017.03.034. View

Westergard L, Christensen H, Harris D . The cellular prion protein (PrP(C)): its physiological function and role in disease. Biochim Biophys Acta. 2007; 1772(6):629-44. PMC: 1986710. DOI: 10.1016/j.bbadis.2007.02.011. View

ORourke K, Baszler T, Besser T, Miller J, Cutlip R, Wells G . Preclinical diagnosis of scrapie by immunohistochemistry of third eyelid lymphoid tissue. J Clin Microbiol. 2000; 38(9):3254-9. PMC: 87369. DOI: 10.1128/JCM.38.9.3254-3259.2000. View

Sharp M, Collings D . Ovine abomasal enlargement and scrapie. Vet Rec. 1987; 120(9):215. DOI: 10.1136/vr.120.9.215. View

Dassanayake R, White S, Madsen-Bouterse S, Schneider D, ORourke K . Role of the PRNP S127 allele in experimental infection of goats with classical caprine scrapie. Anim Genet. 2015; 46(3):341. PMC: 5132141. DOI: 10.1111/age.12291. View

10.

Heim D, Kihm U . Risk management of transmissible spongiform encephalopathies in Europe. Rev Sci Tech. 2003; 22(1):179-99. DOI: 10.20506/rst.22.1.1392. View

11.

Rezaie P, Lantos P . Microglia and the pathogenesis of spongiform encephalopathies. Brain Res Brain Res Rev. 2001; 35(1):55-72. DOI: 10.1016/s0165-0173(01)00042-x. View

12.

Acutis P, Bossers A, Priem J, Riina M, Peletto S, Mazza M . Identification of prion protein gene polymorphisms in goats from Italian scrapie outbreaks. J Gen Virol. 2006; 87(Pt 4):1029-1033. DOI: 10.1099/vir.0.81440-0. View

13.

Espenes A, McL Press C, Landsverk T, Tranulis M, Aleksandersen M, Gunnes G . Detection of PrP(Sc) in rectal biopsy and necropsy samples from sheep with experimental scrapie. J Comp Pathol. 2006; 134(2-3):115-25. DOI: 10.1016/j.jcpa.2005.08.001. View

14.

Siso S, Jeffrey M, Steele P, McGovern G, Martin S, Finlayson J . Occurrence and cellular localization of PrPd in kidneys of scrapie-affected sheep in the absence of inflammation. J Pathol. 2008; 215(2):126-34. DOI: 10.1002/path.2336. View

15.

Benestad S, Arsac J, Goldmann W, Noremark M . Atypical/Nor98 scrapie: properties of the agent, genetics, and epidemiology. Vet Res. 2008; 39(4):19. DOI: 10.1051/vetres:2007056. View

16.

Houston F, McCutcheon S, Goldmann W, Chong A, Foster J, Siso S . Prion diseases are efficiently transmitted by blood transfusion in sheep. Blood. 2008; 112(12):4739-45. DOI: 10.1182/blood-2008-04-152520. View

17.

Le Dur A, Beringue V, Andreoletti O, Reine F, Lai T, Baron T . A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes. Proc Natl Acad Sci U S A. 2005; 102(44):16031-6. PMC: 1276041. DOI: 10.1073/pnas.0502296102. View

18.

Hoinville L . A review of the epidemiology of scrapie in sheep. Rev Sci Tech. 1996; 15(3):827-52. DOI: 10.20506/rst.15.3.959. View

19.

Benestad S, Austbo L, Tranulis M, Espenes A, Olsaker I . Healthy goats naturally devoid of prion protein. Vet Res. 2012; 43:87. PMC: 3542104. DOI: 10.1186/1297-9716-43-87. View

20.

Detwiler L . Scrapie. Rev Sci Tech. 1992; 11(2):491-537. DOI: 10.20506/rst.11.2.607. View