What veterinary practitioners should know about scrapie

Marie S. Bulgin Department of Animal and Veterinary Science, Caine Veterinary Teaching Center, University of Idaho, Caldwell, ID 83607.

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 DVM, MBA, DACVM
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Sharon Sorensen Melson Department of Animal and Veterinary Science, Caine Veterinary Teaching Center, University of Idaho, Caldwell, ID 83607.

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Contributor Notes

Supported in part by a Hatch research fund from Idaho Agricultural Experiment Station, University of Idaho (BGH145), Moscow, ID 83844, and subcontracts of the Department of Defense (contract No. DAMD17-03-1-0746); the National Institute of Neurological Disorders and Stroke, National Institutes of Health (contract No. N01-NS-0-2327); and the National Heart Lung and Blood Institute, National Intitutes of Health. The latter 3 subcontracts were provided through the Baltimore Research and Education Foundation Incorporated, 151-A, 10 N Greene St, Baltimore, MD 21201.

Address correspondence to Dr. Bulgin.
  • 1.

    Detwiler LA, Jenny AL, Rubenstein R, et al. Scrapie: a review. Sheep Goat Res J 1996;12:111–131.

  • 2.

    Pruisner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982;216:136–144.

  • 3.

    Vaccari G, Di Bari MA, Morelli L, et al. Identification of an allelic variant of the goat PrP gene associated with resistance to scrapie. J Gen Virol 2006;87:1395–1402.

    • Search Google Scholar
    • Export Citation
  • 4.

    Manuelidis L. A 25 nm virion is the likely cause of transmissible spongiform encephalopathies. J Cell Biochem 2006;100:897–915.

  • 5.

    Bastian FO, Foster FW. Spiroplasma sp. 165 rDNA in Creutzfeldt-Jacob disease and scrapie as shown by PCR and DNA sequence analyses. Neuropathol Exp Neurol 2001;60:613–620.

    • Search Google Scholar
    • Export Citation
  • 6.

    Ebringer A, Rashid T, Wilson C. Bovine spongiform encephalopathy, multiple sclerosis and Creutzfeldt-Jakob Disease are probably autoimmune diseases evoked by Acinetobacter bacteria. Ann N Y Acad Sci 2005;1050:417–428.

    • Search Google Scholar
    • Export Citation
  • 7.

    Race R, Ernst BA, Jenny A, et al. Diagnostic implications of detection of proteinase K-resistant protein in spleen, lymph nodes, and brain of sheep. Am J Vet Res 1992;53:883–888.

    • Search Google Scholar
    • Export Citation
  • 8.

    Tranulis MA. Influence of the prion protein gene, PRNP, on scrapie susceptibility in sheep. APMIS 2002;110:33–43.

  • 9.

    Hunter N, Foster JD, Benson G, et al. Restriction fragment length polymorphisms of the scrapie-associated fibril protein (PrP) gene and their association with susceptibility to natural scrapie in British sheep. J Gen Virol 1991;72:1287–1292.

    • Search Google Scholar
    • Export Citation
  • 10.

    Goldman W, Hunter N, Foster JD, et al. Two alleles of a neural protein gene linked to scrapie in sheep. Proc Natl Acad Sci U S A 1990;87:2476–2480.

    • Search Google Scholar
    • Export Citation
  • 11.

    Laplanche JL, Chataelain J, Westaway D, et al. PrP polymorphisms associated with natural scrapie discovered by denaturing gradient gel electrophoresis. Genomics 1993;15:30–37.

    • Search Google Scholar
    • Export Citation
  • 12.

    Distribution of genotypes at codon 171 in U.S. sheep. Info sheet. USDA Web site. Available at: www.aphis.usda.gov/vs/ceah/ncahs/nahms/sheep/sheep01/Genotypes.pdf. Accessed Jan 2, 2007.

    • Search Google Scholar
    • Export Citation
  • 13.

    Roucou X, LeBlanc AC. Cellular prion protein neuroprotective function: implications in prion diseases. J Mol Med 2005;83:3–11.

  • 14.

    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;36:1750–1755.

    • Search Google Scholar
    • Export Citation
  • 15.

    Gavier-Widen D, Stack MJ, Baron T, et al. Diagnosis of transmissible spongiform encephalopathies in animals: a review. J Vet Diagn Invest 2005;17:509–527.

    • Search Google Scholar
    • Export Citation
  • 16.

    Chronic wasting disease. USDA, Animal and Plant Health Inspection Service, Veterinary Services Web site. Available at: www.aphis.usda.gov/vs/nahps/cwd/. Accessed Jan 2, 2007.

    • Search Google Scholar
    • Export Citation
  • 17.

    Manual of diagnostic tests and vaccines for terrestrial animals. Chapter 2.4.8. 2004. World Organization for Animal Health Web site. Available at: www.oie.int/. Accessed Jan 2, 2007.

    • Search Google Scholar
    • Export Citation
  • 18.

    Jackman R, Everest DJ, Schmerr MJ. Evaluation of a preclinical blood test for scrapie in sheep using immunocapillary electrophoresis. J AOAC Int 2006;89:720–727.

    • Search Google Scholar
    • Export Citation
  • 19.

    Chang B, Cheng X, Yin S, et al. A blood test for prion: disease associated prion aggregate is detected in the blood of infected but asymptomatic animals. Clin Vaccine Immunol 2007;14:36–43.

    • Search Google Scholar
    • Export Citation
  • 20.

    Phase II: ovine slaughter surveillance study 2003–2006. Information sheet. Fort Collins, Colo: USDA, Animal and Plant Health Inspection Services, Veterinary Services, 2004.

    • Search Google Scholar
    • Export Citation
  • 21.

    Alexander BM, Stobart RH, Russell WC, et al. The incidence of genotypes at codon 171 of the prion protein gene (PRNP) in five breeds of sheep and productions traits of ewes associated with those genotypes. J Anim Sci 2005;83:455–459.

    • Search Google Scholar
    • Export Citation
  • 22.

    Evoniuk JM, Stoltenow CL, O'Rourke KI, et al. Assessment of the genetic risk and impact of lateral transmission in a valine-associated scrapie outbreak in sheep. Am J Vet Res 2005;66:1302–1307.

    • Search Google Scholar
    • Export Citation
  • 23.

    Bulgin MS, Sorensen SJ, Matlock M. Association between incubation time and genotype in sheep experimentally inoculated with scrapie. Am J Vet Res 2006;67:498–504.

    • Search Google Scholar
    • Export Citation
  • 24.

    Baylis M, Chihota C, Stevenson E, et al. Risk of scrapie in British sheep of different prion protein genotype. J Gen Virol 2004;85:2735–2740.

    • Search Google Scholar
    • Export Citation
  • 25.

    Scrapie program. USDA, Animal and Plant Health Inspection Service, Veterinary Services Web site. Available at: www.aphis.usda.gov/vs/nahps/scrapie. Accessed Jan 2, 2007.

    • Search Google Scholar
    • Export Citation
  • 26.

    Dawson M, Hoinville LJ, Hosie BD, et al. Guidance on the use of PrP genotyping as an aid to the control of clinical scrapie. Vet Rec 1998;623–625.

    • Search Google Scholar
    • Export Citation
  • 27.

    Texas Animal Health Commission Web site. Available at: www.tahc.state.tx.us/regs/entry/TAHC_Entry_Summary.pdf. Accessed Jan 2, 2007.

  • 28.

    Washington State Legislature Web site. Available at: www.leg.wa.gov/WAC. Accessed Jan 2, 2007.

  • 29.

    Buschmann A, Luhken G, Schultz J, et al. Neuronal accumulation of abnormal prion protein in sheep carrying a scrapie resistant genotype (PrPARR/ARR). J Gen Virol 2004;85:2727–2733.

    • Search Google Scholar
    • Export Citation
  • 30.

    Le Dur A, Beringue V, Andreoletti O, et al. A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes. Proc Natl Acad Sci U S A 2005;102:16031–16036.

    • Search Google Scholar
    • Export Citation
  • 31.

    Delpha Press Web page. Department for Environment, Food and Rural Affairs Web site. Available at: www.defra.gov.uk/animalh/bse/othertses/scrapie/nsp/Atypicalcases/index.htm. Accessed Jan 2, 2007.

    • Search Google Scholar
    • Export Citation
  • 32.

    Hunter N, Foster JD, Goldman W, et al. Natural scrapie in a closed flock of Cheviot sheep occurs only in specific PrP genotypes. Arch Virol 1996;141:809–824.

    • Search Google Scholar
    • Export Citation
  • 33.

    Goldman W, Hunter N, Smith G, et al. PrP genotype and agent effects in scrapie: change in allelic interaction with different isolates of agent in sheep, a natural host of scrapie. J Gen Virol 1994;75:989–995.

    • Search Google Scholar
    • Export Citation
  • 34.

    Bruce M, Chree A, McConnell I, et al. Transmission and strain typing studies of scrapie and BSE. In: Court L, Dodit B, eds. Transmissible subacute spongiform encephalopathies: prion diseases. Paris: Elsevier, 2004;259–262.

    • Search Google Scholar
    • Export Citation
  • 35.

    Benestad SL, Sarradin P, Thu B, et al. Cases of scrapie with unusual features in Norway and designation of a new type, Nor98. Vet Rec 2000;153:202–208.

    • Search Google Scholar
    • Export Citation
  • 36.

    Onnasch H, Bradshaw BJ, Benestad SK, et al. Two Irish cases of scrapie resembling Nor98. Vet Rec 2004;155:636–637.

  • 37.

    Moum T, Olsaker I, Hopp P, et al. Polymorphisms at codons 141 and 154 in the ovine prion protein gene are associated with scrapie Nor98 cases. J Gen Virol 2005;86:231–235.

    • Search Google Scholar
    • Export Citation
  • 38.

    Hopp P, Ulvuund MJ, Jarp JA. A case control study on scrapie in Norwegian sheep flocks. Prev Vet Med 2001;51:183–198.

  • 39.

    Foote WC, Clark W, Maciulis A, et al. Prevention of scrapie transmission in sheep, using embryo transfer. Am J Vet Res 1993;54:1863–1868.

    • Search Google Scholar
    • Export Citation
  • 40.

    Tuo W, Zhuang D, Knowles DP, et al. PrP-C and PrP-Sc at the fetal-maternal interface. J Biol Chem 2001;276:18229–18234.

  • 41.

    Tuo W, O'Rourke KI, Zhuang D, et al. Pregnancy status and fetal prion genetics determine PrPSC accumulation in placentomes of scrapie-infected sheep. Proc Natl Acad Sci U S A 2002;99:6310–63315.

    • Search Google Scholar
    • Export Citation
  • 42.

    Alverson J, O'Rourke KI, Baszler TV. PrPSC accumulation in fetal cotyledons of scrapie-resistant lambs is influenced by fetus location in the uterus. J Gen Virol 2006;87:1035–1041.

    • Search Google Scholar
    • Export Citation
  • 43.

    Hunter N, Foster J, Chang A, et al. Transmission of prion diseases by blood transfusion. J Gen Virol 2000;83:2897–2905.

  • 44.

    Prinz M, Heikenwalder M, Junt T, et al. Positioning of follicular dendritic cells within the spleen controls prion neuroinvasion. Nature 2003;425:957–962.

    • Search Google Scholar
    • Export Citation
  • 45.

    Heikenwalder M, Aeller N, Seeger H, et al. Chronic lymphocytic inflammation specifies the organ tropism of prions. Science 2005;307:1107–1110.

    • Search Google Scholar
    • Export Citation
  • 46.

    Ligios C, Sigurdson CJ, Santucciu C, et al. PrPSC in mammary glands of sheep affected by scrapie and mastitis. Nature Med 2005;11:1137–1138.

    • Search Google Scholar
    • Export Citation
  • 47.

    Brown P, Gajdusek DC. Survival of scrapie virus after 3 years interment. Lancet 1991;337:269–270.

  • 48.

    Vasina EN, Dejardin P, Rezaei H, et al. Fate of prions in soil: adsorption kinetics of recombinant unglycosylated ovine prion protein onto mica in laminar flow conditions and subsequent desorption. Biomacromolecules 2005;6:3425–3432.

    • Search Google Scholar
    • Export Citation
  • 49.

    Georgsson G, Sigurdarson S, Brown P. Infectious agent of sheep scrapie may persist in the environment for at least 16 years. J Gen Virol 2006;87:3737–3740.

    • Search Google Scholar
    • Export Citation
  • 50.

    Gudmundsdottir KB, Sigurdarson S, Kristinsson J, et al. Iron and iron/manganese ratio in forage from Icelandic sheep farms: relation to scrapie. Acta Vet Scand 2006;48:16. Available at: www.actavetscand.com/content/48/1/16. Accessed Jan 2, 2007.

    • Search Google Scholar
    • Export Citation
  • 51.

    Wisniewski HM, Sigurdarson S, Rubenstein R, et al. Mites as vectors for scrapie. Lancet 1996;347:1114.

  • 52.

    Miller JM, Jenny AL, Taylor WD, et al. Immunohistochemical detection of prion protein in sheep with scrapie. J Vet Diagn Invest 1993;5:309–316.

    • Search Google Scholar
    • Export Citation
  • 53.

    Ersdal C, Ulvund MJ, Benestad SL, et al. Accumulation of pathogenic prion protein (PrPSC) in nervous and lymphoid tissues of sheep with subclinical scrapie. Vet Pathol 2003;40:164–174.

    • Search Google Scholar
    • Export Citation
  • 54.

    O'Rourke KI, Duncan JV, Logan JR, et al. Active surveillance for scrapie by third eyelid biopsy and genetic susceptibility testing of flocks of sheep in Wyoming. Clin Diagn Lab Immunol 2002;9:966–971.

    • Search Google Scholar
    • Export Citation
  • 55.

    Roels S, Vanopdenbosch E, Langeveld JPM, et al. Immunohistochemical evaluation of tonsillar tissue for preclinical screening of scrapie based on surveillance in Belgium. Vet Rec 1999;145:524–525.

    • Search Google Scholar
    • Export Citation
  • 56.

    Corona C, Casalone C, Autis PL, et al. Detection of pathological prion protein in the tongue of sheep infected with naturally occurring scrapie. Vet Res Com 2006;30:239–240.

    • Search Google Scholar
    • Export Citation
  • 57.

    Greenlee JJ, Hamir AN, West Greenlee MH. Abnormal prion accumulation associated with retinal pathology in experimentally inoculated scrapie-affected sheep. Vet Pathol 2006;43:733–739.

    • Search Google Scholar
    • Export Citation
  • 58.

    Espenes A, Press CM, Landsverk T, et al. Detection of PrPSC in rectal biopsy and necropsy samples from sheep with experimental scrapie. J Comp Pathol 2006;115–125.

    • Search Google Scholar
    • Export Citation
  • 59.

    Picard-Hagen N, Gayrard V, Viguie C, et al. Prion protein in the cerebrospinal fluid of healthy and naturally scrapie-affected sheep. J Gen Virol 2006;87:3723–3727.

    • Search Google Scholar
    • Export Citation
  • 60.

    Saa P, Castilla J, Soto C. Presymptomatic detection of prions in blood. Science 2006;313:92–94.

  • 61.

    Ligios C, Cancedda MG, Mandau L, et al. PrPSC deposition in nervous tissues without lymphoid tissue involvement is frequently found in ARQ/ARQ Sarda breed sheep preclinically affected with natural scrapie. Arch Virol 2006;151:2007–2020.

    • Search Google Scholar
    • Export Citation
  • 62.

    Schreuder BEC, van Keulen LJM, Vromans MEW, et al. Tonsillar biopsy and PrPSC detection in the preclinical diagnosis of scrapie. Vet Rec 1998;142:564–568.

    • Search Google Scholar
    • Export Citation
  • 63.

    Race RE, Raymond GJ. Inactivation of transmissible spongiform encephalopathy (prion) agents by environ LpH. J Virol 2004;78:2164–2165.

    • Search Google Scholar
    • Export Citation

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