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Natural Transmission and Genetic Control of Susceptibility of Sheep to Scrapie

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Transmissible Spongiform Encephalopathies:

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 172))

Abstract

Scrapie has often been the subject of controversy and speculation both by farmers and by scientists. Not the least strange of numerous etiologic ideas was that put forward by RocheLubin in 1848 who suggested that scrapie was caused by sexual excess, sunstroke after shearing, or lightning. The recent suggestion that kuru, a human scrapie-like disease, was the result of a volcanic eruption (Schoental 1990) is therefore part of a long tradition. The lightning analogy is not a bad one, however. One of the most common remarks made by farmers having to deal with a scrapie outbreak is that it appeared suddenly and apparently from nowhere; reports like this are still being made from, for instance, Sweden (Elvander et al. 1988), Cyprus (Toumazos 1988) and Italy (Mechelli and Mantovani 1988). Another common belief is that the disease was “brought in” by a recently purchased ram. Such anecdotal evidence is difficult to substantiate, especially as scrapie has such stigma attached to it that true incidence figures have been difficult to obtain. It is probably a much more common disease than would seem from official diagnosis figures (Chatelain et al. 1983; N. Hunter 1991). In addition, the lack of a preclinical dignostic test means that animals incubating scrapie cannot be identified until they develop symptoms. The number of carrier-status animals, if indeed they exist at all, is completely unknown, despite their obvious importance in any hypothesis of natural transmission mechanisms.

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References

  • Asher DM, Masters CL, Gajdusek DC, Gibbs CJ (1983) Familial spongiform encephalopathies. In: , Rowland LP, Sidman RL, Matthysse SW (eds) Genetics of neurological and psychiatric disorders. Raven, New York, pp 273–291

    Google Scholar 

  • Bang FB (1965) Genetic and cellular factors in resistance to virus diseases. In: Gajdusek DC, Gibbs CJA, Alpers M (eds) Slow, latent and temperate virus infections. US Department of Health Education and Welfare, Washington DC, pp 389–392 (Health service publication no 1378) (National Institute of Neurological Diseases and Blindness monograph no 2)

    Google Scholar 

  • Brotherston JG, Renwick CC, Stamp JT, Zlotnik I (1968) Spread of scrapie by contact to goats and sheep. J Comp Pathol 78: 9–17

    Article  PubMed  CAS  Google Scholar 

  • Carlson GA, Kingsbury DT, Goodman PA, Coleman S, Marshall ST, DeArmond S, Westaway D, Prusiner SB (1986) Linkage of prion protein and scrapie incubation time genes. Cell 46: 503–511

    Article  PubMed  CAS  Google Scholar 

  • Carlson GA, Goodman PA, Lovett M, Taylor BA, Marshall ST, Petersa-Forchia M, Westaway D, Prusiner SB (1989) Genetics and polymorphism of the mouse prion gene complex: control of scrapie incubation time. Mol Cell Biol 8: 5528–5540

    Google Scholar 

  • Cathala F, Brown P, Gray F, Sulima M, Chatelain J, Gibbs CJ (1985) Failure to detect scrapie virus in sheep at slaughter in a highly endemic region of France. Eur J Epidemiol 1: 90–93

    Article  PubMed  CAS  Google Scholar 

  • Chandler RL (1963) Experimental scrapie in the mouse. Res Vet Sei 4: 276–285

    Google Scholar 

  • Chatelain J, DeLasnerie-Laupretre N, Cathala F (1983) Scrapie in France: some possible predisposing factors in the naturally-acquired disease of sheep. Vet Microbiol 8: 511–515

    Article  PubMed  CAS  Google Scholar 

  • Collinge J, Harding AE, Owen F, Poulter M, Lofthouse R, Boughey AM, Shah T, Crowe TJ (1989) Diagnosis of Gerstmann-Sträussler syndrome in familial dementia with prion protein gene analysis. Lancet i: 15–17

    Article  Google Scholar 

  • Collis SC, Kimberlin RH (1985) Long-term persistence of scrapie infection in mouse spleens in the absence of clinical disease. FEMS Microbiol Lett 29: 111–114

    Article  Google Scholar 

  • Collis SC, Millson GC (1975) Transferrin polymorphism in Herdwick sheep. Anim Blood Groups Biochem Genet 6: 117–120

    Article  PubMed  CAS  Google Scholar 

  • Collis SC, Millson GC, Kimberlin RH (1977) Genetic markers in Herdwick sheep: no correlation with, susceptibility or resistance to experimental scrapie. Anim Blood Groups Biochem Genet. 8:79–83

    Article  PubMed  CAS  Google Scholar 

  • Cullen PR (1989) Scrapie and the sheep MHC: claims of linkage refuted. Immunogenetics 29:414–416

    Article  PubMed  CAS  Google Scholar 

  • Cullen PR, Brownlie J, Kimberlin RH (1984) sheep lymphocyte antigens and scrapie. J Comp Pathol 94:405–415

    Article  PubMed  CAS  Google Scholar 

  • Davies DC, Kimberlin RH (1985) Selection of Swaledale sheep of reduced susceptibility to experimental scrapie. Vet Rec 116: 211–214

    Article  PubMed  CAS  Google Scholar 

  • Daya M, Wong F, Cervin M, Evans G, Vennema H, Spaan W, Anderson RV (1989) Mutation of host cell determinants which descriminate between lytic and persistent mouse hepatitis virus infection results in a fusion-resistant phenotype. J Gen Virol 70: 3335–3346

    Article  PubMed  Google Scholar 

  • Diamond JM (1987) AIDS: infectious, genetic or both? Nature 328: 199–200

    Article  PubMed  CAS  Google Scholar 

  • Dickinson AG (1974) Natural genetic infection, ‘spontaneous generation’ and scrapie. Nature 252: 179–180

    Article  PubMed  CAS  Google Scholar 

  • Dickinson AG (1975) Host-pathogen interactions in scrapie. Genetics 79: 387–395

    PubMed  Google Scholar 

  • Dickinson AG (1976) Scrapie in sheep and goats. In: Kimberlin RH (ed) Slow virus diseases of animals and man. North-Holland, Amsterdam, pp 209–241

    Google Scholar 

  • Dickinson AG, MacKay JMK (1967) Virus hosts and genetic studies. In: Maramorosch K, Koprowski H (eds) Methods in virology, vol. 1 Academic, New York, pp19–61

    Google Scholar 

  • Dickinson AG, Outram GW (1988) Genetic aspects of unconventional virus infections: the basis of the virino hypothesis. In: Bock G, Morsh J (eds) Novel infectious agents and the central nervous system. Wiley, Chichester, pp 63–83 (CIBA Found symp 135)

    Google Scholar 

  • Dickinson AG, Young GB, Stamp JT, Renwick CC (1964) A note on the distribution of scrapie in sheep of different ages. Anim Prod 6: 375–377

    Article  Google Scholar 

  • Dickinson AG, Young GB, Stamp JT, Renwick CC (1965) An analysis of natural scrapie in Suffolk sheep. Heredity (Edinburgh) 20: 485–503

    CAS  Google Scholar 

  • Dickinson AG, Young GB, Renwick CC (1966) Scrapie: experiments involving maternal transmission in sheep. Report of scrapie seminar, 1964 ARS 91–53. US Department of Agriculture, 244–248, Washington DC

    Google Scholar 

  • Dickinson AG, Stamp JT, Renwick CC, Rennie JC (1968) Some factors controlling the incidence of scrapie in Cheviot sheep injected with a Cheviot passaged scrapie agent. J Comp Pathol 78: 313–321

    Article  PubMed  CAS  Google Scholar 

  • Dickinson AG, Stamp JT, Renwick CC (1974) Maternal and lateral transmission of scrapie in sheep. J Comp Pathol 84: 19–25

    Article  PubMed  CAS  Google Scholar 

  • Dickinson AG, Fraser H, Outram GW (1975) Scrapie incubation time can exceed natural lifespan. Nature 256: 732–733

    Article  PubMed  CAS  Google Scholar 

  • Elvander M, Engvali A, Klingeborn B (1988) Scrapie in sheep in Sweden. Acta Vet Scand 29: 509–510

    PubMed  CAS  Google Scholar 

  • Foster JD, Dickinson AG (1988a) The unusual properties of CH1641, a sheep passaged isolate of scrapie. Vet Rec. 123: 5–8

    Article  PubMed  CAS  Google Scholar 

  • Foster JD, Dickinson AG (1988b) Genetic control of scrapie in cheviot and Suffolk sheep. Vet Rec 123: 159

    Article  PubMed  CAS  Google Scholar 

  • Foster JD, Dickinson AG (1989) Age at death from natural scrapie in a flock of Suffolk sheep. Vet Rec 125:415–417

    Article  PubMed  CAS  Google Scholar 

  • Fraser H (1983) A survey of primary transmission of Icelandic scrapie (rida) to mice. In: Court LA, Cathala F (eds) Virus non conventionnels et affection du system nerveux central. Masson, Paris, pp 34–46

    Google Scholar 

  • Goldmann W, Hunter N, Foster JD Salbaum JM, Beyreuther K, Hope J (1990) Two alleles of a neural protein gene linked to scrapie in sheep. Proc Natl Acad Sci USA 87: 2476–2480

    Article  PubMed  CAS  Google Scholar 

  • Gordon WS (1946) Louping-ill, tick-borne fever and scrapie. Vet Rec 58: 516–520

    PubMed  CAS  Google Scholar 

  • Gordon WS (1966) Variation in susceptibility of sheep to scrapie and genetic implications. In: Report of scrapie seminar, 1964 ARS 91–53. US Department of Agriculture 53–67, Washington DC

    Google Scholar 

  • Greig JR (1940a) Scrapie. Trans Highl Agric Soc Scot 52: 71–90

    Google Scholar 

  • Greig JR (1940b) Observations on the transmission of the disease by mediate contact. Vet J 96: 203–206

    Google Scholar 

  • Greig JR (1950) Scrapie in sheep. J Comp Pathol 60: 263–266

    PubMed  CAS  Google Scholar 

  • Hadlow WT, Kennedy RC, Race RE (1982) Natural infection of Suffolk sheep with scrapie virus. J Infect Dis 146:657–664

    Article  PubMed  CAS  Google Scholar 

  • Hoare M, Davies DC, Pattison IH (1977) Experimental production of scrapie-resistant Swaledale sheep. Vet Rec 101: 482–484

    PubMed  CAS  Google Scholar 

  • Hope J, Morton LJD, Farquhar CF, Multhaup G, Beyreuther K, Kimberlin RH (1986) The major polypeptide of scrapie-associated fibrils (SAF) has the same size, charge distribution and N-terminal sequence as predicted for the normal brain protein (PrP). EMBO J 5: 2591–2597

    PubMed  CAS  Google Scholar 

  • Hourrigan J, Klingsporm A, Clark WW, deCamp M (1979) Epidemiology of scrapie in the United Slates. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic, New York, pp. 331–356

    Google Scholar 

  • Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F, Terwilliger JD, Westaway D, Ott J, Prusiner SB (1989) Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature 338: 342–345

    Article  PubMed  CAS  Google Scholar 

  • Hunter N, Hope J, McConnell I, Dickinson AG (1987) Linkage of the scrapie-associated fibril protein (PrP) gene and Sinc using congenic mice and restriction fragment polymorphism analysis. J Gen Virol 68: 2711–2716

    Article  PubMed  CAS  Google Scholar 

  • Hunter N, Foster JD, Dickinson AG, Hope J (1989) Linkage of the gene for the scrapie-associated fibril protein (PrP) to the Sip gene in Cheviot sheep. Vet Rec 124: 364–366

    Article  PubMed  CAS  Google Scholar 

  • Hunter N, Foster JD, Benson G, Hope J (1991) 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 72: (in press)

    Google Scholar 

  • Joubert L, Lapras M, Gastelli J, Prare M, Laurent D (1972) Un foyer de tremblante du mouton en Provence. Bull Soc Sci Vet Med Comp 74: 165–184

    Google Scholar 

  • Kimberlin RH, Walker CA (1979) Pathogenesis of mouse scrapie: dynamics of agent replication in spleen, spinal cord and brain after infection by different routes. J Comp Pathol 89: 551–562

    Article  PubMed  CAS  Google Scholar 

  • Kiimberlin RH, Walker CA (1980) Pathogenesis of mouse scrapie: evidence for neural spread of infection the CNS. J Gen Virol 51: 183–187

    Article  Google Scholar 

  • Kimberlin RH, Walker CA (1982) Pathogenesis of mouse scrapie: patterns of agent replication in different parts of the CNS following intraperitional infection. J R Soc Med 75: 618–624

    PubMed  CAS  Google Scholar 

  • Kiimberlin RH, Walker CA (1989) Pathogenesis of scrapie in mice after intragastric infection. Virus Res 12: 213–220

    Article  Google Scholar 

  • Lackner AA, Marx, PA, Lerche NW, Gardner UB, Kluge JD, Spinner A, Kwang H-S, Lowenstine LJ (1989) Asymptomatic infection of the central nervous system by the macaque immunosuppressive type D retrovirus-SRV-1. J Gen Virol 70: 1641–1651

    Article  PubMed  Google Scholar 

  • Lodmell DL (1983) Genetic control of resistance to street rabies virus in mice. J Exp Med 157: 451–460

    Article  PubMed  CAS  Google Scholar 

  • Lodmell DL, Wiedbrauk DL, Ewalt LC (1989) Interferon induced within the central nervous system during infection is inconsequential as a mechanism responsible for murine resistance to street rabies virus. J Gen Virol 70: 473–478

    Article  PubMed  CAS  Google Scholar 

  • London WT, Houff SA, Madden DL, Fuccillo DA, Gravell M, Wallen WC, Palmer AE, Sever JL, Padgett BL, Walker DL, ZuRhein GM, Ohashi T (1978) Brain tumors in owl monkeys inoculated with a human polyoma virus (JC virus). Science 201: 1246–1249

    Article  PubMed  CAS  Google Scholar 

  • Lowenstein DH, Butler DA, Westaway D, McKinley MP, DeArmond SJ, Prusiner SB (1990) Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins. Mol Cell Biol 10: 1153–1163

    PubMed  CAS  Google Scholar 

  • Mantijarvi RA, Meurman OH, Vihma L, Berlund B (1973) A human papovavirus (BK): biological properties and seroepidemiology. Ann Clin Res 5: 283

    Google Scholar 

  • Mechelli L, Mantovani AL (1988) Scrapie in sheep in central and southern Italy. Vet Res Commun 12: 165–167

    Article  PubMed  CAS  Google Scholar 

  • Millot P, Chatelain J, Dautheville C, Salmon D, Cathala F (1988) Sheep major histocompatibility (OLA) complex: linkage between a scrapie susceptibility/resistance locus and the OLA complex in Ilede-France sheep progenies. Immunogenetics 27: 1–11

    Article  PubMed  CAS  Google Scholar 

  • Nettleton PF (1987) Pathogenesis and epidemiology of Border disease. Ann Rec Vet 18: 147–155

    CAS  Google Scholar 

  • Nussbaum RE, Henderson WM, Pattison IH, Elcock NV, Davies DC (1975) The establishment of sheep flocks of predictable susceptibility to experimental scrapie. Res Vet. Sei 18: 49–58

    CAS  Google Scholar 

  • Owen F, Poulter M, Lofthouse R, Collinge J, Crow TJ, Risby D, Baker HF, Ridley RM, Hsiao K, Prusiner SB (1989) Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet i: 51–52

    Article  Google Scholar 

  • Owen F, Poulter M, Shah T, Collinge J, Lofthouse J, Baker H, Ridley R, McVey J, Crow TJ (1990) An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease. Mol Brain Res 7: 273–276

    Article  PubMed  CAS  Google Scholar 

  • Padgett BL (1980) Human papovaviruses. In: Tooce J (ed) Molecular biology of tumor viruses, 2nd ed 2: DNA tumor viruses. Cold Spring Harbor Laboratory, New York, pp 339–370

    Google Scholar 

  • Padgett BL, and Walker DL (1973) Prevalence of antibodies in human sera against JC virus, an isolate from a case of PML. J Infect Dis 127: 467

    Article  PubMed  CAS  Google Scholar 

  • Padgett BL, Walker DL, ZuRhein GM, Varakis JN (1977) Differential neurooncogenicity of strains of JC virus, a human polyoma virus, in newborn Syrian hamsters. Cancer Res 37: 718–720

    PubMed  CAS  Google Scholar 

  • Palsson PA (1979) Rida (scrapie) in Iceland its epidemiology. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic, New York, pp 357–366

    Google Scholar 

  • Parry HB (1960) Scrapie: a transmissible hereditary disease of sheep. Nature 185: 441–443

    Article  PubMed  CAS  Google Scholar 

  • Parry HB (1962) Scrapie: a transmissible and hereditary disease of sheep. Heredity 17: 75–105

    Article  PubMed  CAS  Google Scholar 

  • Pattison IH (1966) The relative susceptibility of sheep, goats and mice to two types of the goat scrapie agent. Res Vet Sci 7: 207–212

    PubMed  CAS  Google Scholar 

  • Pattison IH (1974) Scrapie in sheep selectively bred for high susceptibility. Nature 248: 594

    Article  PubMed  CAS  Google Scholar 

  • Pattison IH, Hoare MN, Jebbett JN, Watson WA (1972) Spread of scrapie to sheep and goats by oral dosing with foetal membranes from scrapie affected sheep. Vet Rec 90: 465–468

    Article  PubMed  CAS  Google Scholar 

  • Pattison IH, Hoare MN, Jebbett JN, Watson WA (1974) Further observations on the production of scrapie in sheep by oral dosing with foetal membranes from scrapie-affected sheep. Br Vet J 130: 65–67

    Google Scholar 

  • Primrose SB, Dimmock NJ (1980) Introduction to modern virology, 2nd ed, Blackwell, Oxford

    Google Scholar 

  • Race RE, Graham K, Ernst D, Caughey B, Chesebro B (1990) Analysis of linkage between scrapie incubation period and the prion protein gene in mice. J Gen Virol 71: 493–497

    Article  PubMed  CAS  Google Scholar 

  • Robakis NK, Devine-Gage EA, Jenkins EC, Kascsak RJ, Brown WT, Krawczun MS, Silverman WP (1986) Localization of a human gene homologous to the PrP gene on the p arm of chromosome 20 and detection of PrP-related antigens in normal human brain. Biochem Biophys Res Commun 140:758–765

    Article  PubMed  CAS  Google Scholar 

  • Roche-Lubin (1848) Memoire practique sur la maladie des betes a laine connue sous les noms de prurigo, lombaire, maladie convulsive, trembleuse, tremblant, etc. Rec Med Vet Ser 3 (5): 698–714

    Google Scholar 

  • Rubenstein R, Merz PA, Kascsak RJ, Carp RI, Scalici CL, Fama CL, Wisniewski HM (1987) Detection of scrapie associated fibrils (SAF) and SAF proteins from scrapie-affected sheep. J Infect Dis 156: 36–42

    Article  PubMed  CAS  Google Scholar 

  • Sabin AB (1952) Nature of inherited resistance to viruses affecting the nervous system. Proc Natl Acad Sci USA 38: 540–546

    Article  PubMed  CAS  Google Scholar 

  • Schell K (1960) Studies on the innate resistance of mice to infection with musepox. II. Route of inoculation and resistance: and some observations on the inheritance of resistance. Aust J Exp Biol 38: 289–300

    Article  CAS  Google Scholar 

  • Schoental R (1990) Kuru, mycotoxins and volcanic eruptions. J App Toxicol 10: 153

    Article  CAS  Google Scholar 

  • Scott JL, Blanchard-Channell M, Scibienski RJ, Scott ML (1990) Interaction of bluetongue virus with bovine lymphocytes. J Gen. Virol 71: 363–368

    Article  Google Scholar 

  • Scott M, Foster D, Mirenda C, Serban D, Confal F, Walchli M, Torchis M, Groth D, Carlson G, DeArmond SJ, Westaway D, Prusiner SB (1989) Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell 59: 847–857

    Article  PubMed  CAS  Google Scholar 

  • Shah KV, Daniel RW, Warszawski R (1973) High prevalence of antibodies to BK virus and SV40 related papovaviruses. J Natl Cancer Inst 54: 945

    Google Scholar 

  • Small JA, Scangos GA, Cork L, Jay G, Khoury G (1986) The early region of Human papovavirus JC induces dysmyelination in transgenic mice. Cell 46: 13–18

    Article  PubMed  CAS  Google Scholar 

  • Stamp JT (1962) Scrapie: a transmissible disease of sheep. Vet. Rec 74: 357–362

    Google Scholar 

  • Toumazos P (1988) First report of ovine scrapie in Cyprus. Br Vet J 1144: 98–99

    Article  Google Scholar 

  • Varakis J, ZuRhein GM, Padgett BL, Walker DL (1978) Induction of peripheral neuroblastomas in Syrian hamsters after injection as neonates with JC virus, a human polyoma virus. Cancer Res: 1718–1722

    Google Scholar 

  • Westaway D, Prusiner SB (1986) Conservation of the cellular gene encoding the scrapie prion protein. Nucleic Acids Res 14: 2035–2045

    Article  PubMed  CAS  Google Scholar 

  • Westaway D, Goodman PA, Mirenda CA, McKinley MP, Carlson GA, Prusiner SB (1987) Distinct prion proteins in short and long scrapie incubation period mice. Cell 51: 651–662

    Article  PubMed  CAS  Google Scholar 

  • Zlotnik I, Katiyar RD (1961) The occurrence of scrapie disease in sheep of the remote Himalayan foothills. Vet Rec 73: 543–544

    Google Scholar 

  • Zur Hausen H (1980) Oncogenic herpes viruses. In: Toote J (ed) Molecular biology of tumor viruses, 2nd ed, pt 2. Cold Spring Harbor, New York, pp 747–795

    Google Scholar 

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Hunter, N. (1991). Natural Transmission and Genetic Control of Susceptibility of Sheep to Scrapie. In: Chesebro, B.W. (eds) Transmissible Spongiform Encephalopathies:. Current Topics in Microbiology and Immunology, vol 172. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-76540-7_11

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