Summary
Natural scrapie in a closed flock of South Country Cheviot sheep has resulted in 45 deaths between 1986 and 1995. Of these cases, 35 sheep have been analysed for disease-linked PrP gene polymorphisms and all encode valine at codon 136 on at least one allele with 77% homozygous (VV136) and 23% valine/alanine heterozygotes (VA136). Mean survival time was 907 and 1482 days for VV136 and VA136 scrapie affected animals respectively. VV136 animals were all at great risk of disease if allowed to live long enough. However scrapie occurred only in a specific subgroup of VA136 sheep, survival advantage depending on VA136 animals being heterozygous for other polymorphisms at codons 154 or 171. The flock history has been recorded in great detail since its foundation in 1960 however there was no strong evidence for simple maternal or paternal transmission of disease other than inheritance of PrP genotype.
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References
Albritton LM, Tseng L, Scadden D, Cunningham JM (1989) A putative murine ecotropic retrovirus receptor gene encodes a multiple membrane-spanning protein and confers susceptibility to virus infection. Cell 57: 659–666
Belt PBGM, Muileman IH, Schreuder BEC, Bos-de Ruijter J, Gielkens ALJ, Smits MA (1995) Identification of five allelic variants of the sheep PrP gene and their association with natural scrapie. J Gen Virol 76: 509–517
Chatelain J, Millot P, Bourdon R, Callebert J, Fandon R, Grolleau D, Trouillet D (1992) Diverse biological parameters in clinically healthy sheep from a flock with scrapie: variations and correlations with OLA antigens. Exp Clin Immunogenet 9: 86–94
Clouscard C, Beaudry P, Elsen JM, Milan D, Dussaucy M, Bounneau C, Schelcher F, Chatelain J, Launay JM, Laplanche JL (1995) Different allelic effects of the codons 136 and 171 of the prion protein gene in sheep with natural scrapie. J Gen Virol 76: 2097–2101
Collinge J, Palmer MS, Dryden AJ (1991) Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease. Lancet 337: 1441–1442
Deslys JP, Marce D, Dormont D (1994) Similar genetic susceptibility in iatrogenic and sporadic Creutzfeldt-Jakob disease. J Gen Virol 75: 23–27
Dickinson AG (1974) Natural infection ‘spontaneous generation’ and scrapie. Nature 252: 179–180
Dickinson AG, Outram GW (1988) Genetic aspects of unconventional virus infections: the basis of the virino hypothesis. In: Bock G, Marsh J (eds) Novel infectious agents and the central nervous system. Wiley, Chichester, pp 63–83 (Ciba Symposium, no. 135)
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
Foster JD, Dickinson AG (1988a) The unusual properties of CH1641 a sheep passaged isolate of scrapie. Vet Rec 126: 5–8
Foster JD, Dickinson AG (1988b) Genetic control of scrapie in Cheviot and Suffolk sheep. Vet Rec 123: 159
Foster JD, Hunter N (1991) Partial dominance of the sA allele of theSip gene for controlling experimental scrapie. Vet Rec 128: 548–549
Foster JD, McKelvie WAC, Mylne MJA, Williams A, Hunter N, Hope J, Fraser H (1992) Studies on vertical transmission of scrapie in sheep using embryo transfer. Vet Rec 130: 341–343
Foster JD, Hope J, McConnell I, Bruce M, Fraser H (1992) Transmission of bovine spongiform encephalopathy to sheep goats and mice. Ann New York Acad Sci 724: 300–303
Foster JD, Hope J, Fraser H (1993) Transmission of bovine spongiform encephalopathy to sheep and goats. Vet Rec 133: 339–341
Foster JD, Hunter N, Williams A, Mylne MJA, McKelvey WAC, Hope J, Fraser H, Bostock C (1996) Further observations on the transmission of scrapie in experiments using embryo transfer. Vet Rec (in press)
Goldmann W, Hunter F, 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
Goldmann W, Hunter N, Benson G, Foster JD, Hope J (1991) Different scrapie associated fibril proteins (PrP) are encoded by lines of sheep selected for different alleles of theSip gene. J Gen Virol 72: 2411–2417
Goldmann W, Hunter N, Smith G, Foster JD, Hope J (1994a) PrP genotypes and theSip gene in Cheviot sheep form the basis for scrapie strain typing in sheep. Ann New York Acad Sci 724: 296–299
Goldmann W, Hunter N, Smith G, Foster J, Hope J (1994b) 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 75: 989–995
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
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: 1287–1292
Hunter N, Goldmann W, Benson G, Foster JD, Hope J (1993) Swaledale sheep affected by natural scrapie differ significantly in PrP genotype frequencies from healthy sheep and those selected for reduced incidence of scrapie. J Gen Virol 74: 1025–1031
Hunter N, Goldmann W, Smith G, Hope J (1994) The association of a codon 136 PrP gene variant with the occurrence of natural scrapie. Arch Virol 137: 171–177
Ikeda T, Horiuchi M, Ishiguro N, Muramatsu Y, Kai-Uwe GD, Shinigawa M (1995) Amino acid polymorphisms of PrP with reference to onset of scrapie in Suffolk and Corriedale sheep in Japan. J Gen virol 76: 2577–2581
Laplanche JL, Chatelain J, Westaway D, Thomas S, Dussaucy M, Brugere-Picoux J, Launay JM (1993) PrP polymorphisms associated with natural scrapie discovered by denaturing gradient gel electrophoresis. Genomics 15: 30–37
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 Ile-de-France sheep progenies. Immunogenet 27: 1–11
Parry HB (1962) Scrapie: a transmissible and hereditary disease of sheep. Heredity 17: 75–105
Stear MJ, Pokorny TS, Fryda-Bradley S, Lie O, Bull RW (1990) Genetic analysis of the antigens defined at the third internationalBola workshop. J Immunogenet 17: 21–28
Stear MJ, Bairden K, Bishop SC, Buitkamp J, Epplen JT, Gostomski D, McKellar QA, Schwaiger F-W, Wallace DS (1996) An ovine lymphocyte antigen is associated with reduced faecal egg counts in four month old lambs following natural, predominantlyOstertagia circumcincta infection. Int J Parasitol (in press)
Wakelin D, Blackwell JM (eds) 1988 Genetics of resistance of bacterial and parasitic infection. Taylor & Francis, London
Westaway D, Zuliani V, Mirenda Cooper C, Da Costa M, Neuman S, Jenny AL, Detwiler L, Prusiner SB (1994) Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. Genes Dev 8: 959–969
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Hunter, N., Foster, J.D., Goldmann, W. et al. Natural scrapie in a closed flock of Cheviot sheep occurs only in specific PrP genotypes. Archives of Virology 141, 809–824 (1996). https://doi.org/10.1007/BF01718157
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DOI: https://doi.org/10.1007/BF01718157