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volume 34 issue 1 (march 2013) : 79-85

SCRAPIE: A NEURO DEGENERATIVE DISEASE IN SHEEP - A REVIEW

S
Sharda Choudhary*
M
Madhu Choudhary
1National Rasearch Centre on Seed Spices, Ajmer- 305 206, India

  • Submitted|

  • First Online |

  • doi

Cite article:- Choudhary* Sharda, Choudhary Madhu (2025). SCRAPIE: A NEURO DEGENERATIVE DISEASE IN SHEEP - A REVIEW. Agricultural Reviews. 34(1): 79-85. doi: .

ABSTRACT

Scrapie is a unique disease whose etiology is through protein infection rather than a biological agent. Genetic polymorphism at 136, 154 and 171 codons provide the susceptibility/ resistance to the disease outbreak. PCR-SSCP has been found to be a robust technique for the identification of genetic variants because of SNP in the ORF (open reading frame) of PrP gene efficiently at low cost, evidenced by novel DNA sequences resulted from the SSCP variants. Prevalence of susceptible alleles at 136, 154 and 171 codons however did not result into the scrapie disease clinical symptoms in indigenous sheep breeds. Polymorphism at other loci in indigenous sheep could have played significant role in prevention of disease symptoms in indigenous sheep. The traditional feeding and management exclusively on range grazing without any concentrate supplementation having any animal protein might be responsible for the protection of animals from scrapie disease infection despite presence of susceptible alleles in the indigenous sheep animals.

REFERENCES

  1. Andréoletti O, Orge L, Benestad SL, Beringue V, Litaise C and Simon S. (2011) Atypical/Nor98 scrapie infectivity in sheep peripheral tissues. PLoS Pathog. 7:e1001285. doi:10.1371/journal.pp.100128.
  2. APHIS. (2001). APHIS Activities. Animal and Plant Health Inspection Service,United States Department of Agriculture, Washington, DC.
  3. Baylis M. and Goldmann W. (2004). The genetics of scrapie in sheep and goats. Curr. Mol. Med. 4(4): 385-396.
  4. Belt P.B.G.M., Muileman I.H., Schreuder B.E.C., Ruijter J.B., Gielkens A.L.J. and Smits M.A. (1995). J. Gen. Virol. 76: 509-517.
  5. Choudhary S, Gupta N and Gupta S C. (2012). Identification of SNPs in PRNP gene in Karnah sheep breed of India. Indian J. Anim. Scie. 82 : 609–614.
  6. Choudhary S, Gupta,N, Gaurav S S, Ahlawat SPS and Gupta S C. (2007). Single Strand Conformation polymorphism of PRNP gene exon-III region in Karnah sheep breed of India. Indian J. Anim. Scie.: 77(12):1289-92.
  7. Clark A.M. and Moar J.A.E. (1992). Scrapie: a clinical assessment. Vet. Rec. 130: 377-378.
  8. Clouscard C., Beaudry P., Elsen J.M., Milan D., Dussaucy M., Bounneau C., Schelcher F., Chatelain J., Launay J.M. and Laplanche J.L. (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.
  9. Dawson D.M. and Carter J.L. (1987). Immunosuppression in progressive multiple sclerosis with high dose intravenous cyclophosphamide and monoclonal antibodies. Riv. Neurol. 57 (2): 88-91.
  10. Dickinson A.G. (1976). Scrapie in Sheep and Goats. In: Slow Virus Diseases of Animals and Man. R.H. Kimberlin (Ed.). North-Holland Publishing Company, Amsterdam, the Netherlands. 209-241.
  11. Elsen J.M., Amigues Y., Schelcher F., Ducrocq V., Andreoletti O., Eychenne F., Tien Khang J.V., Poivey J.P., Lantier F. and Laplanche J.L. (1999a) Genetic susceptibility and transmission factors in scrapie: detailed analysis of an epidemic in a closed ûock of Romanov, Arch. Virol. 144: 431–445.
  12. Foster J.D. and Dickinson A.G. (1988). Genetic control of scrapie in Cheviot and Suffolk sheep. Vet. Rec. 123(6):159.
  13. Goldmann W., Hunter N., Smith G., Foster J. and Hope J. (1994) 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.
  14. Goldmann W. (2008) PrP genetics in ruminant transmissible spongiform encephalopathies. Vet Res. 39:30. doi:10.1051/ vetres:2008010, 1–14.
  15. Gupta N, Choudhary S, Malik G, Pandey A and Gupta S C. (2007). Prion protein gene (PRNP) polymorphism in Gaddi sheep breed of India. Full length paper in Proc. Of international Symposium on Animal Genomics for Animal Health, OIE Head quarter, Paris, Oct 23-25.
  16. Hoinville LJ. (1996) A review of the epidemiology of scrapie in sheep. Rev Sci Tech. 15:827–52.
  17. Hunter N., Goldmann W., Benson G., Foster J.D. and 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.
  18. Hunter N., Goldmann W., Smith G. and Hope J. (1994). The association of a codon 136 PrP gene variant with the occurrence of natural scrapie. Arch. Virol. 137: 171-177.
  19. Hunter N. (1996). Novel polymorphisms in the caprine PrP gene: a codon 142 mutation associated with scrapie incubation period. J. Gen. Virol. 77: 2885-2891.
  20. Hunter N. (1997). PrP genetics in sheep and the applications for scrapie and BSE. Trends in Microbiology 5: 331-334.
  21. Hunter N., Goldmann W., Foster J.D, Cairns D. and Smith G. (1997a). Natural scrapie and PrP genotype: case-control studies in British sheep. Vet. Rec. 141: 137-140.
  22. Hunter N., Moore L., Hosie B.D, Dingwall W.S. and Greig A. (1997b). Association between natural scrapie and PrP genotype in a flock of Suffolk sheep in Scotland. Vet. Rec. 140: 59-63.
  23. Ikeda T., Horiuchi M., Ishiguro N., Muramatsu Y., Kai-Uwe G. D. and Shinagawa 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.
  24. Ishiguro N., Shinagawa M., Onoe. S., Yamanouchi K. and Saito T. (1998). Rapid analysis of allelic variants of the sheep PrP gene by oligonucleotide probes. Microbiol. Immunol. 42(8): 579-582.
  25. Konold T, Bone GE, Phelan LJ, Simmons MM, González L. and Sisó S. (2010) Monitoring of clinical signs in goats with transmissible spongiform encephalopathies. BMC Vet Res. 010; 6:13. doi:10.1186/1746-6148-6-13.
  26. Laplanche J.L., Chatelain J., Westaway D., Thomas S., Dussaucy M., Brugere-Picoux J. and Launay J.M. (1993). PrP polymorphisms associated with natural scrapie discovered by denaturing gradient gel electrophoresis. Genomics 15: 30-37.
  27. McGowan J.P. (1922). Scrapie in sheep. Scott J Agric 5: 365–375.
  28. Millot P., Chatelain, J., Dautheville, C., Salmon, D. and Cathala, E. (1988). Sheep major histocompatibility (OLA) complex: linkage between a scrapie susceptibility/resistance locus and the OLA complex in Île-de France sheep progenies. Immunogenetics 27: 1-11.
  29. Mohri, S., Farquhar C.F., Somerville R.A., Jeffrey M., Foster J. and Hope J. (1992). Immunodetection of a disease specific PrP fraction in scrapie-affected sheep and BSE-affected cattle. Vet. Rec. 131(23): 537-539.
  30. Oesch B., Westaway D., Wälchli M., McKinley M., Kent S., Aebersold R., Barry R., Tempst P., Teplow D. and Hood L. (1985). A cellular gene encodes scrapie PrP 27-30 protein. Cell 40 (4): 735-46.
  31. O’Rourke N.A., Sullivan D.P., Kaznowski C.E., Jacobs A.A. and McConnell S.K. (1996) Tangential migration of neurons in the developing cerebral cortex. Development. 121: 2165–2176.
  32. Parry, H.B. (1984) Scrapie Disease in Sheep, ed. Oppenheimer, D.R. (Academic, New York), pp. 31-59.
  33. Prusiner S.B. (1982). Novel proteinaceous infectious particles cause scrapie. Science. 216 (4542). doi:10.1126/ science.278.5336.245.
  34. Tranulis M.A., Osland A., Bratberg B. and Ulvund M.J. (1999). Prion protein gene polymorphisms in sheep with natural scrapie and healthy controls in Norway. Journal of General Virology. 80: 1073-1077.
  35. Vaccari G., Conte M., Morelli L., Di G.G., Petraroli R. and Agrimi U. (2004). Primer extension assay for prion protein genotype determination in sheep. Mol. Cell Probes. 18(1): 33-37.
  36. Westaway D., DeArmond S., Cayetanocanlas J., Groth D., Foster D. and Yang S. (1994). Degeneration of Skeletal- Muscle, Peripheral-Nerves, and the Central-Nervous-System in Transgenic Mice Overexpressing Wild-Type Prion Proteins. Cell. 76 (1): 117-129.
  37. Wylie A.A., Murphy S.K., Orton T.C., and Jirtle R.L. (2000). Polar Overdominance at the Ovine callipyge Locus. Genome Res. 10: 1711–1718.
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    volume 34 issue 1 (march 2013) : 79-85

    SCRAPIE: A NEURO DEGENERATIVE DISEASE IN SHEEP - A REVIEW

    S
    Sharda Choudhary*
    M
    Madhu Choudhary
    1National Rasearch Centre on Seed Spices, Ajmer- 305 206, India

    • Submitted|

    • First Online |

    • doi

    Cite article:- Choudhary* Sharda, Choudhary Madhu (2025). SCRAPIE: A NEURO DEGENERATIVE DISEASE IN SHEEP - A REVIEW. Agricultural Reviews. 34(1): 79-85. doi: .

    ABSTRACT

    Scrapie is a unique disease whose etiology is through protein infection rather than a biological agent. Genetic polymorphism at 136, 154 and 171 codons provide the susceptibility/ resistance to the disease outbreak. PCR-SSCP has been found to be a robust technique for the identification of genetic variants because of SNP in the ORF (open reading frame) of PrP gene efficiently at low cost, evidenced by novel DNA sequences resulted from the SSCP variants. Prevalence of susceptible alleles at 136, 154 and 171 codons however did not result into the scrapie disease clinical symptoms in indigenous sheep breeds. Polymorphism at other loci in indigenous sheep could have played significant role in prevention of disease symptoms in indigenous sheep. The traditional feeding and management exclusively on range grazing without any concentrate supplementation having any animal protein might be responsible for the protection of animals from scrapie disease infection despite presence of susceptible alleles in the indigenous sheep animals.

    REFERENCES

    1. Andréoletti O, Orge L, Benestad SL, Beringue V, Litaise C and Simon S. (2011) Atypical/Nor98 scrapie infectivity in sheep peripheral tissues. PLoS Pathog. 7:e1001285. doi:10.1371/journal.pp.100128.
    2. APHIS. (2001). APHIS Activities. Animal and Plant Health Inspection Service,United States Department of Agriculture, Washington, DC.
    3. Baylis M. and Goldmann W. (2004). The genetics of scrapie in sheep and goats. Curr. Mol. Med. 4(4): 385-396.
    4. Belt P.B.G.M., Muileman I.H., Schreuder B.E.C., Ruijter J.B., Gielkens A.L.J. and Smits M.A. (1995). J. Gen. Virol. 76: 509-517.
    5. Choudhary S, Gupta N and Gupta S C. (2012). Identification of SNPs in PRNP gene in Karnah sheep breed of India. Indian J. Anim. Scie. 82 : 609–614.
    6. Choudhary S, Gupta,N, Gaurav S S, Ahlawat SPS and Gupta S C. (2007). Single Strand Conformation polymorphism of PRNP gene exon-III region in Karnah sheep breed of India. Indian J. Anim. Scie.: 77(12):1289-92.
    7. Clark A.M. and Moar J.A.E. (1992). Scrapie: a clinical assessment. Vet. Rec. 130: 377-378.
    8. Clouscard C., Beaudry P., Elsen J.M., Milan D., Dussaucy M., Bounneau C., Schelcher F., Chatelain J., Launay J.M. and Laplanche J.L. (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.
    9. Dawson D.M. and Carter J.L. (1987). Immunosuppression in progressive multiple sclerosis with high dose intravenous cyclophosphamide and monoclonal antibodies. Riv. Neurol. 57 (2): 88-91.
    10. Dickinson A.G. (1976). Scrapie in Sheep and Goats. In: Slow Virus Diseases of Animals and Man. R.H. Kimberlin (Ed.). North-Holland Publishing Company, Amsterdam, the Netherlands. 209-241.
    11. Elsen J.M., Amigues Y., Schelcher F., Ducrocq V., Andreoletti O., Eychenne F., Tien Khang J.V., Poivey J.P., Lantier F. and Laplanche J.L. (1999a) Genetic susceptibility and transmission factors in scrapie: detailed analysis of an epidemic in a closed ûock of Romanov, Arch. Virol. 144: 431–445.
    12. Foster J.D. and Dickinson A.G. (1988). Genetic control of scrapie in Cheviot and Suffolk sheep. Vet. Rec. 123(6):159.
    13. Goldmann W., Hunter N., Smith G., Foster J. and Hope J. (1994) 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.
    14. Goldmann W. (2008) PrP genetics in ruminant transmissible spongiform encephalopathies. Vet Res. 39:30. doi:10.1051/ vetres:2008010, 1–14.
    15. Gupta N, Choudhary S, Malik G, Pandey A and Gupta S C. (2007). Prion protein gene (PRNP) polymorphism in Gaddi sheep breed of India. Full length paper in Proc. Of international Symposium on Animal Genomics for Animal Health, OIE Head quarter, Paris, Oct 23-25.
    16. Hoinville LJ. (1996) A review of the epidemiology of scrapie in sheep. Rev Sci Tech. 15:827–52.
    17. Hunter N., Goldmann W., Benson G., Foster J.D. and 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.
    18. Hunter N., Goldmann W., Smith G. and Hope J. (1994). The association of a codon 136 PrP gene variant with the occurrence of natural scrapie. Arch. Virol. 137: 171-177.
    19. Hunter N. (1996). Novel polymorphisms in the caprine PrP gene: a codon 142 mutation associated with scrapie incubation period. J. Gen. Virol. 77: 2885-2891.
    20. Hunter N. (1997). PrP genetics in sheep and the applications for scrapie and BSE. Trends in Microbiology 5: 331-334.
    21. Hunter N., Goldmann W., Foster J.D, Cairns D. and Smith G. (1997a). Natural scrapie and PrP genotype: case-control studies in British sheep. Vet. Rec. 141: 137-140.
    22. Hunter N., Moore L., Hosie B.D, Dingwall W.S. and Greig A. (1997b). Association between natural scrapie and PrP genotype in a flock of Suffolk sheep in Scotland. Vet. Rec. 140: 59-63.
    23. Ikeda T., Horiuchi M., Ishiguro N., Muramatsu Y., Kai-Uwe G. D. and Shinagawa 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.
    24. Ishiguro N., Shinagawa M., Onoe. S., Yamanouchi K. and Saito T. (1998). Rapid analysis of allelic variants of the sheep PrP gene by oligonucleotide probes. Microbiol. Immunol. 42(8): 579-582.
    25. Konold T, Bone GE, Phelan LJ, Simmons MM, González L. and Sisó S. (2010) Monitoring of clinical signs in goats with transmissible spongiform encephalopathies. BMC Vet Res. 010; 6:13. doi:10.1186/1746-6148-6-13.
    26. Laplanche J.L., Chatelain J., Westaway D., Thomas S., Dussaucy M., Brugere-Picoux J. and Launay J.M. (1993). PrP polymorphisms associated with natural scrapie discovered by denaturing gradient gel electrophoresis. Genomics 15: 30-37.
    27. McGowan J.P. (1922). Scrapie in sheep. Scott J Agric 5: 365–375.
    28. Millot P., Chatelain, J., Dautheville, C., Salmon, D. and Cathala, E. (1988). Sheep major histocompatibility (OLA) complex: linkage between a scrapie susceptibility/resistance locus and the OLA complex in Île-de France sheep progenies. Immunogenetics 27: 1-11.
    29. Mohri, S., Farquhar C.F., Somerville R.A., Jeffrey M., Foster J. and Hope J. (1992). Immunodetection of a disease specific PrP fraction in scrapie-affected sheep and BSE-affected cattle. Vet. Rec. 131(23): 537-539.
    30. Oesch B., Westaway D., Wälchli M., McKinley M., Kent S., Aebersold R., Barry R., Tempst P., Teplow D. and Hood L. (1985). A cellular gene encodes scrapie PrP 27-30 protein. Cell 40 (4): 735-46.
    31. O’Rourke N.A., Sullivan D.P., Kaznowski C.E., Jacobs A.A. and McConnell S.K. (1996) Tangential migration of neurons in the developing cerebral cortex. Development. 121: 2165–2176.
    32. Parry, H.B. (1984) Scrapie Disease in Sheep, ed. Oppenheimer, D.R. (Academic, New York), pp. 31-59.
    33. Prusiner S.B. (1982). Novel proteinaceous infectious particles cause scrapie. Science. 216 (4542). doi:10.1126/ science.278.5336.245.
    34. Tranulis M.A., Osland A., Bratberg B. and Ulvund M.J. (1999). Prion protein gene polymorphisms in sheep with natural scrapie and healthy controls in Norway. Journal of General Virology. 80: 1073-1077.
    35. Vaccari G., Conte M., Morelli L., Di G.G., Petraroli R. and Agrimi U. (2004). Primer extension assay for prion protein genotype determination in sheep. Mol. Cell Probes. 18(1): 33-37.
    36. Westaway D., DeArmond S., Cayetanocanlas J., Groth D., Foster D. and Yang S. (1994). Degeneration of Skeletal- Muscle, Peripheral-Nerves, and the Central-Nervous-System in Transgenic Mice Overexpressing Wild-Type Prion Proteins. Cell. 76 (1): 117-129.
    37. Wylie A.A., Murphy S.K., Orton T.C., and Jirtle R.L. (2000). Polar Overdominance at the Ovine callipyge Locus. Genome Res. 10: 1711–1718.
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