• Pawel P. Liberski
Part of the Archives of Virology book series (ARCHIVES SUPPL, volume 6)


Since the time of submission of this supplement, no major breakthrough has been achieved but the most important data will be reviewed here. The major leitmotiv of the current scrapie research, the irreconcilable views of scrapie agent as “prion” [14] or a “virus” [2], is still at work. Neither formal paper on a positive transmission from mice with spontaneous neurodegenerative disorder (see paragraph: 2.3.8) nor results of mice without PrP (see paragraph: 2.3.8) inoculated with scrapie virus have been published. It was, however, reported that amphotericin B delayed the onset of clinical signs and Prpsc accumulation in hamster brains but not replication of the 263K strain of scrapie virus [19]. Such a clear dissociation of infectivity titer and Prpsc level may indicate the Prpsc is not a part of the virus but, on the same token, it may be more important than replication by itself for the development of disease. Using transgenic mice harboring hamster Prn-p gene (see: paragraph 2.4.2), Hecker et al. [6] reported total block of replication of the murine isolate of scrapie by the 263K (Sc237) strain (hamster) of scrapie virus. In contrast, there was no competition between two hamster strains of scrapie virus inoculated into Syrian hamsters, despite clear differences of incubation period, neuropathology and PrPsc distribution between them. Furthermore, the sequence of PrPsc appearance in different brain regions seems to followed neuroanatomical connection between them [6]. Scott et al. [16] arrived at the same conclusions using different experimental approach (see: paragraph 3.7) and provided some evidence that neuron-to-neuron spread of the virus is highly restricted by the function of gene Sinc (or Prn-p). Furthermore, the transport of PrPsc along the white matter tracts was recently reported [18].


Prion Protein Fatal Familial Insomnia Prion Protein Gene Scrapie Agent Scrapie Prion 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Borchelt D, Taraboulos A, Prusiner SB (1992) Evidence for synthesis of scrapie prion proteins in the endocytic pathway. J Biol Chem 267: 16188–16199PubMedGoogle Scholar
  2. 2.
    Chesebro B (1992) PrP and the scrapie agent. Nature 356: 560PubMedCrossRefGoogle Scholar
  3. 3.
    Dlouhy SR, Hsiao K, Foroud T, Conneally MP, Prusiner SB, Hodes MB, Ghett B (1992) Linkage of the Indiana kindred of Gerstmann-Straussler-Scheinker disease to the prion protein gene. Nature Genetics 1: 64–67PubMedCrossRefGoogle Scholar
  4. 4.
    Fink JK, Warren JT, Drury I, Murman D, Peacock BA (1992) Allele-specific sequencing confirms novel prion gene polymorphism in Creutzfeldt-Jakob disease. Neurology 41: 1647–1650Google Scholar
  5. 5.
    Foster JD, McKelvey WAC, Mylne MJA, Williams A, Hunter N, Hope K, Fraser H (1992) Studies on maternal transmission of scrapie in sheep by embryo transfer. Vet Rec 130: 341–343PubMedCrossRefGoogle Scholar
  6. 6.
    Hecker R, Taraboulos A, Scott M, Pan K-M, Yang S-L, Torchia M, Jendroska K, DeArmond SJ, Prusiner SB (1992) Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. Genes Develop 6: 1213–1228PubMedCrossRefGoogle Scholar
  7. 7.
    Kitamoto T, Shin R-W, Dohura K, Tomokane M, Miyazano M, Muramoto T, Tateishi J (1992) Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt-Jakob disease. Am J Pathol 140: 1285–1294PubMedGoogle Scholar
  8. 8.
    McKinley MP, Taraboulos A, Kenaga L, Serban D, Stieber A, DeArmond SJ, Prusiner SB, Gonatas N (1991) Ultrastructural localization of scrapie prion proteis in cytoplasmic vesicles of infected cultured cells. Lab Invest 65: 622–628PubMedGoogle Scholar
  9. 9.
    Medori R, Tritschler H-J, LeBlanc A, Villare F, Manetto V, Chen HY, Xue R, Leal S, Montagna P, Cortelli P, Tinuper P, Avoni P, Mochi M, Baruzzi A, Hauw JJ, Ott J, Lugaresi L, Autilio-Gambetti L, Gambetti P (1992) Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. New Eng J Med 326: 444–449PubMedCrossRefGoogle Scholar
  10. 10.
    Meiner Z, Halimi M, Polakiewicz RD, Prusiner SB, Gabizon R (1992) Presence of prion protein in peripheral tissues of Libyan Jews with Creutzfeldt-Jakob disease. Neurology 42: 1355–1360PubMedGoogle Scholar
  11. 11.
    Miyazono M, Iwaki T, Kitamoto T, Kaneko Y, Dohura K, Tateishi J (1992) A comparative immunohistochemical study of kuru and senile plaques with a special reference to glial reaction at various stages of amyloid plaque formation. Am J Pathol 139: 589–598Google Scholar
  12. 12.
    Miyozano M, Kitamoto T, Iwaki T, Tatieshi J (1992) Colocalization of prion protein and beta protein in the same amyloid plaques in patients with Gerstmann- Straussler-Scheinker syndrome. Acta Neuropathol (Berl) 83: 333–339CrossRefGoogle Scholar
  13. 13.
    Miyozano M, Kitamoto T, Doh-Ura K, Iwaki T, Tatieshi J (1992) Creutzfeldt- Jakob disease with codon 129 polymorphism (Valine): a comparative study of patients with codon 102 point mutation or without mutations. Acta Neuropathol (Berl) 84: 349–354Google Scholar
  14. 14.
    Prusiner SB (1992) Molecular biological studies of prion disorders in humans and animals. In: Roos RP (ed) Molecular Neurovirology. Humana Press Inc, Totawa, pp 473–501CrossRefGoogle Scholar
  15. 15.
    Renkawek K, de Jong WW, Merck KB, Frenken CWGM, van Workum EPA, Bosman GJCG (1992) Alpha beta-crystallin is present in reactive glia in Creutzfeldt- Jakon disease. Acta Neuropathol (Berl) 83: 324–327Google Scholar
  16. 16.
    Scott JR, Fraser H (1992) Scrapie in the central nervous system: neuranatomical spread of infection and Sine control of pathogenesis. J Gen Virol 73: 1637–1644PubMedCrossRefGoogle Scholar
  17. 17.
    Taraboulos A, Raeber AJ, Borchelt DR, Serban D, Prusiner SB (1992) Synthesis and tracfficking of prion proteins in cultures cells. Mol Cell Biol 3: 851–863Google Scholar
  18. 18.
    Taraboulos A, Jendroska K, Serban d, Yang S-L, DeArmond SJ, Prusiner SB (1992) Regional mapping of prion proteins in brain. Proc Natl Acad Sci USA 89: 7620–7624Google Scholar
  19. 19.
    Xi YG, Infrosso L, Ladogana A, Masullo C, Pocchiari M (1992) Amphotericin B treatment dissociates in vitro replication of the scrapie agent from PrP accumulation. Nature 356: 598–601PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag/Wien 1993

Authors and Affiliations

  • Pawel P. Liberski
    • 1
  1. 1.Electron Microscopic Laboratory, Department of OncologyMedical Academy LodzŁodzPoland

Personalised recommendations