The DOI matches its Crossref record; 39 of the 39 references it cites were found in a registry or an index.
Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies.
Open accessAbstract
Mice, homozygous for prion protein (PrP) gene ablation (Prn-p0/0), develop normally and remain well > 500 days after inoculation with murine scrapie prions. In contrast, wild-type mice developed scrapie < 165 days after inoculation and most Prn-p0/+ mice, heterozygous for disruption of the PrP gene, exhibited signs of central nervous system dysfunction between 400 and 465 days after inoculation. In situ immunoblots showed widespread deposition of scrapie PrP (PrPSc) in the brains of both wild-type Prn-p+/+ and Prn-p0/+ mice, while neither cellular PrP (PrPC) nor PrPSc was detected in the brains of Prn-p0/0 mice. In contrast to Prn-p+/+ and Prn-p0/+ mice, Prn-p0/0 mice failed to propagate prion infectivity as measured by bioassays. Syrian hamster (SHa) PrP transgenes rendered Prn-p0/0 mice susceptible to prions containing SHaPrPSc. Immunization of Prn-p0/0 mice with purified, infectious mouse or SHa prions dispersed in Freund's adjuvant produced antisera that bound mouse, SHa, and human PrP on Western blots. Presumably, the lack of PrPC expression in Prn-p0/0 mice prevents them from becoming tolerant to the immunogen. The resistance of Prn-p0/0 mice to developing scrapie after inoculation with murine prions supports the hypothesis that PrPSc is essential for both transmission and pathogenesis of the prion diseases.
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Citations
Cites
Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4
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Mice devoid of PrP are resistant to scrapie
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Molecular Biology of Prion Diseases
Prusiner, S. B. (1991) · Science · doi:10.1126/science.1675487 · Cited by 1,988
Self-citationNormal development and behaviour of mice lacking the neuronal cell-surface PrP protein
Büeler, H., Fischer, M., Lang, Y., Bluethmann, H., Lipp, H.-P., DeArmond, S. J., Prusiner, S. B., Aguet, M., & Weissmann, C. (1992) · Nature · doi:10.1038/356577a0 · Cited by 1,655
Self-citationThe Wnt-1 (int-1) proto-oncogene is required for development of a large region of the mouse brain
McMahon, A. P., & Bradley, A. (1990) · Cell · doi:10.1016/0092-8674(90)90385-r · Cited by 1,520
A cellular gene encodes scrapie PrP 27-30 protein
Oesch, B., Westaway, D., Wälchli, M., McKinley, M. P., Kent, S. B. H., Aebersold, R., Barry, R. A., Tempst, P., Teplow, D. B., Hood, L. E., et al. (1985) · Cell · doi:10.1016/0092-8674(85)90333-2 · Cited by 1,513
Self-citationScrapie prions aggregate to form amyloid-like birefringent rods
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Self-citationMouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins
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Cited by
Prusiner, S. B. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.23.13363 · Cited by 5,373
Self-citationBessen, R. A., & Marsh, R. F. (1994) · Journal of Virology · doi:10.1128/jvi.68.12.7859-7868.1994 · Cited by 665
Physiology of the prion protein
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Taraboulos, A. (1995) · The Journal of Cell Biology · doi:10.1083/jcb.129.1.121 · Cited by 543
PATHOLOGIC CONFORMATIONS OF PRION PROTEINS
Cohen, F. E., & Prusiner, S. B. (1998) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.67.1.793 · Cited by 533
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Metadata
- Authors
- Prusiner, S. B., Groth, D., Serban, A., Koehler, R., Foster, D., Torchia, M., Burton, D., Yang, S. L., & DeArmond, S. J.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 1993-11-15
- Volume
- 90
- Issue
- 22
- Pages
- 10608-10612
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2044004977
- Cited by
- 473
- References
- 39
- FWCI
- 7.24
- Type
- journal article
- Language
- en
- Open access
- green
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- Prusiner, S. B., Groth, D., Serban, A., Koehler, R., Foster, D., Torchia, M., Burton, D., Yang, S. L., & DeArmond, S. J. (1993). Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.90.22.10608
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