The DOI matches its Crossref record; 20 of the 20 references it cites were found in a registry or an index.
Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis
Abstract
The mechanisms involved in prion neurotoxicity are unclear, and therapies preventing accumulation of PrP Sc , the disease-associated form of prion protein (PrP), do not significantly prolong survival in mice with central nervous system prion infection. We found that depleting endogenous neuronal PrP c in mice with established neuroinvasive prion infection reversed early spongiform change and prevented neuronal loss and progression to clinical disease. This occurred despite the accumulation of extraneuronal PrP Sc to levels seen in terminally ill wild-type animals. Thus, the propagation of nonneuronal PrP Sc is not pathogenic, but arresting the continued conversion of PrP c to PrP Sc within neurons during scrapie infection prevents prion neurotoxicity.
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Cites
Mice devoid of PrP are resistant to scrapie
Büeler, H., Aguzzi, A., Sailer, A., Greiner, R.-A., Autenried, P., Aguet, M., & Weissmann, C. (1993) · Cell · doi:10.1016/0092-8674(93)90360-3 · Cited by 2,100
Eight prion strains have PrPSc molecules with different conformations
Safar, J., Wille, H., Itri, V., Groth, D., Serban, H., Torchia, M., Cohen, F. E., & Prusiner, S. B. (1998) · Nature Medicine · doi:10.1038/2654 · Cited by 1,245
Site-specific DNA recombination in mammalian cells by the Cre recombinase of bacteriophage P1.
Sauer, B., & Henderson, N. (1988) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.85.14.5166 · Cited by 1,213
Fischer, M., Rülicke, T., Raeber, A., Sailer, A., Moser, M., Oesch, B., Brandner, S., Aguzzi, A., & Weissmann, C. (1996) · The EMBO Journal · doi:10.1002/j.1460-2075.1996.tb00467.x · Cited by 877
Self-citationNormal host prion protein necessary for scrapie-induced neurotoxicity
Brandner, S., Isenmann, S., Raeber, A., Fischer, M., Sailer, A., Kobayashi, Y., Marino, S., Weissmann, C., & Aguzzi, A. (1996) · Nature · doi:10.1038/379339a0 · Cited by 810
Self-citationMallucci, G. R., Ratté, S., Asante, E. A., Linehan, J., Gowland, I., Jefferys, J. G. R., & Collinge, J. (2002) · The EMBO Journal · doi:10.1093/emboj/21.3.202 · Cited by 393
Self-citationAsante, E. A. (2002) · The EMBO Journal · doi:10.1093/emboj/cdf653 · Cited by 357
Porphyrin and Phthalocyanine Antiscrapie Compounds
Priola, S. A., Raines, A., & Caughey, W. S. (2000) · Science · doi:10.1126/science.287.5457.1503 · Cited by 302
Species-barrier-independent prion replication in apparently resistant species
Hill, A. F., Joiner, S., Linehan, J., Desbruslais, M., Lantos, P. L., & Collinge, J. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.97.18.10248 · Cited by 295
Self-citationNo propagation of prions in mice devoid of PrP
Sailer, A. (1994) · Cell · doi:10.1016/0092-8674(94)90436-7 · Cited by 285
Cited by
Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
Ilieva, H., Polymenidou, M., & Cleveland, D. W. (2009) · Journal of Cell Biology · doi:10.1083/jcb.200908164 · Cited by 1,008
Physiology of the prion protein
Linden, R., Martins, V. R., Prado, M. A. M., Cammarota, M., Izquierdo, I., & Brentani, R. R. (2008) · Physiological Reviews · doi:10.1152/physrev.00007.2007 · Cited by 617
Moreno, J. A., Halliday, M., Molloy, C., Radford, H., Verity, N., Axten, J. M., Ortori, C. A., Willis, A. E., Fischer, P. M., Barrett, D. A., et al. (2013) · Science Translational Medicine · doi:10.1126/scitranslmed.3006767 · Cited by 589
Self-citationPrions as adaptive conduits of memory and inheritance
Shorter, J., & Lindquist, S. (2005) · Nature Reviews Genetics · doi:10.1038/nrg1616 · Cited by 555
Quantifying prion disease penetrance using large population control cohorts
Minikel, E. V., Vallabh, S. M., Lek, M., Estrada, K., Samocha, K. E., Sathirapongsasuti, J. F., McLean, C. Y., Tung, J. Y., Yu, L. P. C., Gambetti, P., et al. (2016) · Science Translational Medicine · doi:10.1126/scitranslmed.aad5169 · Cited by 442
Metadata
- Authors
- Mallucci, G., Dickinson, A., Linehan, J., Klöhn, P.-C., Brandner, S., & Collinge, J.
- Venue
- Science
- Published
- 2003-10-31
- Volume
- 302
- Issue
- 5646
- Pages
- 871-874
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2000966016
- Cited by
- 770
- References
- 20
- FWCI
- 14.48
- Type
- journal article
- Language
- en
- Open access
- closed
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- Mallucci, G., Dickinson, A., Linehan, J., Klöhn, P.-C., Brandner, S., & Collinge, J. (2003). Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis. Science. https://doi.org/10.1126/science.1090187
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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