The DOI matches its Crossref record; 103 of the 103 references it cites were found in a registry or an index.
Human prion diseases
Abstract
The prion diseases, sometimes referred to as the “transmissible spongiform encephalopathies,” include kuru, Creutzfeldt‐Jakob disease, and Gerstmann‐Sträussler‐Scheinker disease of humans as well as scrapie and bovine spongiform encephalopathy of animals. For many years, the prion diseases were thought to be caused by viruses despite intriguing evidence to the contrary. The unique characteristic common to all of these disorders, whether sporadic, dominantly inherited, or acquired by infection, is that they involve the aberrant metabolism of the prion protein (PrP). In many cases, the cellular prion protein is converted into the scrapie isoform by a posttranslational process that involves a conformational change. Often, the human prion diseases are transmissible to experimental animals and all of the inherited prion diseases segregate with PrP gene mutations.
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Citations
Cites
Novel Proteinaceous Infectious Particles Cause Scrapie
Prusiner, S. B. (1982) · Science · doi:10.1126/science.6801762 · Cited by 5,118
Self-citationThe International Classification of Sleep Disorders: Diagnostic and Coding Manual
Daroff, R. B. (1991) · Neurology · doi:10.1212/wnl.41.1.160 · Cited by 5,014
Pan, K. M., Baldwin, M., Nguyen, J., Gasset, M., Serban, A., Groth, D., Mehlhorn, I., Huang, Z., Fletterick, R. J., & Cohen, F. E. (1993) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.90.23.10962 · Cited by 2,277
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
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-citationA 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-citationNeurotoxicity of a prion protein fragment
Forloni, G., Angeretti, N., Chiesa, R., Monzani, E., Salmona, M., Bugiani, O., & Tagliavini, F. (1993) · Nature · doi:10.1038/362543a0 · Cited by 943
Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease
Palmer, M. S., Dryden, A. J., Hughes, J. T., & Collinge, J. (1991) · Nature · doi:10.1038/352340a0 · Cited by 890
Prusiner, S. B., Scott, M., Foster, D., Pan, K.-M., Groth, D., Mirenda, C., Torchia, M., Yang, S.-L., Serban, D., Carlson, G. A., et al. (1990) · Cell · doi:10.1016/0092-8674(90)90134-z · Cited by 871
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Cited by
Knopman, D. S., DeKosky, S. T., Cummings, J. L., Chui, H., Corey–Bloom, J., Relkin, N., Small, G. W., Miller, B., & Stevens, J. C. (2001) · Neurology · doi:10.1212/wnl.56.9.1143 · Cited by 1,410
Neurological Illness in Transgenic Mice Expressing a Prion Protein with an Insertional Mutation
Chiesa, R., Piccardo, P., Ghetti, B., & Harris, D. A. (1998) · Neuron · doi:10.1016/s0896-6273(00)80653-4 · Cited by 310
Burke, K. A., Yates, E. A., & Legleiter, J. (2013) · Frontiers in Neurology · doi:10.3389/fneur.2013.00017 · Cited by 127
Familial spongiform encephalopathy associated with a novel prion protein gene mutation
Nitrini, R., Rosemberg, S., Passos‐Bueno, M. R., da Silva, L. S. T., Iughetti, P., Papadopoulos, M., Carrilho, P. M., Caramelli, P., Albrecht, S., Zatz, M., et al. (1997) · Annals of Neurology · doi:10.1002/ana.410420203 · Cited by 106
Goodbrand, I. A., Ironside, J. W., Nicolson, D., & Bell, J. E. (1995) · Neuroscience Letters · doi:10.1016/0304-3940(94)11131-2 · Cited by 47
Metadata
- Authors
- Prusiner, S. B., & Hsiao, K. K.
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 1994-04
- Volume
- 35
- Issue
- 4
- Pages
- 385-395
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W2765241621
- Cited by
- 328
- References
- 103
- FWCI
- 7.97
- Type
- journal article
- Language
- en
- Open access
- closed
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
