The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
GENETICS OF PRIONS
Abstract
▪ Abstract Prions are unprecedented infectious pathogens that cause a group of invariably fatal, neurodegenerative diseases by an entirely novel mechanism. Prion diseases may present as genetic, infectious, or sporadic disorders, all of which involve modification of the prion protein (PrP). The human prion disease Creutzfeldt-Jakob disease (CJD) generally presents as a progressive dementia, whereas scrapie of sheep and bovine spongiform encephalopathy (BSE) are manifest as ataxic illnesses. Prions are devoid of nucleic acid and seem to be composed exclusively of a modified isoform of PrP designated PrP Sc. The normal, cellular PrP designated PrP C is converted into PrP Sc through a process whereby some of its α-helical structure is converted into β-sheet. The species of a particular prion is encoded by the sequence of the chromosomal PrP gene of the mammals in which it last replicated. In contrast to pathogens with a nucleic acid genome, prions encipher strain-specific properties in the tertiary structure of PrP Sc. Transgenetic studies argue that PrP Sc acts as a template upon which PrP C is refolded into a nascent PrP Sc molecule through a process facilitated by another protein.
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Citations
Cites
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
Collinge, J., Sidle, K. C. L., Meads, J., Ironside, J., & Hill, A. F. (1996) · Nature · doi:10.1038/383685a0 · Cited by 1,735
Cited by
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
Self-citationIon mobility–mass spectrometry: a new paradigm for proteomics
McLean, J. A., Ruotolo, B. T., Gillig, K. J., & Russell, D. H. (2005) · International Journal of Mass Spectrometry · doi:10.1016/j.ijms.2004.10.003 · Cited by 309
Deletion of the α(1,3)galactosyl transferase (GGTA1) gene and the prion protein (PrP) gene in sheep
Denning, C., Burl, S., Ainslie, A., Bracken, J., Dinnyes, A., Fletcher, J., King, T., Ritchie, M., Ritchie, W. A., Rollo, M., et al. (2001) · Nature Biotechnology · doi:10.1038/89313 · Cited by 273
Metadata
- Authors
- Prusiner, S. B., & Scott, M. R.
- Venue
- Annual Review of Genetics
- Publisher
- Annual Reviews
- Published
- 1997-12
- Volume
- 31
- Issue
- 1
- Pages
- 139-175
- ISSN
- 0066-4197 (ISSN-L)
- 1545-2948
- OpenAlex
- W2049906745
- Cited by
- 158
- References
- 1
- FWCI
- 0.50
- Type
- journal article
- Language
- en
- Open access
- closed
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- Prusiner, S. B., & Scott, M. R. (1997). GENETICS OF PRIONS. Annual Review of Genetics. https://doi.org/10.1146/annurev.genet.31.1.139
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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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