The DOI matches its Crossref record.
A Transmembrane Form of the Prion Protein in Neurodegenerative Disease
Abstract
At the endoplasmic reticulum membrane, the prion protein (PrP) can be synthesized in several topological forms. The role of these different forms was explored with transgenic mice expressing PrP mutations that alter the relative ratios of the topological forms. Expression of a particular transmembrane form (termed Ctm PrP) produced neurodegenerative changes in mice similar to those of some genetic prion diseases. Brains from these mice contained Ctm PrP but not PrP Sc , the PrP isoform responsible for transmission of prion diseases. Furthermore, in one heritable prion disease of humans, brain tissue contained Ctm PrP but not PrP Sc . Thus, aberrant regulation of protein biogenesis and topology at the endoplasmic reticulum can result in neurodegeneration.
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Citations
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-citationPhysiology 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
von Heijne, G. (2006) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm2063 · Cited by 538
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-citationUbiquitin, cellular inclusions and their role in neurodegeneration
Alves-Rodrigues, A., Gregori, L., & Figueiredo-Pereira, M. E. (1998) · Trends in Neurosciences · doi:10.1016/s0166-2236(98)01276-4 · Cited by 393
Metadata
- Authors
- Hegde, R. S., Mastrianni, J. A., Scott, M. R., DeFea, K. A., Tremblay, P., Torchia, M., DeArmond, S. J., Prusiner, S. B., & Lingappa, V. R.
- Venue
- Science
- Published
- 1998-02-06
- Volume
- 279
- Issue
- 5352
- Pages
- 827-834
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2020258831
- Cited by
- 729
- References
- 0
- FWCI
- 14.18
- Type
- journal article
- Language
- en
- Open access
- closed
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- Hegde, R. S., Mastrianni, J. A., Scott, M. R., DeFea, K. A., Tremblay, P., Torchia, M., DeArmond, S. J., Prusiner, S. B., & Lingappa, V. R. (1998). A Transmembrane Form of the Prion Protein in Neurodegenerative Disease. Science. https://doi.org/10.1126/science.279.5352.827
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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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