| | |  | | | | Record from Web of Science® | | | | | | |
| MOLECULAR-BIOLOGY OF PRION DISEASES |
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| Author(s): PRUSINER SB |
| Source: SCIENCE Volume: 252 Issue: 5012 Pages: 1515-1522 Published: JUN 14 1991 |
| Times Cited: 1,204 References: 194 |
| Abstract: Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann-Straussler-Scheinker diseases of humans. Infectious prion particles are composed largely, if not entirely, of an abnormal isoform of the prion protein, which is encoded by a chromosomal gene. A posttranslational process, as yet unidentified, converts the cellular prion protein into an abnormal isoform. Scrapie incubation times, neuropathology, and prion synthesis in transgenic mice are controlled by the prion protein gene. Point mutations in the prion protein genes of animals and humans are genetically linked to development of neurodegeneration. Transgenic mice expressing mutant prion proteins spontaneously develop neurologic dysfunction and spongiform neuropathology. Understanding prion diseases may advance investigations of other neurodegenerative disorders and of the processes by which neurons differentiate, function for decades, and then grow senescent. |
| Document Type: Article |
| Language: English |
| Reprint Address: PRUSINER, SB (reprint author), UNIV CALIF SAN FRANCISCO, DEPT NEUROL, SAN FRANCISCO, CA 94143 USA |
Addresses:
1. UNIV CALIF SAN FRANCISCO, DEPT BIOCHEM & BIOPHYS, SAN FRANCISCO, CA 94143 USA |
| Publisher: AMER ASSOC ADVANCEMENT SCIENCE, 1200 NEW YORK AVE, NW, WASHINGTON, DC 20005 |
| Subject Category: Multidisciplinary Sciences |
| IDS Number: FR040 |
| ISSN: 0036-8075 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |