NR AQUT
AU Legname,G.; Baskakov,I.V.; Nguyen,H.O.B.; Riesner,D.; Cohen,F.E.; DeArmond,S.J.; Prusiner,S.B.
TI Synthetic mammalian prions
QU Science 2004 Jul 30; 305(5684): 673-6
KI Science. 2004 Jul 30;305(5684):589. PMID: 15286333
PT journal article
AB Recombinant mouse prion protein (recMoPrP) produced in Escherichia coli was polymerized into amyloid fibrils that represent a subset of beta sheet-rich structures. Fibrils consisting of recMoPrP(89-230) were inoculated intracerebrally into transgenic (Tg) mice expressing MoPrP(89-231). The mice developed neurologic dysfunction between 380 and 660 days after inoculation. Brain extracts showed protease-resistant PrP by Western blotting; these extracts transmitted disease to wild-type FVB mice and Tg mice overexpressing PrP, with incubation times of 150 and 90 days, respectively. Neuropathological findings suggest that a novel prion strain was created. Our results provide compelling evidence that prions are infectious proteins.
MH Amyloid/chemistry/metabolism; Animals; Biopolymers; Brain/metabolism/pathology; Brain Chemistry; Escherichia coli/genetics; Female; Glycosylation; Male; Mice; Mice, Transgenic; PrPsc Proteins/analysis/metabolism; Prion Diseases/*etiology/pathology/transmission; Prions/administration & dosage/biosynthesis/chemistry/*pathogenicity; Protein Conformation; Protein Folding; Recombinant Proteins/administration & dosage/biosynthesis/chemistry; Senile Plaques/pathology; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.; Time Factors; Tissue Extracts/administration & dosage; Vacuoles/pathology
AD Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA
SP englisch
PO USA
EA pdf-Datei und Supplement