NR AKQE
AU Scott,M.R.D.; Foster,D.B.; Mirenda,C.; Serban,D.; Coufal,F.; Walchli,M.; Torchia,M.; Groth,D.; Carlson,G.A.; DeArmond,S.J.; Westaway,D.; Prusiner,S.B.
TI Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques
QU Cell 1989 Dec 1; 59(5): 847-57
PT journal article
AB Three transgenic mouse lines designated Tg 69, 71, and 81 were produced harboring a Syrian hamster (Ha) prion protein (PrP) gene; all expressed the cellular HaPrP isoform in their brains. Inoculation of Tg 81 mice or hamsters with Ha prions caused scrapie in integral of 75 days; nontransgenic control mice failed to develop scrapie after greater than 500 days. Tg 71 mice inoculated with Ha prions developed scrapie in integral of 170 days. Both Tg 71 and Tg 81 mice exhibited spongiform degeneration and reactive astrocytic gliosis, and they produced the scrapie HaPrP isoform in their brains. Tg 81 brains also showed HaPrP amyloid plaques characteristic of Ha scrapie and contained integral of 10(9) ID50 units of Ha prions based on Ha bioassays. Our findings argue that the PrP gene modulates scrapie susceptibility, incubation times, and neuropathology; furthermore, they demonstrate synthesis of infectious scrapie prions programmed by a recombinant DNA molecule.
IN Drei transgene Mauslinien mit einem zusätzlichen Hamsterprionproteingen produzieren auch Hamsterprionproteine. Im Gegensatz zu normalen Mäusen lassen sie sich leicht mit Hamsterprionen infizieren. Die neu gebildeten Prione scheinen Hamsterprione zu sein.
MH Amyloid/*biosynthesis; Animal; Base Sequence; Brain/*metabolism/pathology; Cloning, Molecular; Cosmids; Genes, Structural, Viral; Hamsters; Mesocricetus; Mice; *Mice, Transgenic; Molecular Sequence Data; Oligonucleotide Probes; PrPsc Proteins; Prions/*genetics; Restriction Mapping; Scrapie/*genetics/pathology; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.; Viral Proteins/analysis/*genetics/isolation & purification
AD Stephen J. DeArmond, Dallas Foster, Darlene Groth, Carol Mirenda, Stanley B. Prusiner, Michael Scott, Dan Serban, Marilyn Torchia, Monika Wälchli, David Westaway, Department of Neurology University of California, San Francisco, California 94143, USA; Stanley B. Prusiner, Stanley B. Prusiner, Department of Biochemistry and Biophysics University of California, San Francisco, California 94143, USA; Frank Coufal, Stephen J. DeArmond, Department of Pathology University of California, San Francisco, California 94143, USA; George Carlson, McLaughlin Research Institute, Great Falls, Montana 59401, USA
SP englisch
PO USA
OR Prion-Krankheiten 7