Literature DB >> 2574076

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques.

M Scott1, D Foster, C Mirenda, D Serban, F Coufal, M Wälchli, M Torchia, D Groth, G Carlson, S J DeArmond, D Westaway, S B Prusiner.   

Abstract

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.

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Year:  1989        PMID: 2574076     DOI: 10.1016/0092-8674(89)90608-9

Source DB:  PubMed          Journal:  Cell        ISSN: 0092-8674            Impact factor:   41.582


  204 in total

1.  Species-independent inhibition of abnormal prion protein (PrP) formation by a peptide containing a conserved PrP sequence.

Authors:  J Chabry; S A Priola; K Wehrly; J Nishio; J Hope; B Chesebro
Journal:  J Virol       Date:  1999-08       Impact factor: 5.103

2.  Affinity-tagged miniprion derivatives spontaneously adopt protease-resistant conformations.

Authors:  S Supattapone; H O Nguyen; T Muramoto; F E Cohen; S J DeArmond; S B Prusiner; M Scott
Journal:  J Virol       Date:  2000-12       Impact factor: 5.103

3.  Glycosylation influences cross-species formation of protease-resistant prion protein.

Authors:  S A Priola; V A Lawson
Journal:  EMBO J       Date:  2001-12-03       Impact factor: 11.598

4.  Efficient conversion of normal prion protein (PrP) by abnormal hamster PrP is determined by homology at amino acid residue 155.

Authors:  S A Priola; J Chabry; K Chan
Journal:  J Virol       Date:  2001-05       Impact factor: 5.103

Review 5.  Transgenesis applied to transmissible spongiform encephalopathies.

Authors:  Jean-Luc Vilotte; Hubert Laude
Journal:  Transgenic Res       Date:  2002-12       Impact factor: 2.788

6.  Regional mapping of prion proteins in brain.

Authors:  A Taraboulos; K Jendroska; D Serban; S L Yang; S J DeArmond; S B Prusiner
Journal:  Proc Natl Acad Sci U S A       Date:  1992-08-15       Impact factor: 11.205

7.  A specific RFLP type associated with the occurrence of sheep scrapie in Japan.

Authors:  Y Muramatsu; K Tanaka; M Horiuchi; N Ishiguro; M Shinagawa; T Matsui; T Onodera
Journal:  Arch Virol       Date:  1992       Impact factor: 2.574

8.  In vivo reduction of amyloid-beta by a mutant copper transporter.

Authors:  Amie L Phinney; Bettina Drisaldi; Stephen D Schmidt; Stan Lugowski; Veronica Coronado; Yan Liang; Patrick Horne; Jing Yang; Joannis Sekoulidis; Janaky Coomaraswamy; M Azhar Chishti; Diane W Cox; Paul M Mathews; Ralph A Nixon; George A Carlson; Peter St George-Hyslop; David Westaway
Journal:  Proc Natl Acad Sci U S A       Date:  2003-11-14       Impact factor: 11.205

Review 9.  Insights into Mechanisms of Transmission and Pathogenesis from Transgenic Mouse Models of Prion Diseases.

Authors:  Julie A Moreno; Glenn C Telling
Journal:  Methods Mol Biol       Date:  2017

Review 10.  Prion diseases and their biochemical mechanisms.

Authors:  Nathan J Cobb; Witold K Surewicz
Journal:  Biochemistry       Date:  2009-03-31       Impact factor: 3.162

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