Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis

Science

Volumn 302, Issue 5646, 2003, Pages 871-874

  Mallucci, Giovanna ^a   Dickinson, Andrew ^a   Linehan, Jacqueline ^a   Klöhn, Peter Christian ^a   Brandner, Sebastian ^a   Collinge, John ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

NEUROTOXICITY;

DISEASES; PATHOLOGY; PROTEINS; TOXICITY;

NEUROLOGY;

PRION PROTEIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN NERVE CELL; BRAIN SPONGIOSIS; CELL LOSS; CONTROLLED STUDY; DISEASE COURSE; INFECTION PREVENTION; MOUSE; NEUROTOXICITY; NONHUMAN; PRION; PRION DISEASE; PRIORITY JOURNAL; PROTEIN DEPLETION; SCRAPIE; SURVIVAL; WILD TYPE;

ANIMALS; ASTROCYTES; BRAIN; BRAIN CHEMISTRY; DISEASE PROGRESSION; HIPPOCAMPUS; MICE; MICE, TRANSGENIC; NEUROGLIA; NEURONS; PRPC PROTEINS; PRPSC PROTEINS; RECOMBINATION, GENETIC; SCRAPIE; TRANSGENES;

ANIMALIA;

EID: 0242363656     PISSN: 00368075     EISSN: None     Source Type: Journal    
DOI: 10.1126/science.1090187     Document Type: Article

References (26)

1. H. Bueler et al., Cell 73, 1339 (1993).
2. J. C. Manson, A. R. Clarke, P. A. McBride, I. McConnell, J. Hope, Neurodegeneration 3, 331 (1994).
3. M. Fischer et al., EMBO J. 15, 1255 (1996).
4. A. F. Hill et al., Proc. Natl. Acad. Sci. U.S.A. 97, 10248 (2000).
5. R. Medori et al., in Prion Diseases of Humans and Animals, S. B. Prusiner, J. Collinge, J. Powell, B. Anderton, Eds. (Ellis Horwood, London, 1992), chap. 18.
6. J. Collinge et al., Lancet 346, 569 (1995).
7. S. Brandner et al., Nature 379, 339 (1996).
8. A. Aguzzi, M. Glatzel, F. Montrasio, M. Prinz, F. L. Heppner, Nature Rev. Neurosci. 2, 745 (2001).
9. F. Tagliavini et al., Science 276, 1119 (1997).
10. S. J. Collins et al., Ann. Neurol. 52, 503 (2002).
11. S. A. Priola, A. Raines, W. S. Caughey, Science 287, 1503 (2000).
12. A. Sailer, H. Bueler, M. Fischer, A. Aguzzi, C. Weissmann, Cell 77, 967 (1994).
13. G. R. Mallucci et al., EMBO J. 21, 202 (2002).
14. P. Tremblay et al., Proc. Natl. Acad. Sci. U.S.A. 95, 12580 (1998).
15. B. Sauer, N. Henderson, Proc. Natl. Acad. Sci. U.S.A. 85, 5166 (1988).
16. J. P. Julien et al., Gene 68, 307 (1988).
17. Materials and methods are available as supporting material on Science Online.
18. J. Safar et al., Nature Med. 4, 1157 (1998).
19. This step was done after further appropriate Home Office License approval was obtained.
20. C. A. Baker, Z. Y. Lu, I. Zaitsev, L. Manuelidis, J. Virol. 73, 5089 (1999).
21. A. J. Raeber et al., EMBO J. 16, 6057 (1997).
22. M. F. Goelz et al., Lab. Anim. Sci. 48, 34 (1998).
23. c expression under its own promoter. Further, 263K scrapie used to infect the GFAP-PrP mice produces very little neuronal loss, in contrast to the severe neurodegeneration induced by RML infection of MloxP mice.
24. C. A. Baker, D. Martin, L. Manuelidis, J. Virol. 76, 10905 (2002).
25. E. A. Asante et al., EMBO J. 21, 6358 (2002).
26. We thank P. Hudson, C. O'Malley, J. Beake, D. Moore, H. Westby, and R. Ahmet for technical assistance and R. Young for help with graphics. This work was funded by the Medical Research Council, UK.