Humanized Knock-in Mice Expressing Chimeric Prion Protein Showed Varied Susceptibility to Different Human Prions

American Journal of Pathology

Volumn 163, Issue 6, 2003, Pages 2585-2593

  Taguchi, Yuzuru ^a   Mohri, Shirou ^b   Ironside, James W ^d   Muramoto, Tamaki ^a,c   Kitamoto, Tetsuyuki ^a  

^a TOHOKU UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^b KYUSHU UNIVERSITY   (Japan)

^c JAPAN SCIENCE AND TECHNOLOGY AGENCY   (Japan)

^d WESTERN GENERAL HOSPITAL   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

AMYLOID PROTEIN; PRION PROTEIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CARBOXY TERMINAL SEQUENCE; CHIMERA; CONTROLLED STUDY; CREUTZFELDT JAKOB DISEASE; DISEASE COURSE; GENETIC ENGINEERING; GENETIC SUSCEPTIBILITY; HUMAN; HUMAN TISSUE; INCUBATION TIME; INFECTION RESISTANCE; INTERMETHOD COMPARISON; KNOCKOUT MOUSE; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN POLYMERIZATION; VIRUS TRANSMISSION;

EID: 0344844524     PISSN: 00029440     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0002-9440(10)63613-9     Document Type: Article

References (29)

1. Prusiner SB: The prion diseases. Brain Pathol 1998, 8:499-513
2. Mastrianni JA, Nixon R, Layzer R, Telling GC, Han D, DeArmond SJ, Prusiner SB: Prion protein conformation in a patient with sporadic fatal insomnia. N Engl J Med 1999, 340:1630-1638
3. Parchi P, Giese A, Capellari S, Brown P, Schultz-Schaeffer W, Windl O, Zerr I, Budka H, Kopp N, Piccardo P, Poser S, Rojiani A, Streichemberger N, Julein J, Vital C, Ghetti B, Gambetti P, Kretzschmar H: Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999, 46:224-233
4. Budka H, Aguzzi A, Brown P, Brucher JM, Bugiani O, Gullotta F, Haltia M, Hauw JJ, Ironside JW, Jellinger K, Kretzschmar HA, Lantos PL, Masullo C, Schlote W, Tateishi J, Weller RO: Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases). Brain Pathol 1995, 5:459-466
5. Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, Mastrianni J, Lugaresi E, Gambetti P, Prusiner SB: Evidence of the conformation of the pathogenic isoform of the prion protein enciphering and propagating prion diversity. Science 1996, 274:2079-2082
6. Parchi P, Zou W, Wang W, Brown P, Capellari S, Ghetti B, Kopp N, Schulz-Schaeffer WJ, Kretzschmar HA, Head MW, Ironside JW, Gambetti P, Chen SG: Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci USA 2000, 97:10168-10172
7. Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, Farlow M, Dickson DW, Sima AAF, Trojanowski JQ, Petersen RB, Gambetti P: Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996, 39:767-778
8. Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF: Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996, 383:685-690
9. Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, Doey L, Lantos P: The same prion strain causes vCJD and BSE. Nature 1997, 389:448-450
10. Telling GC, Scott M, Hsiao KK, Foster D, Yang S-L, Torchia M, Sidle KCL, Collinge J, DeArmond SJ, Prusiner SB: Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci USA 1994, 91:9936-9940
11. Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen F, DeArmond SJ, Prusiner SB: Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 1995, 83:79-90
12. Asante EA, Linehan JM, Desbruslais M, Joiner S, Gowland I, Wood AL, Welch J, Hill AF, Lloyd SE, Wadsworth JDF, Collinge J: BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO 2002, 21:6358-6366
13. Korth C, Kaneko K, Groth D, Heye N, Telling G, Mastrianni J, Parchi P, Gambetti P, Will R, Ironside J, Heinrich C, Tremblay P, DeArmond SJ, Prusiner SB: Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci USA 2003, 100:4784-4789
14. Kitamoto T, Mohri S, Ironside JW, Miyoshi I, Tanaka T, Kitamoto N, Itohara S, Kasai N, Katsuki M, Higuchi J, Muramoto T, Shin R-W: Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions. Biochem Biophys Res Commun 2002, 294: 280-286
15. Kitamoto T, Nakamura K, Nakao K, Shibuya S, Shin RW, Gondo Y, Katsuki M, Tateishi J: Humanized prion protein knock-in by Cre-induced site-specific recombination in the mouse. Biochem Biophys Res Commun 1996, 222:742-747
16. Shimizu S, Hoshi K, Muramoto T, Honma M, Ironside JW, Kuzuhara S, Sato T, Yamamoto T, Kitamoto T: Creutzfeldt-Jakob disease with florid-type plaques after cadaveric dura mater grafting. Arch Neurol 1999, 56:357-362
17. Ironside JW: Pathology of variant Creutzfeldt-Jakob disease. Arch Virol Suppl 16:143-151
18. Kitamoto T, Ohta M, Doh-ura K, Hitoshi Y, Terao J, Tateishi J: Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Straussler syndrome. Biochem Biophys Res Commun 1993, 191:709-714
19. Mohri S, Tateishi J: Host genetic control of incubation periods of Creutzfeldt-Jakob disease. J Gen Virol 1989, 70:1391-1400
20. Kitamoto T, Shin R-W, Doh-ura K, Tomokane N, Miyazono M, Muramoto T, Tateishi J: Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt-Jakob disease. Am J Pathol 1992, 140:1285-1294
21. Kitamoto T, Muramoto T, Hilbich C, Beyreuther K, Tateishi J: N-terminal sequence of prion protein is also integrated into kuru plaques in patients with Gerstmann-Straussler syndrome. Brain Res 1991, 545:319-321
22. Kitamoto T, Mohri S, Tateishi J: Organ distribution of proteinase-resistant prion protein in humans and mice with Creutzfeldt-Jakob disease. J Gen Virol 1989, 70:3371-3379
23. Kascsak RJ, Rubenstein R, Merz PA, Tonna-DeMasi M, Fersko R, Carp RI, Wisniewski HM, Diringer H: Mouse polyclonal and monoclonal antibody to scrapie-associated fibril protein. J Virol 1987, 61: 3688-3693
24. Kaneko K, Zulianello L, Scott M, Cooper CM, Wallace AC, James TL, Cohen FE, Prusiner SB: Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. Proc Natl Acad Sci USA 1997, 94:10069-10074
25. Flechsig E, Shmerling D, Hegyi I, Raeber AJ, Fischer M, Cozzio A, von Mering C, Aguzzi A, Weissmann C: Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice. Neuron 2000, 27:399-408
26. Caughey B, Raymond GJ, Bessen RA: Strain-dependent differences in β-sheet conformations of abnormal prion protein. J Biol Chem 1998, 273:32230-32235
27. Bessen RA, Kocisko DA, Raymond GJ, Nandan S, Lansbury PT, Caughey B: Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature 1995, 375:698-700
28. Peretz D, Williamson RA, Legname G, Matsunaga Y, Vergala J, Burton DR, DeArmond SJ, Prusiner SB, Scott MR: A change in the conformation of prions accompanies the emergence of a new prion strain. Neuron 2002, 34:921-932
29. Hill AF, Joiner S, Linehan J, Desbuslais M, Lantos PL, Collinge J: Species-barrier-independent prion replication in apparently resistant species. Proc Natl Acad Sci USA 2000, 97:10248-10253