Polymorphisms at codons 108 and 189 in murine PrP play distinct roles in the control of scrapie incubation time

Journal of General Virology

Volumn 86, Issue 3, 2005, Pages 859-868

  Barron, Rona M ^a   Baybutt, Herbert ^a   Tuzi, Nadia L ^a   McCormack, James ^a,c   King, Declan ^a   Moore, Richard C ^b,d   Melton, David W ^b   Manson, Jean C ^a  

^a INSTITUTE FOR ANIMAL HEALTH   (United Kingdom)

^b UNIVERSITY OF EDINBURGH   (United Kingdom)

^c TSE Research Unit   (United Kingdom)

^d UNIVERSITY OF EDINBURGH   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

LEUCINE; PHENYLALANINE; PRION PROTEIN; THREONINE; VALINE;

ALLELE; ARTICLE; CODON; CONTROLLED STUDY; DISEASE CONTROL; GENE EXPRESSION; GENETIC POLYMORPHISM; GENETIC VARIABILITY; HETEROZYGOSITY; INCUBATION TIME; INFECTION SENSITIVITY; INOCULATION; MOLECULAR INTERACTION; MOUSE; MOUSE STRAIN; NONHUMAN; PRION DISEASE; PRIORITY JOURNAL; PROTEIN POLYMORPHISM; PROTEIN STRUCTURE; SCRAPIE; TRANSGENIC MOUSE;

ANIMALS; CODON; DISEASE SUSCEPTIBILITY; MICE; POLYMORPHISM, GENETIC; PRIONS; SCRAPIE; SHEEP; TIME FACTORS;

MURINAE; MUS MUSCULUS; OVIS ARIES; RODENTIA;

EID: 14744302246     PISSN: 00221317     EISSN: None     Source Type: Journal    
DOI: 10.1099/vir.0.80525-0     Document Type: Article

References (42)

1. Alperovitch, A., Zerr, I., Pocchiari, M. & 7 other authors (1999). Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 353, 1673-1674.
2. Barron, R. M. & Manson, J. C. (2004). Targeting the murine PrP gene. In Prions and Prion Diseases: Current Perspectives pp. 103-119. Edited by G. C. Telling. Wymondham, UK: Horizon Bioscience.
3. Barron, R. M., Thomson, V., King, D., Shaw, J., Melton, D. W. & Manson, J. C. (2003). Transmission of murine scrapie to P101L transgenic mice. J Gen Virol 84, 3165-3172.
4. Basler, K., Oesch, B., Scott, M., Westaway, D., Wälchli, M., Groth, D. F., McKinley, M. P., Prusiner, S. B. & Weissmann, C. (1986). Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell 46, 417-428.
5. b of mouse. Eur J Biochem 267, 2452-2459.
6. Carlson, G. A., Kingsbury, D. T., Goodman, P. A., Coleman, S., Marshall, S. T., DeArmond, S., Westaway, D. & Prusiner, S. B. (1986). Linkage of prion protein and scrapie incubation time genes. Cell 46, 503-511.
7. Caughey, B. (2001). Interactions between prion protein isoforms: the kiss of death? Trends Biochem Sci 26, 235-242.
8. Chomczynski, P. & Sacchi, N. (1987). Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction. Anal Biochem 162, 156-159.
9. Dickinson, A. G., Meikle, V. M. & Fraser, H. (1968). Identification of a gene which controls the incubation period of some strains of scrapie agent in mice. J Comp Pathol 78, 293-299.
10. Fischer, M., Rülicke, T., Raeber, A., Sailer, A., Moser, M., Oesch, B., Brandner, S., Aguzzi, A. & Weissmann, C. (1996 . Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J 15, 1255-1264.
11. Flechsig, E., Shmerling, D., Hegyi, I., Raeber, A. J., Fischer, M., Cozzio, A., von Mering, C., Aguzzi, A. & Weissmann, C. (2000). Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice. Neuron 27, 399-408.
12. Fraser, H. & Dickinson, A. G. (1967). Distribution of experimentally induced scrapie lesions in the brain. Nature 216 1310-1311.
13. Goldfarb, L. G., Petersen, R. B., Tabaton, M. & 18 other authors (1992). Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism. Science 258, 806-808.
14. Goldmann, W., Hunter, N., Smith, G., Foster, J. & Hope, J. (1994). PrP genotype and agent effects in scrapie: change in allelic interaction with different isolates of agent in sheep, a natural host of scrapie. J Gen Virol 75, 989-995.
15. Gu, H., Zou, Y.-R. & Rajewsky, K. (1993). Independent control of immunoglobulin switch recombination at individual switch regions evidenced through Cre-loxP-mediated gene targeting. Cell 73, 1155-1164.
16. Horiuchi, M., Priola, S. A., Chabry, J. & Caughey, B. (2000). Interactions between heterologous forms of prion protein: binding, inhibition of conversion, and species barriers. Proc Natl Acad Sci U S A 97, 5836-5841.
17. Jansen, K., Schafer, O., Birkmann, E., Post, K., Serban, H., Prusiner, S. B. & Riesner, D. (2001). Structural intermediates in the putative pathway from the cellular prion protein to the pathogenic form. Biol Chem 382, 683-691.
18. Jarrett, J. T. & Lansbury, P. T., Jr (1993). Seeding "one-dimensional crystallization" of amyloid: a pathogenic mechanism in Alzheimer's disease and scrapie? Cell 73, 1055-1058.
19. Lakso, M., Sauer, B., Mosinger, B., Jr, Lee, E. J., Manning, R. W., Yu, S.-H., Mulder, K. L. & Westphal, H. (1992). Targeted oncogene activation by site-specific recombination in transgenic mice. Proc Natl Acad Sci U S A 89, 6232-6236.
20. Lawson, V. A., Priola, S. A., Wehrly, K. & Chesebro, B. (2001). N-terminal truncation of prion protein affects both formation and conformation of abnormal protease-resistant prion protein generated in vitro. J Biol Chem 276, 35265-35271.
21. Lawson, V. A., Priola, S. A., Meade-White, K., Lawson, M. & Chesebro, B. (2004). Flexible N-terminal region of prion protein influences conformation of protease-resistant prion protein isoforms associated with cross-species scrapie infection in vivo and in vitro. J Biol Chem 279, 13689-13695.
22. Li, R., Liu, T., Wong, B.-S. & 7 other authors (2000). Identification of an epitope in the C terminus of normal prion protein whose expression is modulated by binding events in the N terminus. J Mol Biol 301, 567-573.
23. Lloyd, S. E., Thompson, S. R., Beck, J. A., Linehan, J. M., Wadsworth, J. D. F., Brandner, S., Collinge, J. & Fisher, E. M. C. (2004). Identification and characterization of a novel mouse prion gene allele. Mamm Genome 15, 383-389.
24. Locht, C., Chesebro, B., Race, R. & Keith, J. M. (1986). Molecular cloning and complete sequence of prion protein cDNA from mouse brain infected with the scrapie agent. Proc Natl Acad Sci U S A 83, 6372-6376.
25. Sc in vivo and antagonizes prion disease. Cell 113, 49-60.
26. C) not observed with recombinant PrP. J Biol Chem 275, 38081-38087.
27. Moore, R. C. & Melton, D. W. (1995). Models of human disease through gene targeting. Biochem Soc Trans 23, 398-403.
28. Moore, R. C., Redhead, N. J., Selfridge, J., Hope, J., Manson, J. C. & Melton, D. W. (1995). Double replacement gene targeting for the production of a series of mouse strains with different prion protein gene alterations. Biotechnology (N Y) 13, 999-1004.
29. Moore, R. C., Hope, J., McBride, P. A., McConnell, I., Selfridge, J., Melton, D. W. & Manson, J. C. (1998). Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent. Nat Genet 18, 118-125.
30. Palmer, M. S., Dryden, A. J., Hughes, J. T. & Collinge, J. (1991). Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 352, 340-342.
31. Priola, S. A., Caughey, B., Wehrly, K. & Chesebro, B. (1995). A 60-kDa prion protein (PrP) with properties of both the normal and scrapie-associated forms of PrP. J Biol Chem 270, 3299-3305.
32. Prusiner, S. B. (1982). Novel proteinaceous infectious particles cause scrapie. Science 216, 136-144.
33. Prusiner, S. B. (1996). Molecular biology and pathogenesis of prion diseases. Trends Biochem Sci 21, 482-487.
34. Selfridge, J., Pow, A. M., McWhir, J., Magin, T. M. & Melton, D. W. (1992). Gene targeting using a mouse HPRT minigene/HPRT-deficient embryonic stem cell system: inactivation of the mouse ERCC-1 gene. Somat Cell Mol Genet 18, 325-336.
35. Shmerling, D., Hegyi, I., Fischer, M. & 10 other authors (1998). Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell 93, 203-214.
36. Supattapone, S., Muramoto, T., Legname, G., Mehlhorn, I., Cohen, F. E., DeArmond, S. J., Prusiner, S. B. & Scott, M. R. (2001 . Identification of two prion protein regions that modify scrapie incubation time. J Virol 75, 1408-1413.
37. Thompson, S., Clarke, A. R., Pow, A. M., Hooper, M. L. & Melton, D. W. (1989). Germ line transmission and expression of a corrected HPRT gene produced by gene targeting in embryonic stem cells. Cell 56, 313-321.
38. Warwicker, J. (1997). Species barriers in a model for specific prion protein dimerisation. Biochem Biophys Res Commun 232, 508-512.
39. Warwicker, J. (2000). Modeling a prion protein dimer: predictions for fibril formation. Biochem Biophys Res Commun 278 646-652.
40. Weissmann, C. (1996). Molecular biology of transmissible spongiform encephalopathies. FEBS Lett 389, 3-11.
41. Westaway, D., Goodman, P. A., Mirenda, C. A., McKinley, M. P., Carlson, G. A. & Prusiner, S. B. (1987). Distinct prion proteins in short and long scrapie incubation period mice. Cell 51, 651-662.
42. Zeidler, M., Stewart, G., Cousens, S. N., Estibeiro, K. & Will, R. G. (1997). Codon 129 genotype and new variant CJD. Lancet 350, 668.