Spontaneous generation of rapidly transmissible prions in transgenic mice expressing wild-type bank vole prion protein

Proceedings of the National Academy of Sciences of the United States of America

Volumn 109, Issue 9, 2012, Pages 3498-3503

  Watts, Joel C ^a   Giles, Kurt ^a,b   Stöhr, Jan ^a   Oehler, Abby ^a   Bhardwaj, Sumita ^a   Grillo, Sunny K ^a   Patel, Smita ^a   DeArmond, Stephen J ^a   Prusiner, Stanley B ^a,b  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Abbreviated incubation times; Bioluminescence imaging; Neurodegeneration

Indexed keywords

PRION PROTEIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; ASTROCYTOSIS; BANK VOLE; BRAIN HOMOGENATE; CENTRAL NERVOUS SYSTEM DISEASE; CREUTZFELDT JAKOB DISEASE; MOUSE; NONHUMAN; NUCLEOTIDE SEQUENCE; PRION DISEASE; PRIORITY JOURNAL; PROTEIN CONFORMATION; PROTEIN EXPRESSION; PROTEIN FOLDING; VOLE;

AMINO ACID SEQUENCE; ANIMALS; ARVICOLINAE; BRAIN; BRAIN CHEMISTRY; CODON; DISEASE MODELS, ANIMAL; GENES, REPORTER; HOST SPECIFICITY; MICE; MICE, TRANSGENIC; MOLECULAR SEQUENCE DATA; NERVE TISSUE PROTEINS; PRION DISEASES; PRIONS; PROMOTER REGIONS, GENETIC; PROTEIN FOLDING; PROTEIN STRUCTURE, SECONDARY; RECOMBINANT FUSION PROTEINS; SEQUENCE ALIGNMENT; SEQUENCE HOMOLOGY, AMINO ACID; SPECIES SPECIFICITY; TISSUE EXTRACTS;

ANIMALIA; CLETHRIONOMYS GLAREOLUS; MUS; MUS MUSCULUS;

EID: 84857756257     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1121556109     Document Type: Article

References (46)

1. Colby DW, Prusiner SB (2011) Prions. Cold Spring Harb Perspect Biol 3:a006833.
2. Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 216:136-144.
3. Li M, et al. (2011) Widespread RNA and DNA sequence differences in the human transcriptome. Science 333:53-58.
4. Deleault NR, Harris BT, Rees JR, Supattapone S (2007) Formation of native prions from minimal components in vitro. Proc Natl Acad Sci USA 104:9741-9746. (Pubitemid 47175337)
5. Colby DW, et al. (2009) Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci USA 106:20417-20422.
6. Makarava N, et al. (2010) Recombinant prion protein induces a new transmissible prion disease in wild-type animals. Acta Neuropathol 119:177-187.
7. Wang F, Wang X, Yuan C-G, Ma J (2010) Generating a prion with bacterially expressed recombinant prion protein. Science 327:1132-1135.
8. Edgeworth JA, et al. (2010) Spontaneous generation of mammalian prions. Proc Natl Acad Sci USA 107:14402-14406.
9. Hsiao KK, et al. (1990) Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 250:1587-1590. (Pubitemid 120034374)
10. Telling GC, et al. (1996) Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev 10:1736-1750. (Pubitemid 26272380)
11. Chiesa R, Piccardo P, Ghetti B, Harris DA (1998) Neurological illness in transgenic mice expressing a prion protein with an insertional mutation. Neuron 21:1339-1351. (Pubitemid 29022536)
12. Dossena S, et al. (2008) Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model. Neuron 60:598-609.
13. Jackson WS, et al. (2009) Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice. Neuron 63:438-450.
14. Friedman-Levi Y, et al. (2011) Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease. PLoS Pathog 7:e1002350.
15. Asante EA, et al. (2009) Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins. J Gen Virol 90:546-558.
16. Kitamoto T, Tateishi J, Sawa H, Doh-Ura K (1989) Positive transmission of Creutzfeldt- Jakob disease verified by murine kuru plaques. Lab Invest 60:507-512. (Pubitemid 19141799)
17. Nonno R, et al. (2006) Efficient transmission and characterization of Creutzfeldt-Jakob disease strains in bank voles. PLoS Pathog 2:e12.
18. Agrimi U, et al. (2008) Prion protein amino acid determinants of differential susceptibility and molecular feature of prion strains in mice and voles. PLoS Pathog 4:e1000113.
19. Heisey DM, et al. (2010) Chronic wasting disease (CWD) susceptibility of several North American rodents that are sympatric with cervid CWD epidemics. J Virol 84:210-215.
20. Cartoni C, et al. (2005) Identification of the pathological prion protein allotypes in scrapie-infected heterozygous bank voles (Clethrionomys glareolus) by high-performance liquid chromatography-mass spectrometry. J Chromatogr A 1081:122-126. (Pubitemid 40799240)
21. Will RG, et al. (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347:921-925. (Pubitemid 26102546)
22. Watts JC, et al. (2011) Protease-resistant prions selectively decrease shadoo protein. PLoS Pathog 7:e1002382.
23. Wilham JM, et al. (2010) Rapid end-point quantitation of prion seeding activity with sensitivity comparable to bioassays. PLoS Pathog 6:e1001217.
24. Hsiao KK, et al. (1994) Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc Natl Acad Sci USA 91:9126-9130.
25. Nazor KE, et al. (2005) Immunodetection of disease-associated mutant PrP, which accelerates disease in GSS transgenic mice. EMBO J 24:2472-2480. (Pubitemid 41032597)
26. Stöhr J, et al. (2011) Spontaneous generation of anchorless prions in transgenic mice. Proc Natl Acad Sci USA 108:21223-21228.
27. Zhu L, et al. (2004) Non-invasive imaging of GFAP expression after neuronal damage in mice. Neurosci Lett 367:210-212. (Pubitemid 39141229)
28. Tamgüney G, et al. (2009) Measuring prions by bioluminescence imaging. Proc Natl Acad Sci USA 106:15002-15006.
29. Watts JC, et al. (2011) Bioluminescence imaging of Abeta deposition in bigenic mouse models of Alzheimer's disease. Proc Natl Acad Sci USA 108:2528-2533.
30. Carlson GA, et al. (1994) Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proc Natl Acad Sci USA 91:5690-5694. (Pubitemid 24174394)
31. Colby DW, et al. (2010) Protease-sensitive synthetic prions. PLoS Pathog 6:e1000736.
32. Ma J, Lindquist S (2002) Conversion of PrP to a self-perpetuating PrPSc-like conformation in the cytosol. Science 298:1785-1788. (Pubitemid 35404121)
33. Jansen C, et al. (2010) Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP. Acta Neuropathol 119:189-197.
34. Sigurdson CJ, et al. (2009) De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. Proc Natl Acad Sci USA 106:304-309.
35. Christen B, Pérez DR, Hornemann S, Wüthrich K (2008) NMR structure of the bank vole prion protein at 20 degrees C contains a structured loop of residues 165-171. J Mol Biol 383:306-312.
36. Sigurdson CJ, et al. (2010) A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest 120:2590-2599.
37. Sigurdson CJ, et al. (2011) Spongiform encephalopathy in transgenic mice expressing a point mutation in the β2-α2 loop of the prion protein. J Neurosci 31:13840-13847.
38. Moore RC, et al. (1998) Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent. Nat Genet 18:118-125. (Pubitemid 28082457)
39. Barron RM, et al. (2005) Polymorphisms at codons 108 and 189 in murine PrP play distinct roles in the control of scrapie incubation time. J Gen Virol 86:859-868. (Pubitemid 40331268)
40. Hegde RS, et al. (1998) A transmembrane form of the prion protein in neurodegenerative disease. Science 279:827-834. (Pubitemid 28106269)
41. Collinge J, Clarke AR (2007) A general model of prion strains and their pathogenicity. Science 318:930-936. (Pubitemid 350098981)
42. Sandberg MK, Al-Doujaily H, Sharps B, Clarke AR, Collinge J (2011) Prion propagation and toxicity in vivo occur in two distinct mechanistic phases. Nature 470:540-542.
43. Scott MR, Köhler R, Foster D, Prusiner SB (1992) Chimeric prion protein expression in cultured cells and transgenic mice. Protein Sci 1:986-997. (Pubitemid 23009207)
44. Carlson GA, et al. (1988) Genetics and polymorphism of the mouse prion gene complex: Control of scrapie incubation time. Mol Cell Biol 8:5528-5540.
45. Safar JG, et al. (2002) Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice. Nat Biotechnol 20:1147-1150. (Pubitemid 35285445)
46. Williamson RA, et al. (1998) Mapping the prion protein using recombinant antibodies. J Virol 72:9413-9418. (Pubitemid 28471899)