Prion strains

Safar J, Wille H, Itri V, Groth D, Serban H, Torchia M, Cohen FE, Prusiner SB. Eight prion strains have PrP(Sc) molecules with different conformations [see comments], Nat Med 1998 4 1157-1165. 

Both PrP sequence and prion strain type influence prion transmission barriers 800... 

B-cell deficient mice are resistant to intraperitoneal inoculation with prions probably because of their involvement with FDC maturation and maintenance. The interface between FDCs and sympathetic nerves represents a critical site for the transfer of lymphoid prions into the nervous system however, the mechanism by which this is achieved remains unknown. Distinct forms of prion disease show differences in lymphoreticular involvement that may be related to the etiology of the disease or to divergent properties of distinct prion strains. For a review of prion disease peripheral pathogenesis see [18]. 

Collinge, J. et al. Molecular analysis of prion strain variation and the aetiology of new variant CJD. Nature 383 685-690, 1996. 

Asante, E. A. et al. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J. 21 6358-6366,2002. 

King, C. Y. (2001). Supporting the structural basis of prion strains Induction and identification of l Sf variants./ Mol. Biol. 307, 1247-1260. 

King, C. Y., and Diaz-Avalos, R. (2004). Protein-only transmission of three yeast prion strains. Nature 428, 319-323. 

Schlumpberger, M., Prusiner, S. B., and Herskowitz, I. (2001). Induction of distinct [URE3] yeast prion strains. Mol. Cell. Biol. 21, 7035-7046. 

Tanaka, M., Chien, P., Naber, N., Cooke, R., and Weissman,J. S. (2004). Conformational variations in an infectious protein determine prion strain differences. Nature 428, 323-328. 

Fig. 20 Fluorescent images of prion deposits associated with distinct prion strains, murine chronic wasting disease (mCWD) (a) and murine sheep scrapie (mSS) (b), which has been stained by PTAA. (c) Correlation diagram of the ratios, R532/639 and R532/ max, of the intensity of the emitted light from PTAA bound to prion deposits originating from individual mice infected with CWD (black symbols) or sheep scrapie (purple symbols) [35]...

Sigurdson CJ, Nilsson KPR, Homemann S, Manco G, Polymenidou M, Schwarz P, Leclerc M, Hammarstrdm P, Wuthrich K, Aguzzi A (2007) Prion strain discrimination using luminescent conjugated polymers. Nat Methods 4 1023-1030... 

Current prion disinfection assays are slow, laborious and costly —they employ contaminated test tissue homogenates (prion strain and concentration variable, possible disinfectant inactivation), test animals which serve to assess the viability of treated tissue, with the log decrease being calculated from incubation period assays rather than from end-point titrations. 

Each of the Quantitative Trait Loci (QTL) studies had unique features that maximised the potential for novel locus discovery but makes comparisons difficult. The first source of variation was the choice of mouse strains. Three different combinations were used CAST x NZW (F2 intercross) [55, 56], CAST x SJL (F2 intercross) [57] and C57 x RIII (reciprocal backcross and F2 intercross) [57, 59]. Using only two strains of mice in a cross simplifies the analysis but limits the number of potential alleles compared to those expected in an outbred population. The use of closely related laboratory strains also limits the potential for detecting variation. The second source of variation was the choice of prion strain. Across all... 

Keywords Animal Atypical Atypical/Nor98 scrapie BSE-H BSE-L Human Prion disease Prion strain Prion type... 

By combining these methods for PrPSc typing with neuropathological lesion profiles [49] and, importantly, patterns of brain PrPSc deposits [50-53], prion strains can be fairly well characterized. However, current protocols are quite cumbersome and time-consuming. Therefore, other approaches for discriminating between prion strains, using conformation-sensitive probes [54] or cell culture systems [55], have been advocated. 

Despite steady progress in non-bioassay discrimination of prion strains, the mouse bioassays remain superior. This was recently demonstrated when two strains of chronic wasting disease (CWD), a prion disease affecting cervids, with biochemically indistinguishable PrPSc aggregates, were recognized as separate CWD-1 and... 

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