Prion scrapie susceptibility

Eghiaian F, Grosclaude J, Lesceu S et al (2004) Insight into the PrPC->PrPSc conversion from the structures of antibody-bound ovine prion scrapie-susceptibility variants. Proc Natl Acad Sci USA 101 10254-10259... 

Jackson, G. S., Hosszu, L. L. R, Power, A., Hill, A. F., Kenney, J., Seiibil, H., Craven, C. J., Waltho, J. R, Qarke, A. R., and Collinge, J. 1999. Reversible conversion of monomeric human prion protein between native emd fibrilogenic conformations. Science 283 1935-1937 Jeffrey, M., Gonzalez, L., Espenes, A., Press, C. M., Martin, S., Chaplin, M., DAvis, L., Landsverk, T., MacAldowie, C., Eaton, S., and McGovern, G. 2006. Transportation of prion protein across the intestineil mucosa of scrapie-susceptible and scrapie-resistant sheep. J. Pathol. 209 4-14... 

In other acquired prion diseases, notably scrapie of sheep and variant Creutzfeldt-Jakob disease (vCJD) in humans, amino acids encoded at certain key positions in the endogenous host prion protein are strongly associated with susceptibility to prion infection [57-59]. Studies of CWD in elk, mule deer, white-tailed deer, and moose have found that similar correlations between PrP amino acid sequence and likelihood of CWD infection could exist in these species as well. 

Westaway D, Zuliani V, Cooper CM, Da Costa M, Neuman S, Jenny AL, Detwiler L, Prusiner SB (1994) Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. Genes Dev 8 959-969... 

Based on the finding that selective expression of PrP in astrocytes is sufficient to restore susceptibility of PrP / mice to scrapie infection, it was advanced that astroglial cells feature in prion disease pathogenesis, possibly by an indirect toxic effect on neurons (Raeber et al, 1997). This hypothesis is supported by an in vitro study where PrP / neurons became susceptible to PrP106-126 neurotoxicity when co-cultured with Prp+/+ astrocytes (Brown, 1999). 

Gomi, H., Yokoyama, T., Fujimoto, K., Ikeda, T, Katoh, A., Itoh, T. and Itohara, S. (1995). Mice devoid of the glial fibrillary acidic protein develop normally and are susceptible to scrapie prions. Neuron 14, 29-41. 

Fischer, M., Rtilicke, T, Raeber, A., Sailer, A., Moser, M., Oesch, B., Brandner, S., Aguzzi, A., and Weissmann, C. (1996). Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBOJ. 15, 1255-1264. 

Race, E.R., Priola, S.A., Bessen, R.A., Ernst, D., Dockter, J., Mucke, L., Chesebro, B., and Oldstone, M.B.A. (1995). Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent. Neuron 15,1183-1191. 

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