The glycosylation status of PrPC is a key factor in determining transmissible spongiform encephalopathy transmission between species.
Bottom Line: The absence of glycosylation at both or the first PrP(C) glycosylation site in the host results in almost complete resistance to disease.We infected mice that express different forms of glycosylated PrP(C) with three different TSE agents.We demonstrate that changing the glycosylation status of the host can have profound effects on disease transmission, changing host susceptibility and incubation times.
Affiliation: Neurobiology Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Easter Bush, United Kingdom.Show MeSH
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Mentions: To ascertain whether the protein levels of PrPC in any of the lines of mice could influence the incubation times in the transmissions described above, we undertook analysis of the PrPC levels in all mouse lines. Immunoblot analysis showed the expected banding patterns for both the transgenic and wild-type mice. Truncated PrPC, designated C1, was observed in all mice and was included in the measurement of total PrPC (selected antibodies are shown in Fig. 4A and B). All of the glycosylation-deficient mice expressed significantly less PrPC than wild-type mice (P < 0.0001). G1 and G2 mice expressed approximately 50% and G3 mice only 32% of that found in wild-type mice, with G3 mice expressing significantly less (P < 0.01) than both G1 and G2 mice. There was no significant difference in total PrP in each mouse line using different antibodies (Fig. 4A and B).
Affiliation: Neurobiology Division, The Roslin Institute and R(D)SVS, University of Edinburgh, Easter Bush, United Kingdom.