2014
DOI: 10.4161/19336896.2014.983396
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Sheep prions with molecular properties intermediate between classical scrapie, BSE and CH1641–scrapie

Abstract: Efforts to differentiate bovine spongiform encephalopathy (BSE) from scrapie in prion infected sheep have resulted in effective methods to decide about the absence of BSE. In rare instances uncertainties remain due to assumptions that BSE, classical scrapie and CH1641–a rare scrapie variant–could occur as mixtures. In field samples including those from fallen stock, triplex Western blotting analyses of variations in the molecular properties of the proteinase K resistant part of the disease‑associated form of p… Show more

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Cited by 8 publications
(13 citation statements)
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“…Intriguingly, recent PrP Sc molecular studies have found evidence of natural mixed infections including CH1641-like strains in sheep (Langeveld et al, 2014) and experimental inoculation studies have suggested that the phenotype of atypical scrapie can in some instances change into that of CH1641 during passage in sheep (Simmons et al, 2015). It is therefore more likely that natural TSE infections of ruminants involve mixtures of strains, or have a more fluid identity, rather than the single strains normally used in laboratory studies.…”
Section: Discussionmentioning
confidence: 97%
“…Intriguingly, recent PrP Sc molecular studies have found evidence of natural mixed infections including CH1641-like strains in sheep (Langeveld et al, 2014) and experimental inoculation studies have suggested that the phenotype of atypical scrapie can in some instances change into that of CH1641 during passage in sheep (Simmons et al, 2015). It is therefore more likely that natural TSE infections of ruminants involve mixtures of strains, or have a more fluid identity, rather than the single strains normally used in laboratory studies.…”
Section: Discussionmentioning
confidence: 97%
“…CH1641 is characterized by N-terminal PrP res features resembling those of BSE 8,21 , but is distinguishable from BSE by discriminatory immunohistochemistry 22 and by improved molecular typing approaches [23][24][25][26] . Intensified surveillance in the EU allowed to identify molecular and pathological properties reminiscent of experimental CH1641 in naturally affected sheep and goats ; 12,24,25,27,28 these cases have been often referred to as CH1641-like scrapie. Finally, Nor98 was first detected in 1998 in Norway as a new form of scrapie characterized by distinctive clinical and pathological features 29 , and has been later recognized as a sporadic, putatively spontaneous or poorly transmissible, form of scrapie affecting small ruminants worldwide 30 .…”
mentioning
confidence: 99%
“…Immunochemical quantification of PrP res was subsequently performed by fluorimetric detection monitored in a three-laser-beam imager (Typhoon Trio variable-mode imager; Amersham Biosciences) (38). For estimation of the ARR-and VRQ-PrP fractions in PrP res , a mixture of two antibodies was applied, one of which (SAF84) binds only if the 171Q polymorphism is present (VRQ-PrP or ARQ-PrP) while the other binds equally well to VRQ-, ARQ-, and ARR-PrP (33,38,39). Two different mixtures with SAF84 were used: SAF84 with L42 (L42/SAF84 combination) and SAF84 with Sha31 (Sha31/SAF84 combination).…”
Section: Methodsmentioning
confidence: 99%
“…SAF84 detection was carried out with a Zenon labeling Alexa 488 kit, and L42 or Sha31 was detected with a Zenon labeling Alexa 647 kit (see above for kit specifications). The VRQ-PrP and ARQ-PrP fractions in PrP res samples were calculated as follows (33,38,39). When the SAF84/L42 antibody combination was used, the fraction of the 171Q-PrP product [Fr(171Q-PrP); the VRQ-or ARQ-PrP levels] in scrapie or BSE was obtained by applying the formula Fr(171Q-PrP) ϭ ratio x /ratio Q/Q , where ratio x is the SAF84/L42 ratio of an unknown sample, and ratio Q/Q is the SAF84/L42 ratio determined for Q/Q homozygous material, which is the average of measurements of the different scrapie (n ϭ 10) or BSE (n ϭ 8) Q/Q samples; the fraction of the 171R-PrP product (the ARR-PrP level) could be de- duced from the formula (ratio Q/Q Ϫ ratio x )/ratio Q/Q .…”
Section: Methodsmentioning
confidence: 99%
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