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Updated: Jun 10, 2026
Clinical Examination Protocol to Detect Atypical and Classical Scrapie in Sheep
Published on: January 19, 2014
PrP(Sc) is associated with B cells in the blood of scrapie-infected sheep
Jane C Edwards1, S Jo Moore, Jeremy A Hawthorn
1Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency, Woodham Lane, New Haw, Surrey, UK.
Abstract:
Recently, we reported that PrP(Sc), a surrogate marker for prion disease, is associated with the cellular fraction of blood from scrapie-infected sheep using a ligand-based immunoassay. In the study reported here, we found that a subset of peripheral blood mononuclear cells is most likely to sequester PrP(Sc) during both the preclinical phase of disease and at clinical end point. These cells had a cell surface phenotype of MHC class II DQ(+), surface immunoglobulin(+), CD11b(+), CD11c(+), CD21(+/)(-), which is consistent with a subpopulation of B cells. What role these cells play in the pathogenesis of scrapie is unclear, but they may contribute to the trafficking of prions to the spleen during early pathogenesis of the disease. Furthermore, tests for preclinical diagnostics could be further improved by targeting these cells.
Insights
Prion diseases like scrapie involve PrP(Sc) accumulating in specific blood cells. Identifying these prion-infected cells could improve early diagnosis and understanding of disease spread.
Area of Science:
- Veterinary Medicine
- Immunology
- Neuroscience
Background:
- Prion diseases, such as scrapie in sheep, are characterized by the accumulation of abnormal prion protein (PrPSc).
- Previous research identified PrPSc in the cellular fraction of blood from scrapie-infected sheep using a ligand-based immunoassay.
Purpose of the Study:
- To identify the specific blood cell type that sequesters PrPSc in scrapie-infected sheep.
- To investigate the role of these cells in prion disease pathogenesis and potential diagnostic applications.
Main Methods:
- Analysis of peripheral blood mononuclear cells (PBMCs) from scrapie-infected sheep.
- Characterization of cell surface phenotype using flow cytometry, including markers for MHC class II DQ, surface immunoglobulin, CD11b, CD11c, and CD21.
- Assessment of PrPSc presence in identified cell populations.
Main Results:
- A specific subset of PBMCs was identified as the primary sequesterer of PrPSc.
- These cells exhibited a phenotype consistent with a subpopulation of B cells (MHC class II DQ+, surface immunoglobulin+, CD11b+, CD11c+, CD21+/-).
- PrPSc was found in these cells during both preclinical and clinical stages of scrapie.
Conclusions:
- A subset of B cells is likely responsible for sequestering PrPSc in the blood of scrapie-infected sheep.
- These cells may play a role in prion trafficking to the spleen during early disease pathogenesis.
- Targeting these prion-infected B cells could enhance preclinical diagnostic tests for prion diseases.
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