Journal article

Mouse-adapted sporadic human Creutzfeldt-Jakob disease prions propagate in cell culture

Victoria A Lawson, Laura J Velia, James D Stewart, Robyn A Sharples, Helen Klemm, Dorothy M Machalek, Colin L Masters, Roberto Cappai, Steven J Collins, Andrew F Hill



Cell based models used for the study of prion diseases have traditionally employed mouse-adapted strains of sheep scrapie prions. To date, attempts to generate human prion propagation in cell culture have been unsuccessful. Rabbit kidney epithelial cells (RK13) are permissive to infection with prions from a variety of species upon expression of cognate PrP transgenes. We explored RK13 cells expressing human PrP for their utility as a cell line capable of sustaining infection with human prions. RK13 cells processed exogenously expressed human PrP similarly to exogenously expressed mouse PrP but were not permissive to infection when exposed to sporadic Creutzfeldt-Jakob disease prions. Transmi..

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Awarded by NHMRC Program

Awarded by NHMRC Practitioner Fellowship

Funding Acknowledgements

We thank Professor Charles Weissmann for the gift of the Tga20 mice, Professor John Collinge for the gift of monoclonal antibody ICSM18 and Dr Bruce Chesebro for the gift of the monoclonal antibody 3F4. We thank the animal facility staff of the Department of Pathology, Ms. L. Leone and Ms. L. Forester for performing histology and immunohistochemistry, and Mr. B. Kreunen for preparation of figures. This work was supported by an NHMRC Program Grant #400202. LJV is the recipient of a University of Melbourne Postgraduate Research Scholarship, VAL is the recipient of an NHMRC Howard Florey Fellowship, AFH is the recipient of an NHMRC RD Wright Career Development Award, RC is a recipient of an NHMRC Senior Research Fellowship, and SJC is a recipient of an NHMRC Practitioner Fellowship #400183.