title: The standard scrapie cell assay: development, utility and prospects.
abstract: Prion diseases are a family of fatal neurodegenerative diseases that involve the misfolding of a host protein, PrPC. Measuring prion infectivity is necessary for determining efficacy of a treatment or infectivity of a prion purification procedure; animal bioassays are, however, very expensive and time consuming. The Standard Scrapie Cell Assay (SSCA) provides an alternative approach. The SSCA facilitates quantitative in vitro analysis of prion strains, titres and biological properties. Given its robust nature and potential for high throughput, the SSCA has substantial utility for in vitro characterization of prions and can be deployed in a number of settings. Here we provide an overview on establishing the SSCA, its use in studies of disease dissemination and pathogenesis, potential pitfalls and a number of remaining challenges.
authors.affiliation: Centre for Prions and Protein Folding Diseases, University of Alberta, Alberta, T6G2M8, Canada. jqvander@ualberta.ca.
authors.forename: Jacques
authors.initials: J
authors.lastname: van der Merwe
authors.affiliation: Centre for Prions and Protein Folding Diseases, University of Alberta, Alberta, T6G2M8, Canada. jmaiken@ualberta.ca.
authors.forename: Judd
authors.initials: J
authors.lastname: Aiken
authors.affiliation: Centre for Prions and Protein Folding Diseases, University of Alberta, Alberta, T6G2M8, Canada. david.westaway@ualberta.ca.
authors.forename: David
authors.initials: D
authors.lastname: Westaway
authors.affiliation: Centre for Prions and Protein Folding Diseases, University of Alberta, Alberta, T6G2M8, Canada. debbie.mckenzie@ualberta.ca.
authors.forename: Debbie
authors.initials: D
authors.lastname: McKenzie
mesh_terms: D000818:Animals; D002460:Cell Line; D003584:Cytological Techniques / Q000379:methods*; D006801:Humans; D051379:Mice; D011328:Prions / Q000032:analysis* / Q000302:isolation & purification* / Q000378:metabolism; D011817:Rabbits; D012608:Scrapie / Q000175:diagnosis*
chemical_list: D011328:Prions