Prion strain discrimination in cell culture: the cell panel assay

SP Mahal, CA Baker, CA Demczyk… - Proceedings of the …, 2007 - National Acad Sciences
SP Mahal, CA Baker, CA Demczyk, EW Smith, C Julius, C Weissmann
Proceedings of the National Academy of Sciences, 2007National Acad Sciences
Prions are thought to consist mainly or entirely of misfolded PrP, a constitutively expressed
host protein. Prions associated with the same PrP sequence may occur in the form of
different strains; the strain phenotype is believed to be encoded by the conformation of the
PrP. Some cell lines can be persistently infected by prions and, interestingly, show
preference for certain strains. We report that a cloned murine neuroblastoma cell population,
N2a-PK1, is highly heterogeneous in regard to its susceptibility to RML and 22L prions …
Prions are thought to consist mainly or entirely of misfolded PrP, a constitutively expressed host protein. Prions associated with the same PrP sequence may occur in the form of different strains; the strain phenotype is believed to be encoded by the conformation of the PrP. Some cell lines can be persistently infected by prions and, interestingly, show preference for certain strains. We report that a cloned murine neuroblastoma cell population, N2a-PK1, is highly heterogeneous in regard to its susceptibility to RML and 22L prions. Remarkably, sibling subclones may show very different relative susceptibilities to the two strains, indicating that the responses can vary independently. We have assembled four cell lines, N2a-PK1, N2a-R33, LD9 and CAD5, which show widely different responses to prion strains RML, 22L, 301C, and Me7, into a panel that allows their discrimination in vitro within 2 weeks, using the standard scrapie cell assay (SSCA).
National Acad Sciences