Work

Cell-specific susceptibility to prion strains is a property of the intact cell

Year: 2012

Type: article

Abstract: Prions consist of PrP (Sc), a misfolded version of the cellular protein PrP (C). They occur in a variety of strains that share the amino acid sequence of PrP but differ in phenotypic properties, such as cell tropism and pathogenicity; strain-ness is attributed to the conformation of PrP (Sc). To gain insight as to how susceptibility of cells to a given prion strain comes about, we compared amplification of RML prions by PMCA, using cell lysates from related, RML-resistant and RML-susceptible cell lines as substrate. We found that both lysates supported amplification of RML PrP (Sc) equally well, despite a 280-fold difference in the susceptibility of the cells from which they were derived. Thus, susceptibility is an attribute of the intact cell.

Source: Prion

DOI: 10.4161/pri.20198

Authors: Maria E. Herva, Charles Weissmann

Language: English

Topic: Prion Diseases and Protein Misfolding

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

Open Access status: bronze

Funders: National Institutes of Health, U.S. Public Health Service, National Institute of Neurological Disorders and Stroke

Awards: Emergence of drug resistance in prion populations, G8850185918

OpenAlex ID: W2090733594

FWCI: 0.361

Cites: 33

Cited by: 8

Related to: 10