Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins.

Abstract:

:Scrapie and Creutzfeldt-Jakob disease are transmissible, degenerative neurological diseases caused by prions. Considerable evidence argues that prions contain protease-resistant sialoglycoproteins, designated PrPSc, encoded by a cellular gene. The prion protein (PrP) gene also encodes a normal cellular protein designated PrPC. We established clonal cell lines which support the replication of mouse scrapie or Creutzfeldt-Jakob disease prions. Mouse neuroblastoma N2a cells were exposed to mouse scrapie prions and subsequently cloned. After limited proteinase K digestion, three PrP-immunoreactive proteins with apparent molecular masses ranging between 20 and 30 kilodaltons were detected in extracts of scrapie-infected N2a cells by Western (immuno-) blotting. The authenticity of these PrPSc molecules was established by using monospecific antiserum raised against a synthetic peptide corresponding to a portion of the prion protein. Those clones synthesizing PrPSc molecules possessed scrapie prion infectivity as measured by bioassay; clones without PrPSc failed to demonstrate infectivity. Detection of PrPSc molecules in scrapie-infected N2a cells supports the contention that PrPSc is a component of the infectious scrapie particle and opens new approaches to the study of prion diseases.

journal_name

J Virol

journal_title

Journal of virology

authors

Butler DA,Scott MR,Bockman JM,Borchelt DR,Taraboulos A,Hsiao KK,Kingsbury DT,Prusiner SB

doi

10.1128/JVI.62.5.1558-1564.1988

subject

Has Abstract

pub_date

1988-05-01 00:00:00

pages

1558-64

issue

5

eissn

0022-538X

issn

1098-5514

journal_volume

62

pub_type

杂志文章