NR ACCQ
AU Butler,D.A.; Scott,M.R.D.; Bockman,J.M.; Borchelt,D.R.; Taraboulos,A.; Hsiao,K.K.; Kingsbury,D.T.; Prusiner,S.B.
TI Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins
QU Journal of Virology 1988 May; 62(5): 1558-64
IA http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=253182&blobtype=pdf
PT journal article
AB 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.
MH Animal; Antibodies, Monoclonal; Biological Assay; Clone Cells; Creutzfeldt-Jakob Syndrome/genetics; Endopeptidase K; Hamsters; Immunosorbent Techniques; Neuroblastoma/metabolism/*microbiology; Peptide Hydrolases/*metabolism; *Prions; Scrapie/*genetics; Serine Endopeptidases/metabolism; Sialoglycoproteins/metabolism; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Darel A. Butler, Michael R. D. Scott, David R. Borchelt, Albert Taraboulos, Karen K. Hsiao, Stanley B. Prusiner, Department of Neurology, University of California, San Francisco 94143-0518; Jeffrey M. Bockman, David T. Kingsbury, Department of Microbiology, George Washington University Medical Center, Washington, D.C. 200372; Stanley B. Prusiner, Department of Biochemistry and Biophysics, University of California, San Francisco, California 94143-0518
SP englisch
PO USA