NR AJPO
AU Priola,S.A.; Chesebro,B.
TI A single hamster PrP amino acid blocks conversion to protease-resistant PrP in scrapie-infected mouse neuroblastoma cells
QU Journal of Virology 1995 Dec; 69(12): 7754-8
PT journal article
AB Neurodegeneration caused by the transmissible spongiform encephalopathies is associated with the conversion of a normal host protein, PrP-sen, into an abnormal aggregated protease-resistant form, PrPres. In scrapie-infected mouse neuroblastoma cells, mouse PrP-sen is converted into PrPres but recombinant hamster PrP-sen expressed in these cells is not. In the present studies, recombinant hamster/mouse PrP-sen molecules were expressed in these scrapie-infected cells to define specific PrP amino acid residues critical for the conversion to PrPres. The results showed that homology to the region of mouse PrP-sen from amino acid residues 112 to 138 was required for conversion of recombinant PrP-sen to PrPres in scrapie-infected mouse cells. Furthermore, a single hamster-specific PrP amino acid at residue 138 could inhibit the conversion of the recombinant PrP-sen into PrPres. The data are consistent with studies in humans which show that specific amino acid residue changes within PrP can influence disease pathogenesis and transmission of transmissible spongiform encephalopathies across species barriers.
IN In scrapieinfizierten Mausneuroblastomzellen wurden Mausprionproteine, nicht aber Hamsterprionproteine in die proteaseresistente Form überführt. Experimente mit verschiedenen rekombinierten Maus-Hamster-Prionproteinen zeigten, dass hierfür Unterschiede in der Region 112-138 verantwortlich sind.
ZR 41
MH Amino Acid Sequence; Animal; Base Sequence; Cell Line; Cloning, Molecular; DNA Primers; Drug Resistance, Microbial; Endopeptidases/*metabolism/pharmacology; Hamsters; Mice; Molecular Sequence Data; Mutagenesis, Site-Directed; Neuroblastoma; *Point Mutation; Polymerase Chain Reaction; Prions/drug effects/genetics/*metabolism; Recombinant Proteins/biosynthesis/drug effects/metabolism; Scrapie; Tumor Cells, Cultured
AD Laboratory of Persistent Viral Diseases, National Institute of Allergy and Infectious Diseases, Rocky Mountain Laboratories, Hamilton, Montana 59840, USA
SP englisch
PO USA
OR Prion-Krankheiten 6