NR AKQI
AU Scott,M.R.D.; Groth,D.; Tatzelt,J.; Torchia,M.; Tremblay,P.; DeArmond,S.J.; Prusiner,S.B.
TI Propagation of prion strains through specific conformers of the prion protein
QU Journal of Virology 1997 Dec; 71(12): 9032-44
PT journal article
AB Two prion strains with identical incubation periods in mice exhibited distinct incubation periods and different neuropathological profiles upon serial transmission to transgenic mice expressing chimeric Syrian hamster/mouse (MH2M) prion protein (PrP) genes [Tg(MH2M) mice] and subsequent transmission to Syrian hamsters. After transmission to Syrian hamsters, the Me7 strain was indistinguishable from the previously established Syrian hamster strain Sc237, despite having been derived from an independent ancestral source. This apparent convergence suggests that prion diversity may be limited. The Me7 mouse strain could also be transmitted directly to Syrian hamsters, but when derived in this way, its properties were distinct from those of Me7 passaged through Tg(MH2M) mice. The Me7 strain did not appear permanently altered in either case, since the original incubation period could be restored by effectively reversing the series of passages. Prion diversity enciphered in the conformation of the scrapie isoform of PrP (PrPsc) (G. C. Telling et al., Science 274:2079-2082, 1996) seems to be limited by the sequence of the PrP substrates serially converted into PrPsc, while prions are propagated through interactions between the cellular and scrapie isoforms of PrP.
MH Animal; Brain/metabolism/pathology; Chimeric Proteins/chemistry/metabolism; Hamsters; Mesocricetus; Mice; Mice, Inbred C57BL; PrPsc Proteins/chemistry/metabolism; Prion Diseases/*etiology/pathology/transmission; Prions/*biosynthesis/*chemistry/classification/isolation & purification; Protein Conformation; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Department of Neurology, University of California, San Francisco 94143, USA
SP englisch
PO USA