NR ACFM
AU Carlson,G.A.; DeArmond,S.J.; Torchia,M.; Westaway,D.; Prusiner,S.B.
TI Genetics of prion diseases and prion diversity in mice
QU Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences 1994 Mar 29; 343(1306): 363-9
IA http://www.journals.royalsoc.ac.uk/media/e3d054fe8l2tpl881dcg/contributions/j/5/6/7/j567h7m1x7g36643.pdf
PT journal article; review; review, tutorial
AB Linkage of the prion protein (PrP) and scrapie incubation time genes in mice provided strong evidence for the central role of PrP in determining susceptibility to prion disorders. Considerable evidence now argues that the prion protein and incubation time genes are identical. The mouse prion protein gene (Prn-p) may act both quantitatively and qualitatively in modulating prion incubation time. Differences at positions 108 and 189 between PrP-A and PrP-B allotypes can place constraints on interaction between the normal cellular and the scrapie-specific isoforms of PrP (PrPc and PrPsc), although the supply of PrPc available for post-translational conversion to PrPsc can also influence incubation time. Results using transgenic (Tg) mice in studies on scrapie 'strains' or isolates suggest that incubation time characteristics of scrapie isolates can be explained by these two properties of PrP. The final section of this report discusses the novel finding that uninoculated Tg mice overexpressing wild-type (wt) PrP transgenes spontaneously develop a late-onset degenerative neuromyopathy, broadening the spectrum of prion diseases and providing new information on PrP function in both normal and pathological states.
ZR 32
IN Da das Scrapie-Inkubationszeit-Gen mit dem Prionproteingen identisch zu sein scheint, spielt das Prionprotein offenbar eine zentrale Rolle bei Empfänglichkeit für BSE. Außerdem beeinflußt das anscheinend infizierende Prionprotein den Krankheitsverlauf. Mäuse mit einer Überexpression des normalen Prionproteingens entwickeln im Alter ebenfalls BSE.
MH Animal; Disease Models, Animal; Linkage (Genetics); Mice; Mice, Transgenic; Nerve Tissue Proteins/*genetics; Neuromuscular Diseases/etiology/genetics; PrPsc Proteins; Prion Diseases/*genetics; Prions/*genetics; Scrapie/etiology/genetics; Variation (Genetics)
AD McLaughlin Research Institute, Great Falls, Montana 59405.
SP englisch
PO England