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• W2775683821 endingPage "e0188989" @default.
• W2775683821 startingPage "e0188989" @default.
• W2775683821 abstract "Prion diseases have been linked to impaired copper homeostasis and copper induced-oxidative damage to the brain. Divalent metal ions, such as Cu2+ and Zn2+, bind to cellular prion protein (PrPC) at octapeptide repeat (OR) and non-OR sites within the N-terminal half of the protein but information on the impact of such binding on conversion to the misfolded isoform often derives from studies using either OR and non-OR peptides or bacterially-expressed recombinant PrP. Here we created new transgenic mouse lines expressing PrP with disrupted copper binding sites within all four histidine-containing OR's (sites 1-4, H60G, H68G, H76G, H84G, TetraH>G allele) or at site 5 (composed of residues His-95 and His-110; H95G allele) and monitored the formation of misfolded PrP in vivo. Novel transgenic mice expressing PrP(TetraH>G) at levels comparable to wild-type (wt) controls were susceptible to mouse-adapted scrapie strain RML but showed significantly prolonged incubation times. In contrast, amino acid replacement at residue 95 accelerated disease progression in corresponding PrP(H95G) mice. Neuropathological lesions in terminally ill transgenic mice were similar to scrapie-infected wt controls, but less severe. The pattern of PrPSc deposition, however, was not synaptic as seen in wt animals, but instead dense globular plaque-like accumulations of PrPSc in TgPrP(TetraH>G) mice and diffuse PrPSc deposition in (TgPrP(H95G) mice), were observed throughout all brain sections. We conclude that OR and site 5 histidine substitutions have divergent phenotypic impacts and that cis interactions between the OR region and the site 5 region modulate pathogenic outcomes by affecting the PrP globular domain." @default.
• W2775683821 created "2017-12-22" @default.
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• W2775683821 date "2017-12-08" @default.
• W2775683821 modified "2023-10-06" @default.
• W2775683821 title "Substitutions of PrP N-terminal histidine residues modulate scrapie disease pathogenesis and incubation time in transgenic mice" @default.
• W2775683821 cites W1502562397 @default.
• W2775683821 cites W1559685724 @default.
• W2775683821 cites W1563777667 @default.
• W2775683821 cites W1574440508 @default.
• W2775683821 cites W1574856056 @default.
• W2775683821 cites W1624018543 @default.
• W2775683821 cites W1760677314 @default.
• W2775683821 cites W1784563469 @default.
• W2775683821 cites W1964352617 @default.
• W2775683821 cites W1965265620 @default.
• W2775683821 cites W1970161699 @default.
• W2775683821 cites W1975075866 @default.
• W2775683821 cites W1983345119 @default.
• W2775683821 cites W1985384585 @default.
• W2775683821 cites W1986344430 @default.
• W2775683821 cites W1990497827 @default.
• W2775683821 cites W1991644458 @default.
• W2775683821 cites W1992084965 @default.
• W2775683821 cites W1992380356 @default.
• W2775683821 cites W1996648035 @default.
• W2775683821 cites W1996941571 @default.
• W2775683821 cites W2004994031 @default.
• W2775683821 cites W2009092503 @default.
• W2775683821 cites W2009266044 @default.
• W2775683821 cites W2009608116 @default.
• W2775683821 cites W2012128918 @default.
• W2775683821 cites W2013618043 @default.
• W2775683821 cites W2016170408 @default.
• W2775683821 cites W2019066837 @default.
• W2775683821 cites W2020787157 @default.
• W2775683821 cites W2023338142 @default.
• W2775683821 cites W2023918463 @default.
• W2775683821 cites W2028107568 @default.
• W2775683821 cites W2028172736 @default.
• W2775683821 cites W2028186280 @default.
• W2775683821 cites W2028345570 @default.
• W2775683821 cites W2030091053 @default.
• W2775683821 cites W2031682941 @default.
• W2775683821 cites W2032264036 @default.
• W2775683821 cites W2034655619 @default.
• W2775683821 cites W2037138700 @default.
• W2775683821 cites W2050369906 @default.
• W2775683821 cites W2053263337 @default.
• W2775683821 cites W2057396616 @default.
• W2775683821 cites W2059935249 @default.
• W2775683821 cites W2064305178 @default.
• W2775683821 cites W2064568495 @default.
• W2775683821 cites W2070515057 @default.
• W2775683821 cites W2070613523 @default.
• W2775683821 cites W2071991805 @default.
• W2775683821 cites W2074824082 @default.
• W2775683821 cites W2077994571 @default.
• W2775683821 cites W2079291061 @default.
• W2775683821 cites W2081327351 @default.
• W2775683821 cites W2083050608 @default.
• W2775683821 cites W2083160536 @default.
• W2775683821 cites W2083275855 @default.
• W2775683821 cites W2088178594 @default.
• W2775683821 cites W2091486566 @default.
• W2775683821 cites W2101783641 @default.
• W2775683821 cites W2110946337 @default.
• W2775683821 cites W2111531366 @default.
• W2775683821 cites W2117016125 @default.
• W2775683821 cites W2119837637 @default.
• W2775683821 cites W2124577524 @default.
• W2775683821 cites W2141663125 @default.
• W2775683821 cites W2143795630 @default.
• W2775683821 cites W2147276565 @default.
• W2775683821 cites W2154027377 @default.
• W2775683821 cites W2155389749 @default.
• W2775683821 cites W2159768773 @default.
• W2775683821 cites W2167626370 @default.
• W2775683821 cites W2171526610 @default.
• W2775683821 cites W2324660492 @default.
• W2775683821 cites W2409802975 @default.
• W2775683821 cites W2468830870 @default.
• W2775683821 cites W4247351534 @default.

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