FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2471935916> ?p ?o ?g. }

• W2471935916 endingPage "624" @default.
• W2471935916 startingPage "611" @default.
• W2471935916 abstract "Mammalian prions are unusual infectious agents, as they are thought to consist solely of aggregates of misfolded prion protein (PrP). Generation of synthetic prions, composed of recombinant PrP (recPrP) refolded into fibrils, has been utilised to address whether PrP aggregates are, indeed, infectious prions. In several reports, neurological disease similar to transmissible spongiform encephalopathy (TSE) has been described following inoculation and passage of various forms of fibrils in transgenic mice and hamsters. However, in studies described here, we show that inoculation of recPrP fibrils does not cause TSE disease, but, instead, seeds the formation of PrP amyloid plaques in PrP-P101L knock-in transgenic mice (101LL). Importantly, both WT-recPrP fibrils and 101L-recPrP fibrils can seed plaque formation, indicating that the fibrillar conformation, and not the primary sequence of PrP in the inoculum, is important in initiating seeding. No replication of infectious prions or TSE disease was observed following both primary inoculation and subsequent subpassage. These data, therefore, argue against recPrP fibrils being infectious prions and, instead, indicate that these pre-formed seeds are acting to accelerate the formation of PrP amyloid plaques in 101LL Tg mice. In addition, these data reproduce a phenotype which was previously observed in 101LL mice following inoculation with brain extract containing in vivo-generated PrP amyloid fibrils, which has not been shown for other synthetic prion models. These data are reminiscent of the “prion-like” spread of aggregated forms of the beta-amyloid peptide (Aβ), α-synuclein and tau observed following inoculation of transgenic mice with pre-formed seeds of each misfolded protein. Hence, even when the protein is PrP, misfolding and aggregation do not reproduce the full clinicopathological phenotype of disease. The initiation and spread of protein aggregation in transgenic mouse lines following inoculation with pre-formed fibrils may, therefore, more closely resemble a seeded proteinopathy than an infectious TSE disease." @default.
• W2471935916 created "2016-07-22" @default.
• W2471935916 creator A5016615089 @default.
• W2471935916 creator A5017945971 @default.
• W2471935916 creator A5023524926 @default.
• W2471935916 creator A5028700274 @default.
• W2471935916 creator A5059364858 @default.
• W2471935916 creator A5065812402 @default.
• W2471935916 creator A5083887972 @default.
• W2471935916 date "2016-07-04" @default.
• W2471935916 modified "2023-10-11" @default.
• W2471935916 title "PrP aggregation can be seeded by pre-formed recombinant PrP amyloid fibrils without the replication of infectious prions" @default.
• W2471935916 cites W1493986974 @default.
• W2471935916 cites W1557724928 @default.
• W2471935916 cites W1584834830 @default.
• W2471935916 cites W1846679411 @default.
• W2471935916 cites W1928837524 @default.
• W2471935916 cites W1965235990 @default.
• W2471935916 cites W1967261289 @default.
• W2471935916 cites W1980156175 @default.
• W2471935916 cites W1982797857 @default.
• W2471935916 cites W1992675300 @default.
• W2471935916 cites W1996714383 @default.
• W2471935916 cites W1998949210 @default.
• W2471935916 cites W1998974062 @default.
• W2471935916 cites W2002320632 @default.
• W2471935916 cites W2006103103 @default.
• W2471935916 cites W2011476386 @default.
• W2471935916 cites W2013400360 @default.
• W2471935916 cites W2013679718 @default.
• W2471935916 cites W2014062349 @default.
• W2471935916 cites W2015075898 @default.
• W2471935916 cites W2019061095 @default.
• W2471935916 cites W2020258831 @default.
• W2471935916 cites W2021034011 @default.
• W2471935916 cites W2022051519 @default.
• W2471935916 cites W2027937601 @default.
• W2471935916 cites W2034504118 @default.
• W2471935916 cites W2034655619 @default.
• W2471935916 cites W2036078080 @default.
• W2471935916 cites W2037138700 @default.
• W2471935916 cites W2041939047 @default.
• W2471935916 cites W2043565278 @default.
• W2471935916 cites W2043936774 @default.
• W2471935916 cites W2047555789 @default.
• W2471935916 cites W2052317760 @default.
• W2471935916 cites W2054181870 @default.
• W2471935916 cites W2054314658 @default.
• W2471935916 cites W2055346376 @default.
• W2471935916 cites W2057293794 @default.
• W2471935916 cites W2063198655 @default.
• W2471935916 cites W2070217278 @default.
• W2471935916 cites W2071941241 @default.
• W2471935916 cites W2074199271 @default.
• W2471935916 cites W2076016709 @default.
• W2471935916 cites W2076844276 @default.
• W2471935916 cites W2078025540 @default.
• W2471935916 cites W2082438097 @default.
• W2471935916 cites W2090486549 @default.
• W2471935916 cites W2098022922 @default.
• W2471935916 cites W2099093150 @default.
• W2471935916 cites W2099340107 @default.
• W2471935916 cites W2107037134 @default.
• W2471935916 cites W2115362514 @default.
• W2471935916 cites W2125507585 @default.
• W2471935916 cites W2130240156 @default.
• W2471935916 cites W2131082569 @default.
• W2471935916 cites W2138889916 @default.
• W2471935916 cites W2143291306 @default.
• W2471935916 cites W2149005426 @default.
• W2471935916 cites W2156228052 @default.
• W2471935916 cites W2158089391 @default.
• W2471935916 cites W2165827032 @default.
• W2471935916 cites W2165894129 @default.
• W2471935916 cites W2169849112 @default.
• W2471935916 cites W2172007047 @default.
• W2471935916 cites W2186568579 @default.
• W2471935916 cites W2199476661 @default.
• W2471935916 cites W2259779111 @default.
• W2471935916 cites W2304136784 @default.
• W2471935916 cites W2408079270 @default.
• W2471935916 cites W2409100080 @default.
• W2471935916 cites W2424562508 @default.
• W2471935916 cites W2781304806 @default.
• W2471935916 doi "https://doi.org/10.1007/s00401-016-1594-5" @default.
• W2471935916 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5023723" @default.
• W2471935916 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/27376534" @default.
• W2471935916 hasPublicationYear "2016" @default.
• W2471935916 type Work @default.
• W2471935916 sameAs 2471935916 @default.
• W2471935916 citedByCount "28" @default.
• W2471935916 countsByYear W24719359162016 @default.
• W2471935916 countsByYear W24719359162017 @default.
• W2471935916 countsByYear W24719359162018 @default.
• W2471935916 countsByYear W24719359162019 @default.
• W2471935916 countsByYear W24719359162020 @default.
• W2471935916 countsByYear W24719359162021 @default.
• W2471935916 countsByYear W24719359162022 @default.

• next