Matches in SemOpenAlex for { <https://semopenalex.org/work/W2022705758> ?p ?o ?g. }
- W2022705758 endingPage "1948" @default.
- W2022705758 startingPage "1935" @default.
- W2022705758 abstract "Familial amyloidotic polyneuropathy (FAP) is a lethal autosomal dominant disorder characterized by systemic extracellular deposition of transthyretin (TTR) amyloid fibrils. Several groups have generated transgenic mice carrying human TTR Val30Met, the most common mutation in FAP. To study amyloidogenicity and cytotoxicity of different TTRs, we produced transgenic mice expressing human TTR Leu55Pro, one of the most aggressive FAP-related mutations. TTR deposition and presence of amyloid fibrils was investigated and compared to animals carrying the human TTR Val30Met gene kept under the same conditions. Deposition in a C57BL/6J background (TTR-Leu55Pro mice) and in a TTR-null background [TTR-Leu55Pro X TTR-knockout (KO) mice] was compared. Animals in a C57BL/6J background presented early (1 to 3 months) nonfibrillar TTR deposition but amyloid was absent. In a TTR-null background, presence of amyloid fibrils was detected starting at 4 to 8 months with a particular involvement of the gastrointestinal tract and skin. This data suggested that TTR homotetramers are more prone to fibril formation than TTR murine wild-type/human mutant heterotetramers. The nature of the deposited material was further investigated by immunocytochemistry. Both amorphous aggregates and small TTR fibrils were present in TTR-Leu55Pro X TTR-KO transgenics. We observed that these TTR deposits mimic the toxic effect of TTR deposits in FAP: animals with TTR deposition, present approximately twofold increased levels of nitrotyrosine in sites related to deposition. The TTR-Leu55Pro X TTR-KO mice here described are an important tool for the dual purpose of investigating factors involved in amyloidogenesis and in cytotoxicity of deposited TTR." @default.
- W2022705758 created "2016-06-24" @default.
- W2022705758 creator A5002856605 @default.
- W2022705758 creator A5011688843 @default.
- W2022705758 creator A5028488158 @default.
- W2022705758 creator A5034241498 @default.
- W2022705758 creator A5046903959 @default.
- W2022705758 creator A5062406788 @default.
- W2022705758 date "2002-11-01" @default.
- W2022705758 modified "2023-10-10" @default.
- W2022705758 title "Evidence for Early Cytotoxic Aggregates in Transgenic Mice for Human Transthyretin Leu55Pro" @default.
- W2022705758 cites W1522890520 @default.
- W2022705758 cites W1585658616 @default.
- W2022705758 cites W1662308449 @default.
- W2022705758 cites W1968283357 @default.
- W2022705758 cites W1973784131 @default.
- W2022705758 cites W1975536578 @default.
- W2022705758 cites W1977191559 @default.
- W2022705758 cites W1977195971 @default.
- W2022705758 cites W1981570625 @default.
- W2022705758 cites W1982652047 @default.
- W2022705758 cites W1983789627 @default.
- W2022705758 cites W1985918137 @default.
- W2022705758 cites W1990943598 @default.
- W2022705758 cites W2009556855 @default.
- W2022705758 cites W2016370501 @default.
- W2022705758 cites W2019264979 @default.
- W2022705758 cites W2027832362 @default.
- W2022705758 cites W2033738806 @default.
- W2022705758 cites W2033744993 @default.
- W2022705758 cites W2042159493 @default.
- W2022705758 cites W2047427804 @default.
- W2022705758 cites W2061995199 @default.
- W2022705758 cites W2064146660 @default.
- W2022705758 cites W2064309041 @default.
- W2022705758 cites W2066969586 @default.
- W2022705758 cites W2070157434 @default.
- W2022705758 cites W2072738635 @default.
- W2022705758 cites W2084718171 @default.
- W2022705758 cites W2093861673 @default.
- W2022705758 cites W2101341993 @default.
- W2022705758 cites W2118195391 @default.
- W2022705758 cites W2126264030 @default.
- W2022705758 cites W2128022125 @default.
- W2022705758 cites W2129227455 @default.
- W2022705758 cites W2133791055 @default.
- W2022705758 cites W2137399373 @default.
- W2022705758 cites W2141506960 @default.
- W2022705758 cites W2144086680 @default.
- W2022705758 cites W2150999725 @default.
- W2022705758 cites W2155894885 @default.
- W2022705758 cites W2165021720 @default.
- W2022705758 cites W2198986897 @default.
- W2022705758 cites W220969662 @default.
- W2022705758 cites W2212236250 @default.
- W2022705758 cites W2344385595 @default.
- W2022705758 cites W2407993004 @default.
- W2022705758 cites W30870368 @default.
- W2022705758 cites W93954546 @default.
- W2022705758 doi "https://doi.org/10.1016/s0002-9440(10)64469-0" @default.
- W2022705758 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/1850789" @default.
- W2022705758 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/12414539" @default.
- W2022705758 hasPublicationYear "2002" @default.
- W2022705758 type Work @default.
- W2022705758 sameAs 2022705758 @default.
- W2022705758 citedByCount "99" @default.
- W2022705758 countsByYear W20227057582012 @default.
- W2022705758 countsByYear W20227057582013 @default.
- W2022705758 countsByYear W20227057582014 @default.
- W2022705758 countsByYear W20227057582015 @default.
- W2022705758 countsByYear W20227057582016 @default.
- W2022705758 countsByYear W20227057582017 @default.
- W2022705758 countsByYear W20227057582018 @default.
- W2022705758 countsByYear W20227057582019 @default.
- W2022705758 countsByYear W20227057582020 @default.
- W2022705758 countsByYear W20227057582021 @default.
- W2022705758 countsByYear W20227057582022 @default.
- W2022705758 countsByYear W20227057582023 @default.
- W2022705758 crossrefType "journal-article" @default.
- W2022705758 hasAuthorship W2022705758A5002856605 @default.
- W2022705758 hasAuthorship W2022705758A5011688843 @default.
- W2022705758 hasAuthorship W2022705758A5028488158 @default.
- W2022705758 hasAuthorship W2022705758A5034241498 @default.
- W2022705758 hasAuthorship W2022705758A5046903959 @default.
- W2022705758 hasAuthorship W2022705758A5062406788 @default.
- W2022705758 hasBestOaLocation W20227057581 @default.
- W2022705758 hasConcept C102230213 @default.
- W2022705758 hasConcept C104317684 @default.
- W2022705758 hasConcept C121157162 @default.
- W2022705758 hasConcept C134018914 @default.
- W2022705758 hasConcept C141035611 @default.
- W2022705758 hasConcept C142724271 @default.
- W2022705758 hasConcept C153911025 @default.
- W2022705758 hasConcept C185592680 @default.
- W2022705758 hasConcept C203014093 @default.
- W2022705758 hasConcept C27523624 @default.
- W2022705758 hasConcept C2776156784 @default.
- W2022705758 hasConcept C2777633098 @default.