FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2082012193> ?p ?o ?g. }

• W2082012193 endingPage "369" @default.
• W2082012193 startingPage "363" @default.
• W2082012193 abstract "For the vast majority of cases of amyotrophic lateral sclerosis (ALS) the etiology remains unknown. After the discovery of missense mutations in the gene coding for the Cu/Zn superoxide dismutase 1 (SOD1) in subsets of familial ALS, several transgenic mouse lines have been generated with various forms of SOD1 mutants overexpressed at different levels. Studies with these mice yielded complex results with multiple targets of damage in disease including mitochondria, proteasomes, and secretory pathways. Many unexpected discoveries were made. For instance, the toxicity of mutant SOD1 seems unrelated to copper-mediated catalysis but rather to formation of misfolded SOD1 species and aggregates. Transgenic studies revealed a potential role of wtSOD1 in exacerbating mutant SOD1-mediated disease. Another key finding came from chimeric mouse studies and from Cre-lox mediated gene deletion experiments which have highlighted the importance of non-neuronal cells in the disease progression. Involvement of cytoskeletal components in ALS pathogenesis is supported by several mouse models of motor neuron disease with neurofilament abnormalities and with genetic defects in microtubule-based transport. Recently, the generation of new animal models of ALS has been made possible with the discovery of ALS-linked mutations in other genes encoding for alsin, dynactin, senataxin, VAPB, TDP-43 and FUS. Following the discovery of mutations in the TARDBP gene linked to ALS, there have been some reports of transgenic mice with high level overexpression of WT or mutant forms of TDP-43 under strong gene promoters. However, these TDP-43 transgenic mice do not exhibit all pathological features the human ALS disease. Here, we will describe these new TDP-43 transgenic mice and discuss their validity as animal models of human ALS." @default.
• W2082012193 created "2016-06-24" @default.
• W2082012193 creator A5048053054 @default.
• W2082012193 creator A5091664460 @default.
• W2082012193 date "2011-03-01" @default.
• W2082012193 modified "2023-10-17" @default.
• W2082012193 title "ALS pathogenesis: Recent insights from genetics and mouse models" @default.
• W2082012193 cites W1525022089 @default.
• W2082012193 cites W1527155761 @default.
• W2082012193 cites W1527585259 @default.
• W2082012193 cites W1619082318 @default.
• W2082012193 cites W1969523250 @default.
• W2082012193 cites W1977399503 @default.
• W2082012193 cites W1977649097 @default.
• W2082012193 cites W1979179514 @default.
• W2082012193 cites W1984979116 @default.
• W2082012193 cites W1985655958 @default.
• W2082012193 cites W1985812526 @default.
• W2082012193 cites W1986234504 @default.
• W2082012193 cites W1989227252 @default.
• W2082012193 cites W1992002234 @default.
• W2082012193 cites W1995185770 @default.
• W2082012193 cites W1996431592 @default.
• W2082012193 cites W1999574352 @default.
• W2082012193 cites W1999638129 @default.
• W2082012193 cites W1999899839 @default.
• W2082012193 cites W2003618511 @default.
• W2082012193 cites W2008563656 @default.
• W2082012193 cites W2009030515 @default.
• W2082012193 cites W2011396520 @default.
• W2082012193 cites W2011575330 @default.
• W2082012193 cites W2012851706 @default.
• W2082012193 cites W2013135033 @default.
• W2082012193 cites W2015266340 @default.
• W2082012193 cites W2017222498 @default.
• W2082012193 cites W2023556666 @default.
• W2082012193 cites W2024153744 @default.
• W2082012193 cites W2025504772 @default.
• W2082012193 cites W2027047473 @default.
• W2082012193 cites W2035397639 @default.
• W2082012193 cites W2041451000 @default.
• W2082012193 cites W2048880814 @default.
• W2082012193 cites W2049135007 @default.
• W2082012193 cites W2053362200 @default.
• W2082012193 cites W2055022577 @default.
• W2082012193 cites W2058546812 @default.
• W2082012193 cites W2069054574 @default.
• W2082012193 cites W2069350252 @default.
• W2082012193 cites W2070086306 @default.
• W2082012193 cites W2071821511 @default.
• W2082012193 cites W2072413533 @default.
• W2082012193 cites W2072978915 @default.
• W2082012193 cites W2074726646 @default.
• W2082012193 cites W2091299458 @default.
• W2082012193 cites W2092729353 @default.
• W2082012193 cites W2093546938 @default.
• W2082012193 cites W2096335797 @default.
• W2082012193 cites W2096386031 @default.
• W2082012193 cites W2097066519 @default.
• W2082012193 cites W2098189071 @default.
• W2082012193 cites W2099071260 @default.
• W2082012193 cites W2100515174 @default.
• W2082012193 cites W2101555677 @default.
• W2082012193 cites W2104483408 @default.
• W2082012193 cites W2107542754 @default.
• W2082012193 cites W2107768418 @default.
• W2082012193 cites W2108240482 @default.
• W2082012193 cites W2109021011 @default.
• W2082012193 cites W2110029962 @default.
• W2082012193 cites W2110289399 @default.
• W2082012193 cites W2110551795 @default.
• W2082012193 cites W2110580010 @default.
• W2082012193 cites W2123939448 @default.
• W2082012193 cites W2124084307 @default.
• W2082012193 cites W2124976615 @default.
• W2082012193 cites W2127861463 @default.
• W2082012193 cites W2130565053 @default.
• W2082012193 cites W2135522980 @default.
• W2082012193 cites W2135607950 @default.
• W2082012193 cites W2136057227 @default.
• W2082012193 cites W2136448211 @default.
• W2082012193 cites W2143366726 @default.
• W2082012193 cites W2144475886 @default.
• W2082012193 cites W2148247362 @default.
• W2082012193 cites W2150090276 @default.
• W2082012193 cites W2157150055 @default.
• W2082012193 cites W2159289924 @default.
• W2082012193 cites W2159556459 @default.
• W2082012193 cites W2160475742 @default.
• W2082012193 cites W2163311553 @default.
• W2082012193 cites W4246573955 @default.
• W2082012193 doi "https://doi.org/10.1016/j.pnpbp.2010.08.006" @default.
• W2082012193 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/20728492" @default.
• W2082012193 hasPublicationYear "2011" @default.
• W2082012193 type Work @default.
• W2082012193 sameAs 2082012193 @default.
• W2082012193 citedByCount "47" @default.
• W2082012193 countsByYear W20820121932012 @default.

• next