Matches in SemOpenAlex for { <https://semopenalex.org/work/W2026532100> ?p ?o ?g. }
- W2026532100 abstract "Expansion of polyglutamine (polyQ) tract may cause protein misfolding and aggregation that lead to cytotoxicity and neurodegeneration, but the underlying mechanism remains to be elucidated. We applied ataxin-3 (Atx3), a polyQ tract-containing protein, as a model to study sequestration of normal cellular proteins. We found that the aggregates formed by polyQ-expanded Atx3 sequester its interacting partners, such as P97/VCP and ubiquitin conjugates, into the protein inclusions through specific interactions both in vitro and in cells. Moreover, this specific sequestration impairs the normal cellular function of P97 in down-regulating neddylation. However, expansion of polyQ tract in Atx3 does not alter the conformation of its surrounding regions and the interaction affinities with the interacting partners, although it indeed facilitates misfolding and aggregation of the Atx3 protein. Thus, we propose a loss-of-function pathology for polyQ diseases that sequestration of the cellular essential proteins via specific interactions into inclusions by the polyQ aggregates causes dysfunction of the corresponding proteins, and consequently leads to neurodegeneration." @default.
- W2026532100 created "2016-06-24" @default.
- W2026532100 creator A5005937175 @default.
- W2026532100 creator A5011740024 @default.
- W2026532100 creator A5039213269 @default.
- W2026532100 creator A5047191861 @default.
- W2026532100 creator A5055578352 @default.
- W2026532100 creator A5071784544 @default.
- W2026532100 creator A5091565329 @default.
- W2026532100 date "2014-09-18" @default.
- W2026532100 modified "2023-10-05" @default.
- W2026532100 title "Aggregation of polyglutamine-expanded ataxin-3 sequesters its specific interacting partners into inclusions: Implication in a loss-of-function pathology" @default.
- W2026532100 cites W1970456057 @default.
- W2026532100 cites W1975403755 @default.
- W2026532100 cites W1978124503 @default.
- W2026532100 cites W1982411679 @default.
- W2026532100 cites W1992436832 @default.
- W2026532100 cites W1995877511 @default.
- W2026532100 cites W2002559713 @default.
- W2026532100 cites W2002856540 @default.
- W2026532100 cites W2004879226 @default.
- W2026532100 cites W2005572424 @default.
- W2026532100 cites W2008130030 @default.
- W2026532100 cites W2011637489 @default.
- W2026532100 cites W2012612404 @default.
- W2026532100 cites W2013029120 @default.
- W2026532100 cites W2016217377 @default.
- W2026532100 cites W2024442371 @default.
- W2026532100 cites W2032255653 @default.
- W2026532100 cites W2033088972 @default.
- W2026532100 cites W2037449788 @default.
- W2026532100 cites W2042811939 @default.
- W2026532100 cites W2045183210 @default.
- W2026532100 cites W2060391279 @default.
- W2026532100 cites W2061600637 @default.
- W2026532100 cites W2062203120 @default.
- W2026532100 cites W2064605629 @default.
- W2026532100 cites W2066536601 @default.
- W2026532100 cites W2067760363 @default.
- W2026532100 cites W2072911058 @default.
- W2026532100 cites W2073922528 @default.
- W2026532100 cites W2086735032 @default.
- W2026532100 cites W2090054185 @default.
- W2026532100 cites W2094935682 @default.
- W2026532100 cites W2099141103 @default.
- W2026532100 cites W2104650717 @default.
- W2026532100 cites W2106200048 @default.
- W2026532100 cites W2108570063 @default.
- W2026532100 cites W2109350663 @default.
- W2026532100 cites W2111531780 @default.
- W2026532100 cites W2111700402 @default.
- W2026532100 cites W2113998330 @default.
- W2026532100 cites W2117138295 @default.
- W2026532100 cites W2120715236 @default.
- W2026532100 cites W2120947942 @default.
- W2026532100 cites W2123544060 @default.
- W2026532100 cites W2128341112 @default.
- W2026532100 cites W2128674001 @default.
- W2026532100 cites W2132513906 @default.
- W2026532100 cites W2134802476 @default.
- W2026532100 cites W2136364567 @default.
- W2026532100 cites W2140741960 @default.
- W2026532100 cites W2152351845 @default.
- W2026532100 cites W2158722583 @default.
- W2026532100 cites W2162057703 @default.
- W2026532100 cites W2162194331 @default.
- W2026532100 cites W2162470793 @default.
- W2026532100 cites W2165186407 @default.
- W2026532100 cites W2165844675 @default.
- W2026532100 cites W2167215915 @default.
- W2026532100 cites W2326990520 @default.
- W2026532100 doi "https://doi.org/10.1038/srep06410" @default.
- W2026532100 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5377324" @default.
- W2026532100 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/25231079" @default.
- W2026532100 hasPublicationYear "2014" @default.
- W2026532100 type Work @default.
- W2026532100 sameAs 2026532100 @default.
- W2026532100 citedByCount "95" @default.
- W2026532100 countsByYear W20265321002014 @default.
- W2026532100 countsByYear W20265321002015 @default.
- W2026532100 countsByYear W20265321002016 @default.
- W2026532100 countsByYear W20265321002017 @default.
- W2026532100 countsByYear W20265321002018 @default.
- W2026532100 countsByYear W20265321002019 @default.
- W2026532100 countsByYear W20265321002020 @default.
- W2026532100 countsByYear W20265321002021 @default.
- W2026532100 countsByYear W20265321002022 @default.
- W2026532100 countsByYear W20265321002023 @default.
- W2026532100 crossrefType "journal-article" @default.
- W2026532100 hasAuthorship W2026532100A5005937175 @default.
- W2026532100 hasAuthorship W2026532100A5011740024 @default.
- W2026532100 hasAuthorship W2026532100A5039213269 @default.
- W2026532100 hasAuthorship W2026532100A5047191861 @default.
- W2026532100 hasAuthorship W2026532100A5055578352 @default.
- W2026532100 hasAuthorship W2026532100A5071784544 @default.
- W2026532100 hasAuthorship W2026532100A5091565329 @default.
- W2026532100 hasBestOaLocation W20265321001 @default.
- W2026532100 hasConcept C104317684 @default.
- W2026532100 hasConcept C12554922 @default.
- W2026532100 hasConcept C136238340 @default.