Matches in SemOpenAlex for { <https://semopenalex.org/work/W2605283453> ?p ?o ?g. }
- W2605283453 endingPage "57.e4" @default.
- W2605283453 startingPage "48" @default.
- W2605283453 abstract "Onset of neurodegenerative disorders, including Huntington's disease, is strongly influenced by aging. Hallmarks of aged cells include compromised nuclear envelope integrity, impaired nucleocytoplasmic transport, and accumulation of DNA double-strand breaks. We show that mutant huntingtin markedly accelerates all of these cellular phenotypes in a dose- and age-dependent manner in cortex and striatum of mice. Huntingtin-linked polyglutamine initially accumulates in nuclei, leading to disruption of nuclear envelope architecture, partial sequestration of factors essential for nucleocytoplasmic transport (Gle1 and RanGAP1), and intranuclear accumulation of mRNA. In aged mice, accumulation of RanGAP1 together with polyglutamine is shifted to perinuclear and cytoplasmic areas. Consistent with findings in mice, marked alterations in nuclear envelope morphology, abnormal localization of RanGAP1, and nuclear accumulation of mRNA were found in cortex of Huntington's disease patients. Overall, our findings identify polyglutamine-dependent inhibition of nucleocytoplasmic transport and alteration of nuclear integrity as a central component of Huntington's disease." @default.
- W2605283453 created "2017-04-14" @default.
- W2605283453 creator A5004331062 @default.
- W2605283453 creator A5004532456 @default.
- W2605283453 creator A5019496408 @default.
- W2605283453 creator A5030584160 @default.
- W2605283453 creator A5033998542 @default.
- W2605283453 creator A5036604750 @default.
- W2605283453 creator A5048591919 @default.
- W2605283453 creator A5060941872 @default.
- W2605283453 creator A5076687967 @default.
- W2605283453 creator A5079372873 @default.
- W2605283453 date "2017-04-01" @default.
- W2605283453 modified "2023-10-17" @default.
- W2605283453 title "Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport" @default.
- W2605283453 cites W1137678651 @default.
- W2605283453 cites W1497938915 @default.
- W2605283453 cites W1796096082 @default.
- W2605283453 cites W1797572428 @default.
- W2605283453 cites W1806909159 @default.
- W2605283453 cites W1971803508 @default.
- W2605283453 cites W1975707356 @default.
- W2605283453 cites W1979176029 @default.
- W2605283453 cites W1983315505 @default.
- W2605283453 cites W1985775217 @default.
- W2605283453 cites W1987140385 @default.
- W2605283453 cites W1992921611 @default.
- W2605283453 cites W1997887740 @default.
- W2605283453 cites W2024097058 @default.
- W2605283453 cites W2024729521 @default.
- W2605283453 cites W2032556660 @default.
- W2605283453 cites W2033318695 @default.
- W2605283453 cites W2035777213 @default.
- W2605283453 cites W2042716629 @default.
- W2605283453 cites W2042742939 @default.
- W2605283453 cites W2048323087 @default.
- W2605283453 cites W2062682951 @default.
- W2605283453 cites W2068247002 @default.
- W2605283453 cites W2068275936 @default.
- W2605283453 cites W2068434142 @default.
- W2605283453 cites W2072810104 @default.
- W2605283453 cites W2073466689 @default.
- W2605283453 cites W2075639278 @default.
- W2605283453 cites W2080777776 @default.
- W2605283453 cites W2088778610 @default.
- W2605283453 cites W2091241169 @default.
- W2605283453 cites W2091874767 @default.
- W2605283453 cites W2096587251 @default.
- W2605283453 cites W2102872810 @default.
- W2605283453 cites W2108575147 @default.
- W2605283453 cites W2113963642 @default.
- W2605283453 cites W2116449089 @default.
- W2605283453 cites W2126214044 @default.
- W2605283453 cites W2126525177 @default.
- W2605283453 cites W2134829658 @default.
- W2605283453 cites W2136367337 @default.
- W2605283453 cites W2136721455 @default.
- W2605283453 cites W2150460967 @default.
- W2605283453 cites W2150523249 @default.
- W2605283453 cites W2152165298 @default.
- W2605283453 cites W2153353641 @default.
- W2605283453 cites W2154945114 @default.
- W2605283453 cites W2171136950 @default.
- W2605283453 cites W2181224463 @default.
- W2605283453 cites W2203120038 @default.
- W2605283453 cites W2219462594 @default.
- W2605283453 cites W2239611834 @default.
- W2605283453 cites W2308742329 @default.
- W2605283453 cites W2335878205 @default.
- W2605283453 cites W2484951111 @default.
- W2605283453 cites W2533664860 @default.
- W2605283453 cites W2569387537 @default.
- W2605283453 cites W890895450 @default.
- W2605283453 doi "https://doi.org/10.1016/j.neuron.2017.03.027" @default.
- W2605283453 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5479704" @default.
- W2605283453 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/28384474" @default.
- W2605283453 hasPublicationYear "2017" @default.
- W2605283453 type Work @default.
- W2605283453 sameAs 2605283453 @default.
- W2605283453 citedByCount "176" @default.
- W2605283453 countsByYear W26052834532017 @default.
- W2605283453 countsByYear W26052834532018 @default.
- W2605283453 countsByYear W26052834532019 @default.
- W2605283453 countsByYear W26052834532020 @default.
- W2605283453 countsByYear W26052834532021 @default.
- W2605283453 countsByYear W26052834532022 @default.
- W2605283453 countsByYear W26052834532023 @default.
- W2605283453 crossrefType "journal-article" @default.
- W2605283453 hasAuthorship W2605283453A5004331062 @default.
- W2605283453 hasAuthorship W2605283453A5004532456 @default.
- W2605283453 hasAuthorship W2605283453A5019496408 @default.
- W2605283453 hasAuthorship W2605283453A5030584160 @default.
- W2605283453 hasAuthorship W2605283453A5033998542 @default.
- W2605283453 hasAuthorship W2605283453A5036604750 @default.
- W2605283453 hasAuthorship W2605283453A5048591919 @default.
- W2605283453 hasAuthorship W2605283453A5060941872 @default.
- W2605283453 hasAuthorship W2605283453A5076687967 @default.
- W2605283453 hasAuthorship W2605283453A5079372873 @default.