guest ::
| Home > Publications Database > Deregulated Splicing Is a Major Mechanism of RNA-Induced Toxicity in Huntington's Disease. > print |
| Home > Publications Database > Deregulated Splicing Is a Major Mechanism of RNA-Induced Toxicity in Huntington's Disease. > print |
| 001 | 140674 | ||
| 005 | 20240321220843.0 | ||
| 024 | 7 | _ | |a 10.1016/j.jmb.2019.01.034 |2 doi |
| 024 | 7 | _ | |a pmid:30711541 |2 pmid |
| 024 | 7 | _ | |a 0022-2836 |2 ISSN |
| 024 | 7 | _ | |a 1089-8638 |2 ISSN |
| 024 | 7 | _ | |a altmetric:54814099 |2 altmetric |
| 037 | _ | _ | |a DZNE-2020-06996 |
| 041 | _ | _ | |a English |
| 082 | _ | _ | |a 610 |
| 100 | 1 | _ | |a Schilling, Judith |0 P:(DE-2719)2810643 |b 0 |e First author |u dzne |
| 245 | _ | _ | |a Deregulated Splicing Is a Major Mechanism of RNA-Induced Toxicity in Huntington's Disease. |
| 260 | _ | _ | |a Amsterdam [u.a.] |c 2019 |b Elsevier |
| 264 | _ | 1 | |3 print |2 Crossref |b Elsevier BV |c 2019-04-01 |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1685011861_32155 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
| 336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
| 336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
| 520 | _ | _ | |a Huntington's disease (HD) is caused by an expanded CAG repeat in the huntingtin (HTT) gene, translating into an elongated polyglutamine stretch. In addition to the neurotoxic mutant HTT protein, the mutant CAG repeat RNA can exert toxic functions by trapping RNA-binding proteins. While few examples of proteins that aberrantly bind to mutant HTT RNA and execute abnormal function in conjunction with the CAG repeat RNA have been described, an unbiased approach to identify the interactome of mutant HTT RNA is missing. Here, we describe the analysis of proteins that preferentially bind mutant HTT RNA using a mass spectrometry approach. We show that (I) the majority of proteins captured by mutant HTT RNA belong to the spliceosome pathway, (II) expression of mutant CAG repeat RNA induces mis-splicing in a HD cell model, (III) overexpression of one of the splice factors trapped by mutant HTT ameliorates the HD phenotype in a fly model and (VI) deregulated splicing occurs in human HD brain. Our data suggest that deregulated splicing is a prominent mechanism of RNA-induced toxicity in HD. |
| 536 | _ | _ | |a 342 - Disease Mechanisms and Model Systems (POF3-342) |0 G:(DE-HGF)POF3-342 |c POF3-342 |f POF III |x 0 |
| 536 | _ | _ | |a 341 - Molecular Signaling (POF3-341) |0 G:(DE-HGF)POF3-341 |c POF3-341 |f POF III |x 1 |
| 542 | _ | _ | |i 2019-04-01 |2 Crossref |u https://www.elsevier.com/tdm/userlicense/1.0/ |
| 542 | _ | _ | |i 2019-02-02 |2 Crossref |u http://creativecommons.org/licenses/by-nc-nd/4.0/ |
| 588 | _ | _ | |a Dataset connected to CrossRef, PubMed, |
| 650 | _ | 2 | |a Animals |2 MeSH |
| 650 | _ | 2 | |a Humans |2 MeSH |
| 650 | _ | 2 | |a Huntingtin Protein: genetics |2 MeSH |
| 650 | _ | 2 | |a Huntington Disease: genetics |2 MeSH |
| 650 | _ | 2 | |a RNA: genetics |2 MeSH |
| 650 | _ | 2 | |a RNA Splicing: genetics |2 MeSH |
| 650 | _ | 2 | |a Spliceosomes: genetics |2 MeSH |
| 700 | 1 | _ | |a Broemer, Meike |0 P:(DE-2719)2810307 |b 1 |u dzne |
| 700 | 1 | _ | |a Atanassov, Ilian |b 2 |
| 700 | 1 | _ | |a Dürnberger, Yvonne |0 P:(DE-2719)2810519 |b 3 |u dzne |
| 700 | 1 | _ | |a Vorberg, Ina |0 P:(DE-2719)2481765 |b 4 |u dzne |
| 700 | 1 | _ | |a Dieterich, Christoph |b 5 |
| 700 | 1 | _ | |a Dagane, Alina |b 6 |
| 700 | 1 | _ | |a Dittmar, Gunnar |b 7 |
| 700 | 1 | _ | |a Wanker, Erich |b 8 |
| 700 | 1 | _ | |a van Roon-Mom, Willeke |b 9 |
| 700 | 1 | _ | |a Winter, Jennifer |b 10 |
| 700 | 1 | _ | |a Krauß, Sybille |0 P:(DE-2719)2421562 |b 11 |e Last author |u dzne |
| 773 | 1 | 8 | |a 10.1016/j.jmb.2019.01.034 |b : Elsevier BV, 2019-04-01 |n 9 |p 1869-1877 |3 journal-article |2 Crossref |t Journal of Molecular Biology |v 431 |y 2019 |x 0022-2836 |
| 773 | _ | _ | |a 10.1016/j.jmb.2019.01.034 |g Vol. 431, no. 9, p. 1869 - 1877 |0 PERI:(DE-600)1355192-9 |n 9 |q 431:9<1869 - 1877 |p 1869-1877 |t Journal of molecular biology |v 431 |y 2019 |x 0022-2836 |
| 856 | 4 | _ | |y OpenAccess |u https://pub.dzne.de/record/140674/files/DZNE-2020-06996.pdf |
| 856 | 4 | _ | |y OpenAccess |x pdfa |u https://pub.dzne.de/record/140674/files/DZNE-2020-06996.pdf?subformat=pdfa |
| 909 | C | O | |o oai:pub.dzne.de:140674 |p openaire |p open_access |p VDB |p driver |p dnbdelivery |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 0 |6 P:(DE-2719)2810643 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 1 |6 P:(DE-2719)2810307 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 3 |6 P:(DE-2719)2810519 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 4 |6 P:(DE-2719)2481765 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 11 |6 P:(DE-2719)2421562 |
| 913 | 1 | _ | |a DE-HGF |b Gesundheit |l Erkrankungen des Nervensystems |1 G:(DE-HGF)POF3-340 |0 G:(DE-HGF)POF3-342 |3 G:(DE-HGF)POF3 |2 G:(DE-HGF)POF3-300 |4 G:(DE-HGF)POF |v Disease Mechanisms and Model Systems |x 0 |
| 913 | 1 | _ | |a DE-HGF |b Gesundheit |l Erkrankungen des Nervensystems |1 G:(DE-HGF)POF3-340 |0 G:(DE-HGF)POF3-341 |3 G:(DE-HGF)POF3 |2 G:(DE-HGF)POF3-300 |4 G:(DE-HGF)POF |v Molecular Signaling |x 1 |
| 914 | 1 | _ | |y 2019 |
| 915 | _ | _ | |a Creative Commons Attribution-NonCommercial-NoDerivs CC BY-NC-ND 4.0 |0 LIC:(DE-HGF)CCBYNCND4 |2 HGFVOC |
| 915 | _ | _ | |a OpenAccess |0 StatID:(DE-HGF)0510 |2 StatID |
| 915 | _ | _ | |a Nationallizenz |0 StatID:(DE-HGF)0420 |2 StatID |d 2022-11-08 |w ger |
| 915 | _ | _ | |a JCR |0 StatID:(DE-HGF)0100 |2 StatID |b J MOL BIOL : 2021 |d 2022-11-08 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0200 |2 StatID |b SCOPUS |d 2022-11-08 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0300 |2 StatID |b Medline |d 2022-11-08 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0600 |2 StatID |b Ebsco Academic Search |d 2022-11-08 |
| 915 | _ | _ | |a Peer Review |0 StatID:(DE-HGF)0030 |2 StatID |b ASC |d 2022-11-08 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0199 |2 StatID |b Clarivate Analytics Master Journal List |d 2022-11-08 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0150 |2 StatID |b Web of Science Core Collection |d 2022-11-08 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)1050 |2 StatID |b BIOSIS Previews |d 2022-11-08 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)1030 |2 StatID |b Current Contents - Life Sciences |d 2022-11-08 |
| 915 | _ | _ | |a IF >= 5 |0 StatID:(DE-HGF)9905 |2 StatID |b J MOL BIOL : 2021 |d 2022-11-08 |
| 920 | 1 | _ | |0 I:(DE-2719)1011006 |k AG Krauß ; AG Krauß |l Regulatory RNA-protein interaction in neurodegenerative diseases |x 0 |
| 920 | 1 | _ | |0 I:(DE-2719)5000021 |k AG Brömer 1 ; AG Brömer 1 |l Ubiquitin-Mediated Processes in Neurodegenerative Diseases |x 1 |
| 920 | 1 | _ | |0 I:(DE-2719)1013004 |k AG Vorberg |l Prion Cell Biology |x 2 |
| 980 | _ | _ | |a journal |
| 980 | _ | _ | |a VDB |
| 980 | _ | _ | |a UNRESTRICTED |
| 980 | _ | _ | |a I:(DE-2719)1011006 |
| 980 | _ | _ | |a I:(DE-2719)5000021 |
| 980 | _ | _ | |a I:(DE-2719)1013004 |
| 980 | 1 | _ | |a FullTexts |
| Library | Collection | CLSMajor | CLSMinor | Language | Author |
|---|