SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits
The RNA for ALS- and frontotemporal dementia-associated C9ORF72 gene is exported from nucleus via an unknown mechanism. This study shows that reduction of nuclear export adaptor SRSF1 can alleviate neuronal cell death and nuclear export of C9ORF72 inDrosophilaand patient-derived induced motor neuron...
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2017
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oai:doaj.org-article:26d01605fb7344faaa67f88fe2c80e8f2021-12-02T14:41:19ZSRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits10.1038/ncomms160632041-1723https://doaj.org/article/26d01605fb7344faaa67f88fe2c80e8f2017-07-01T00:00:00Zhttps://doi.org/10.1038/ncomms16063https://doaj.org/toc/2041-1723The RNA for ALS- and frontotemporal dementia-associated C9ORF72 gene is exported from nucleus via an unknown mechanism. This study shows that reduction of nuclear export adaptor SRSF1 can alleviate neuronal cell death and nuclear export of C9ORF72 inDrosophilaand patient-derived induced motor neurons.Guillaume M. HautbergueLydia M. CastelliLaura FerraiuoloAlvaro Sanchez-MartinezJohnathan Cooper-KnockAdrian HigginbottomYa-Hui LinClaudia S. BauerJennifer E. DoddMonika A. MyszczynskaSarah M. AlamPierre GarneretJayanth S. ChandranEvangelia KarykaMatthew J. StopfordEmma F. SmithJanine KirbyKathrin MeyerBrian K. KasparAdrian M. IsaacsSherif F. El-KhamisyKurt J. De VosKe NingMimoun AzzouzAlexander J. WhitworthPamela J. ShawNature PortfolioarticleScienceQENNature Communications, Vol 8, Iss 1, Pp 1-18 (2017) |
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Science Q Guillaume M. Hautbergue Lydia M. Castelli Laura Ferraiuolo Alvaro Sanchez-Martinez Johnathan Cooper-Knock Adrian Higginbottom Ya-Hui Lin Claudia S. Bauer Jennifer E. Dodd Monika A. Myszczynska Sarah M. Alam Pierre Garneret Jayanth S. Chandran Evangelia Karyka Matthew J. Stopford Emma F. Smith Janine Kirby Kathrin Meyer Brian K. Kaspar Adrian M. Isaacs Sherif F. El-Khamisy Kurt J. De Vos Ke Ning Mimoun Azzouz Alexander J. Whitworth Pamela J. Shaw SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits |
| description |
The RNA for ALS- and frontotemporal dementia-associated C9ORF72 gene is exported from nucleus via an unknown mechanism. This study shows that reduction of nuclear export adaptor SRSF1 can alleviate neuronal cell death and nuclear export of C9ORF72 inDrosophilaand patient-derived induced motor neurons. |
| format |
article |
| author |
Guillaume M. Hautbergue Lydia M. Castelli Laura Ferraiuolo Alvaro Sanchez-Martinez Johnathan Cooper-Knock Adrian Higginbottom Ya-Hui Lin Claudia S. Bauer Jennifer E. Dodd Monika A. Myszczynska Sarah M. Alam Pierre Garneret Jayanth S. Chandran Evangelia Karyka Matthew J. Stopford Emma F. Smith Janine Kirby Kathrin Meyer Brian K. Kaspar Adrian M. Isaacs Sherif F. El-Khamisy Kurt J. De Vos Ke Ning Mimoun Azzouz Alexander J. Whitworth Pamela J. Shaw |
| author_facet |
Guillaume M. Hautbergue Lydia M. Castelli Laura Ferraiuolo Alvaro Sanchez-Martinez Johnathan Cooper-Knock Adrian Higginbottom Ya-Hui Lin Claudia S. Bauer Jennifer E. Dodd Monika A. Myszczynska Sarah M. Alam Pierre Garneret Jayanth S. Chandran Evangelia Karyka Matthew J. Stopford Emma F. Smith Janine Kirby Kathrin Meyer Brian K. Kaspar Adrian M. Isaacs Sherif F. El-Khamisy Kurt J. De Vos Ke Ning Mimoun Azzouz Alexander J. Whitworth Pamela J. Shaw |
| author_sort |
Guillaume M. Hautbergue |
| title |
SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits |
| title_short |
SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits |
| title_full |
SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits |
| title_fullStr |
SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits |
| title_full_unstemmed |
SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits |
| title_sort |
srsf1-dependent nuclear export inhibition of c9orf72 repeat transcripts prevents neurodegeneration and associated motor deficits |
| publisher |
Nature Portfolio |
| publishDate |
2017 |
| url |
https://doaj.org/article/26d01605fb7344faaa67f88fe2c80e8f |
| work_keys_str_mv |
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1718389919196381184 |