Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

Abnormal cytoplasmic aggregates of FUS are a hallmark of some forms of amyotrophic lateral sclerosis (ALS). Here, using neurons derived from patients with FUS-ALS, the authors demonstrate that impairment of PARP-dependent DNA damage signaling is an event that occurs upstream of neurodegeneration and...

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Autores principales:	Marcel Naumann, Arun Pal, Anand Goswami, Xenia Lojewski, Julia Japtok, Anne Vehlow, Maximilian Naujock, René Günther, Mengmeng Jin, Nancy Stanslowsky, Peter Reinhardt, Jared Sterneckert, Marie Frickenhaus, Francisco Pan-Montojo, Erik Storkebaum, Ina Poser, Axel Freischmidt, Jochen H. Weishaupt, Karlheinz Holzmann, Dirk Troost, Albert C. Ludolph, Tobias M. Boeckers, Stefan Liebau, Susanne Petri, Nils Cordes, Anthony A. Hyman, Florian Wegner, Stephan W. Grill, Joachim Weis, Alexander Storch, Andreas Hermann
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2018
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/421d7fe19126448d9734fe819843c747
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spelling	oai:doaj.org-article:421d7fe19126448d9734fe819843c7472021-12-02T17:32:33ZImpaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation10.1038/s41467-017-02299-12041-1723https://doaj.org/article/421d7fe19126448d9734fe819843c7472018-01-01T00:00:00Zhttps://doi.org/10.1038/s41467-017-02299-1https://doaj.org/toc/2041-1723Abnormal cytoplasmic aggregates of FUS are a hallmark of some forms of amyotrophic lateral sclerosis (ALS). Here, using neurons derived from patients with FUS-ALS, the authors demonstrate that impairment of PARP-dependent DNA damage signaling is an event that occurs upstream of neurodegeneration and cytoplasmic aggregate formation in FUS-ALS.Marcel NaumannArun PalAnand GoswamiXenia LojewskiJulia JaptokAnne VehlowMaximilian NaujockRené GüntherMengmeng JinNancy StanslowskyPeter ReinhardtJared SterneckertMarie FrickenhausFrancisco Pan-MontojoErik StorkebaumIna PoserAxel FreischmidtJochen H. WeishauptKarlheinz HolzmannDirk TroostAlbert C. LudolphTobias M. BoeckersStefan LiebauSusanne PetriNils CordesAnthony A. HymanFlorian WegnerStephan W. GrillJoachim WeisAlexander StorchAndreas HermannNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-17 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Marcel Naumann Arun Pal Anand Goswami Xenia Lojewski Julia Japtok Anne Vehlow Maximilian Naujock René Günther Mengmeng Jin Nancy Stanslowsky Peter Reinhardt Jared Sterneckert Marie Frickenhaus Francisco Pan-Montojo Erik Storkebaum Ina Poser Axel Freischmidt Jochen H. Weishaupt Karlheinz Holzmann Dirk Troost Albert C. Ludolph Tobias M. Boeckers Stefan Liebau Susanne Petri Nils Cordes Anthony A. Hyman Florian Wegner Stephan W. Grill Joachim Weis Alexander Storch Andreas Hermann Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation
description	Abnormal cytoplasmic aggregates of FUS are a hallmark of some forms of amyotrophic lateral sclerosis (ALS). Here, using neurons derived from patients with FUS-ALS, the authors demonstrate that impairment of PARP-dependent DNA damage signaling is an event that occurs upstream of neurodegeneration and cytoplasmic aggregate formation in FUS-ALS.
format	article
author	Marcel Naumann Arun Pal Anand Goswami Xenia Lojewski Julia Japtok Anne Vehlow Maximilian Naujock René Günther Mengmeng Jin Nancy Stanslowsky Peter Reinhardt Jared Sterneckert Marie Frickenhaus Francisco Pan-Montojo Erik Storkebaum Ina Poser Axel Freischmidt Jochen H. Weishaupt Karlheinz Holzmann Dirk Troost Albert C. Ludolph Tobias M. Boeckers Stefan Liebau Susanne Petri Nils Cordes Anthony A. Hyman Florian Wegner Stephan W. Grill Joachim Weis Alexander Storch Andreas Hermann
author_facet	Marcel Naumann Arun Pal Anand Goswami Xenia Lojewski Julia Japtok Anne Vehlow Maximilian Naujock René Günther Mengmeng Jin Nancy Stanslowsky Peter Reinhardt Jared Sterneckert Marie Frickenhaus Francisco Pan-Montojo Erik Storkebaum Ina Poser Axel Freischmidt Jochen H. Weishaupt Karlheinz Holzmann Dirk Troost Albert C. Ludolph Tobias M. Boeckers Stefan Liebau Susanne Petri Nils Cordes Anthony A. Hyman Florian Wegner Stephan W. Grill Joachim Weis Alexander Storch Andreas Hermann
author_sort	Marcel Naumann
title	Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation
title_short	Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation
title_full	Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation
title_fullStr	Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation
title_full_unstemmed	Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation
title_sort	impaired dna damage response signaling by fus-nls mutations leads to neurodegeneration and fus aggregate formation
publisher	Nature Portfolio
publishDate	2018
url	https://doaj.org/article/421d7fe19126448d9734fe819843c747
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Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

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