ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

The mechanism by which FUS mutations cause familial ALS remains unclear. Here, the authors use mouse transgenic models to show that a toxic gain-of-function underlies motor neuron degeneration, and that the toxicity of mutant FUS does not depend on a loss or excess of FUS activity.

Guardado en:

Detalles Bibliográficos
Autores principales:	Aarti Sharma, Alexander K. Lyashchenko, Lei Lu, Sara Ebrahimi Nasrabady, Margot Elmaleh, Monica Mendelsohn, Adriana Nemes, Juan Carlos Tapia, George Z. Mentis, Neil A. Shneider
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2016
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/320664c72a834d799df983f0633c7d56
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:320664c72a834d799df983f0633c7d56
record_format	dspace
spelling	oai:doaj.org-article:320664c72a834d799df983f0633c7d562021-12-02T16:49:57ZALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function10.1038/ncomms104652041-1723https://doaj.org/article/320664c72a834d799df983f0633c7d562016-02-01T00:00:00Zhttps://doi.org/10.1038/ncomms10465https://doaj.org/toc/2041-1723The mechanism by which FUS mutations cause familial ALS remains unclear. Here, the authors use mouse transgenic models to show that a toxic gain-of-function underlies motor neuron degeneration, and that the toxicity of mutant FUS does not depend on a loss or excess of FUS activity.Aarti SharmaAlexander K. LyashchenkoLei LuSara Ebrahimi NasrabadyMargot ElmalehMonica MendelsohnAdriana NemesJuan Carlos TapiaGeorge Z. MentisNeil A. ShneiderNature PortfolioarticleScienceQENNature Communications, Vol 7, Iss 1, Pp 1-14 (2016)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Aarti Sharma Alexander K. Lyashchenko Lei Lu Sara Ebrahimi Nasrabady Margot Elmaleh Monica Mendelsohn Adriana Nemes Juan Carlos Tapia George Z. Mentis Neil A. Shneider ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
description	The mechanism by which FUS mutations cause familial ALS remains unclear. Here, the authors use mouse transgenic models to show that a toxic gain-of-function underlies motor neuron degeneration, and that the toxicity of mutant FUS does not depend on a loss or excess of FUS activity.
format	article
author	Aarti Sharma Alexander K. Lyashchenko Lei Lu Sara Ebrahimi Nasrabady Margot Elmaleh Monica Mendelsohn Adriana Nemes Juan Carlos Tapia George Z. Mentis Neil A. Shneider
author_facet	Aarti Sharma Alexander K. Lyashchenko Lei Lu Sara Ebrahimi Nasrabady Margot Elmaleh Monica Mendelsohn Adriana Nemes Juan Carlos Tapia George Z. Mentis Neil A. Shneider
author_sort	Aarti Sharma
title	ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
title_short	ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
title_full	ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
title_fullStr	ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
title_full_unstemmed	ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
title_sort	als-associated mutant fus induces selective motor neuron degeneration through toxic gain of function
publisher	Nature Portfolio
publishDate	2016
url	https://doaj.org/article/320664c72a834d799df983f0633c7d56
work_keys_str_mv	AT aartisharma alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT alexanderklyashchenko alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT leilu alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT saraebrahiminasrabady alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT margotelmaleh alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT monicamendelsohn alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT adriananemes alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT juancarlostapia alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT georgezmentis alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction AT neilashneider alsassociatedmutantfusinducesselectivemotorneurondegenerationthroughtoxicgainoffunction
_version_	1718383148040978432

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

Ejemplares similares