Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins

Here, the authors show in human iPSC-derived motor neurons from ALS patients and a TDP-43 mouse model that axonal TDP-43 forms G3BP1 positive RNP condensates, which sequester mRNA of nuclear encoded mitochondrial proteins and decrease local protein synthesis in motor neuron axons and neuromuscular j...

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Autores principales:	Topaz Altman, Ariel Ionescu, Amjad Ibraheem, Dominik Priesmann, Tal Gradus-Pery, Luba Farberov, Gayster Alexandra, Natalia Shelestovich, Ruxandra Dafinca, Noam Shomron, Florence Rage, Kevin Talbot, Michael E. Ward, Amir Dori, Marcus Krüger, Eran Perlson
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/9c6dfe446ae343e18acd042aa8d017a6
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spelling	oai:doaj.org-article:9c6dfe446ae343e18acd042aa8d017a62021-11-28T12:32:57ZAxonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins10.1038/s41467-021-27221-82041-1723https://doaj.org/article/9c6dfe446ae343e18acd042aa8d017a62021-11-01T00:00:00Zhttps://doi.org/10.1038/s41467-021-27221-8https://doaj.org/toc/2041-1723Here, the authors show in human iPSC-derived motor neurons from ALS patients and a TDP-43 mouse model that axonal TDP-43 forms G3BP1 positive RNP condensates, which sequester mRNA of nuclear encoded mitochondrial proteins and decrease local protein synthesis in motor neuron axons and neuromuscular junctions.Topaz AltmanAriel IonescuAmjad IbraheemDominik PriesmannTal Gradus-PeryLuba FarberovGayster AlexandraNatalia ShelestovichRuxandra DafincaNoam ShomronFlorence RageKevin TalbotMichael E. WardAmir DoriMarcus KrügerEran PerlsonNature PortfolioarticleScienceQENNature Communications, Vol 12, Iss 1, Pp 1-17 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Topaz Altman Ariel Ionescu Amjad Ibraheem Dominik Priesmann Tal Gradus-Pery Luba Farberov Gayster Alexandra Natalia Shelestovich Ruxandra Dafinca Noam Shomron Florence Rage Kevin Talbot Michael E. Ward Amir Dori Marcus Krüger Eran Perlson Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
description	Here, the authors show in human iPSC-derived motor neurons from ALS patients and a TDP-43 mouse model that axonal TDP-43 forms G3BP1 positive RNP condensates, which sequester mRNA of nuclear encoded mitochondrial proteins and decrease local protein synthesis in motor neuron axons and neuromuscular junctions.
format	article
author	Topaz Altman Ariel Ionescu Amjad Ibraheem Dominik Priesmann Tal Gradus-Pery Luba Farberov Gayster Alexandra Natalia Shelestovich Ruxandra Dafinca Noam Shomron Florence Rage Kevin Talbot Michael E. Ward Amir Dori Marcus Krüger Eran Perlson
author_facet	Topaz Altman Ariel Ionescu Amjad Ibraheem Dominik Priesmann Tal Gradus-Pery Luba Farberov Gayster Alexandra Natalia Shelestovich Ruxandra Dafinca Noam Shomron Florence Rage Kevin Talbot Michael E. Ward Amir Dori Marcus Krüger Eran Perlson
author_sort	Topaz Altman
title	Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
title_short	Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
title_full	Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
title_fullStr	Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
title_full_unstemmed	Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
title_sort	axonal tdp-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/9c6dfe446ae343e18acd042aa8d017a6
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Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins

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