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
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Lenguaje:EN
Publicado: Nature Portfolio 2021
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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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