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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Nature Portfolio
2021
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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) |
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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 |
work_keys_str_mv |
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