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A KO mouse model for the lncRNA Lhx1os produces motor neuron alterations and locomotor impairment

Here, we describe a conserved motor neuron-specific long non-coding RNA, Lhx1os, whose knockout in mice produces motor impairment and postnatal reduction of mature motor neurons (MNs). The ER stress-response pathway result specifically altered with the downregulation of factors involved in the unfol...

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Autores principales: Pellegrini, Flaminia, Padovano, Vittorio, Biscarini, Silvia, Santini, Tiziana, Setti, Adriano, Galfrè, Silvia Giulia, Silenzi, Valentina, Vitiello, Erika, Mariani, Davide, Nicoletti, Carmine, Torromino, Giulia, De Leonibus, Elvira, Martone, Julie, Bozzoni, Irene
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9840152/
https://www.ncbi.nlm.nih.gov/pubmed/36647387
http://dx.doi.org/10.1016/j.isci.2022.105891
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author Pellegrini, Flaminia
Padovano, Vittorio
Biscarini, Silvia
Santini, Tiziana
Setti, Adriano
Galfrè, Silvia Giulia
Silenzi, Valentina
Vitiello, Erika
Mariani, Davide
Nicoletti, Carmine
Torromino, Giulia
De Leonibus, Elvira
Martone, Julie
Bozzoni, Irene
author_facet Pellegrini, Flaminia
Padovano, Vittorio
Biscarini, Silvia
Santini, Tiziana
Setti, Adriano
Galfrè, Silvia Giulia
Silenzi, Valentina
Vitiello, Erika
Mariani, Davide
Nicoletti, Carmine
Torromino, Giulia
De Leonibus, Elvira
Martone, Julie
Bozzoni, Irene
author_sort Pellegrini, Flaminia
collection PubMed
description Here, we describe a conserved motor neuron-specific long non-coding RNA, Lhx1os, whose knockout in mice produces motor impairment and postnatal reduction of mature motor neurons (MNs). The ER stress-response pathway result specifically altered with the downregulation of factors involved in the unfolded protein response (UPR). Lhx1os was found to bind the ER-associated PDIA3 disulfide isomerase and to affect the expression of the same set of genes controlled by this protein, indicating that the two factors act in conjunction to modulate the UPR. Altogether, the observed phenotype and function of Lhx1os indicate its important role in the control of MN homeostasis and function.
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spelling pubmed-98401522023-01-15 A KO mouse model for the lncRNA Lhx1os produces motor neuron alterations and locomotor impairment Pellegrini, Flaminia Padovano, Vittorio Biscarini, Silvia Santini, Tiziana Setti, Adriano Galfrè, Silvia Giulia Silenzi, Valentina Vitiello, Erika Mariani, Davide Nicoletti, Carmine Torromino, Giulia De Leonibus, Elvira Martone, Julie Bozzoni, Irene iScience Article Here, we describe a conserved motor neuron-specific long non-coding RNA, Lhx1os, whose knockout in mice produces motor impairment and postnatal reduction of mature motor neurons (MNs). The ER stress-response pathway result specifically altered with the downregulation of factors involved in the unfolded protein response (UPR). Lhx1os was found to bind the ER-associated PDIA3 disulfide isomerase and to affect the expression of the same set of genes controlled by this protein, indicating that the two factors act in conjunction to modulate the UPR. Altogether, the observed phenotype and function of Lhx1os indicate its important role in the control of MN homeostasis and function. Elsevier 2022-12-28 /pmc/articles/PMC9840152/ /pubmed/36647387 http://dx.doi.org/10.1016/j.isci.2022.105891 Text en © 2023 The Authors. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Article
Pellegrini, Flaminia
Padovano, Vittorio
Biscarini, Silvia
Santini, Tiziana
Setti, Adriano
Galfrè, Silvia Giulia
Silenzi, Valentina
Vitiello, Erika
Mariani, Davide
Nicoletti, Carmine
Torromino, Giulia
De Leonibus, Elvira
Martone, Julie
Bozzoni, Irene
A KO mouse model for the lncRNA Lhx1os produces motor neuron alterations and locomotor impairment
title A KO mouse model for the lncRNA Lhx1os produces motor neuron alterations and locomotor impairment
title_full A KO mouse model for the lncRNA Lhx1os produces motor neuron alterations and locomotor impairment
title_fullStr A KO mouse model for the lncRNA Lhx1os produces motor neuron alterations and locomotor impairment
title_full_unstemmed A KO mouse model for the lncRNA Lhx1os produces motor neuron alterations and locomotor impairment
title_short A KO mouse model for the lncRNA Lhx1os produces motor neuron alterations and locomotor impairment
title_sort ko mouse model for the lncrna lhx1os produces motor neuron alterations and locomotor impairment
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9840152/
https://www.ncbi.nlm.nih.gov/pubmed/36647387
http://dx.doi.org/10.1016/j.isci.2022.105891
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