Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models

The (G4C2)-RNA hexanucleotide repeat expansion upstream of the start codon of the C9orf72 gene plays a critical role in familial ALS. The authors show that Sig1R, a ligand-regulated molecular chaperone, counteracts the aberrant nucleocytoplasmic distribution of Ran caused by the (G4C2)-RNA repeats.

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Autores principales:	Pin-Tse Lee, Jean-Charles Liévens, Shao-Ming Wang, Jian-Ying Chuang, Bilal Khalil, Hsiang-en Wu, Wen-Chang Chang, Tangui Maurice, Tsung-Ping Su
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2020
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/3e69e0a8edd7453b8b1babe7a2048d98
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spelling	oai:doaj.org-article:3e69e0a8edd7453b8b1babe7a2048d982021-12-02T16:49:44ZSigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models10.1038/s41467-020-19396-32041-1723https://doaj.org/article/3e69e0a8edd7453b8b1babe7a2048d982020-11-01T00:00:00Zhttps://doi.org/10.1038/s41467-020-19396-3https://doaj.org/toc/2041-1723The (G4C2)-RNA hexanucleotide repeat expansion upstream of the start codon of the C9orf72 gene plays a critical role in familial ALS. The authors show that Sig1R, a ligand-regulated molecular chaperone, counteracts the aberrant nucleocytoplasmic distribution of Ran caused by the (G4C2)-RNA repeats.Pin-Tse LeeJean-Charles LiévensShao-Ming WangJian-Ying ChuangBilal KhalilHsiang-en WuWen-Chang ChangTangui MauriceTsung-Ping SuNature PortfolioarticleScienceQENNature Communications, Vol 11, Iss 1, Pp 1-14 (2020)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Pin-Tse Lee Jean-Charles Liévens Shao-Ming Wang Jian-Ying Chuang Bilal Khalil Hsiang-en Wu Wen-Chang Chang Tangui Maurice Tsung-Ping Su Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models
description	The (G4C2)-RNA hexanucleotide repeat expansion upstream of the start codon of the C9orf72 gene plays a critical role in familial ALS. The authors show that Sig1R, a ligand-regulated molecular chaperone, counteracts the aberrant nucleocytoplasmic distribution of Ran caused by the (G4C2)-RNA repeats.
format	article
author	Pin-Tse Lee Jean-Charles Liévens Shao-Ming Wang Jian-Ying Chuang Bilal Khalil Hsiang-en Wu Wen-Chang Chang Tangui Maurice Tsung-Ping Su
author_facet	Pin-Tse Lee Jean-Charles Liévens Shao-Ming Wang Jian-Ying Chuang Bilal Khalil Hsiang-en Wu Wen-Chang Chang Tangui Maurice Tsung-Ping Su
author_sort	Pin-Tse Lee
title	Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models
title_short	Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models
title_full	Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models
title_fullStr	Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models
title_full_unstemmed	Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models
title_sort	sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and drosophila als/ftd models
publisher	Nature Portfolio
publishDate	2020
url	https://doaj.org/article/3e69e0a8edd7453b8b1babe7a2048d98
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Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models

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