The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis

There is ample evidence that nucleocytoplasmic-transport deficits could play an important role in the pathology of amyotrophic lateral sclerosis (ALS). However, the currently available data are often circumstantial and do not fully clarify the exact causal and temporal role of nucleocytoplasmic tran...

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Autores principales:	Joni Vanneste, Ludo Van Den Bosch
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	neurodegeneration motor neuron ALS Biology (General) QH301-705.5 Chemistry QD1-999
Acceso en línea:	https://doaj.org/article/11716a30f4114af7a5c822f1cc632ebc
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spelling	oai:doaj.org-article:11716a30f4114af7a5c822f1cc632ebc2021-11-25T17:54:08ZThe Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis10.3390/ijms2222121751422-00671661-6596https://doaj.org/article/11716a30f4114af7a5c822f1cc632ebc2021-11-01T00:00:00Zhttps://www.mdpi.com/1422-0067/22/22/12175https://doaj.org/toc/1661-6596https://doaj.org/toc/1422-0067There is ample evidence that nucleocytoplasmic-transport deficits could play an important role in the pathology of amyotrophic lateral sclerosis (ALS). However, the currently available data are often circumstantial and do not fully clarify the exact causal and temporal role of nucleocytoplasmic transport deficits in ALS patients. Gaining this knowledge will be of great significance in order to be able to target therapeutically nucleocytoplasmic transport and/or the proteins involved in this process. The availability of good model systems to study the nucleocytoplasmic transport process in detail will be especially crucial in investigating the effect of different mutations, as well as of other forms of stress. In this review, we discuss the evidence for the involvement of nucleocytoplasmic transport defects in ALS and the methods used to obtain these data. In addition, we provide an overview of the therapeutic strategies which could potentially counteract these defects.Joni VannesteLudo Van Den BoschMDPI AGarticleneurodegenerationmotor neuronALSBiology (General)QH301-705.5ChemistryQD1-999ENInternational Journal of Molecular Sciences, Vol 22, Iss 12175, p 12175 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	neurodegeneration motor neuron ALS Biology (General) QH301-705.5 Chemistry QD1-999
spellingShingle	neurodegeneration motor neuron ALS Biology (General) QH301-705.5 Chemistry QD1-999 Joni Vanneste Ludo Van Den Bosch The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis
description	There is ample evidence that nucleocytoplasmic-transport deficits could play an important role in the pathology of amyotrophic lateral sclerosis (ALS). However, the currently available data are often circumstantial and do not fully clarify the exact causal and temporal role of nucleocytoplasmic transport deficits in ALS patients. Gaining this knowledge will be of great significance in order to be able to target therapeutically nucleocytoplasmic transport and/or the proteins involved in this process. The availability of good model systems to study the nucleocytoplasmic transport process in detail will be especially crucial in investigating the effect of different mutations, as well as of other forms of stress. In this review, we discuss the evidence for the involvement of nucleocytoplasmic transport defects in ALS and the methods used to obtain these data. In addition, we provide an overview of the therapeutic strategies which could potentially counteract these defects.
format	article
author	Joni Vanneste Ludo Van Den Bosch
author_facet	Joni Vanneste Ludo Van Den Bosch
author_sort	Joni Vanneste
title	The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis
title_short	The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis
title_full	The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis
title_fullStr	The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis
title_full_unstemmed	The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis
title_sort	role of nucleocytoplasmic transport defects in amyotrophic lateral sclerosis
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/11716a30f4114af7a5c822f1cc632ebc
work_keys_str_mv	AT jonivanneste theroleofnucleocytoplasmictransportdefectsinamyotrophiclateralsclerosis AT ludovandenbosch theroleofnucleocytoplasmictransportdefectsinamyotrophiclateralsclerosis AT jonivanneste roleofnucleocytoplasmictransportdefectsinamyotrophiclateralsclerosis AT ludovandenbosch roleofnucleocytoplasmictransportdefectsinamyotrophiclateralsclerosis
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The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis

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