SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS

Copper-zinc superoxide dismutase (SOD1) is a detoxifying enzyme localized in the cytosol, nucleus, peroxisomes, and mitochondria. The discovery that mutations in SOD1 gene cause a subset of familial amyotrophic lateral sclerosis (FALS) has attracted great attention, and studies to date have been mai...

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Main Authors: Pamela Milani, Stella Gagliardi, Emanuela Cova, Cristina Cereda
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Neurology Research International
Online Access:http://dx.doi.org/10.1155/2011/458427
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author Pamela Milani
Stella Gagliardi
Emanuela Cova
Cristina Cereda
author_facet Pamela Milani
Stella Gagliardi
Emanuela Cova
Cristina Cereda
author_sort Pamela Milani
collection DOAJ
description Copper-zinc superoxide dismutase (SOD1) is a detoxifying enzyme localized in the cytosol, nucleus, peroxisomes, and mitochondria. The discovery that mutations in SOD1 gene cause a subset of familial amyotrophic lateral sclerosis (FALS) has attracted great attention, and studies to date have been mainly focused on discovering mutations in the coding region and investigation at protein level. Considering that changes in SOD1 mRNA levels have been associated with sporadic ALS (SALS), a molecular understanding of the processes involved in the regulation of SOD1 gene expression could not only unravel novel regulatory pathways that may govern cellular phenotypes and changes in diseases but also might reveal therapeutic targets and treatments. This review seeks to provide an overview of SOD1 gene structure and of the processes through which SOD1 transcription is controlled. Furthermore, we emphasize the importance to focus future researches on investigating posttranscriptional mechanisms and their relevance to ALS.
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institution Kabale University
issn 2090-1852
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spelling doaj-art-1d59c54809964a149b7005f02378a2102025-02-03T05:50:26ZengWileyNeurology Research International2090-18522090-18602011-01-01201110.1155/2011/458427458427SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALSPamela Milani0Stella Gagliardi1Emanuela Cova2Cristina Cereda3Laboratory of Experimental Neurobiology, IRCCS, National Neurological Institute “C. Mondino,” Via Mondino 2, 27100 Pavia, ItalyLaboratory of Experimental Neurobiology, IRCCS, National Neurological Institute “C. Mondino,” Via Mondino 2, 27100 Pavia, ItalyLaboratory of Experimental Neurobiology, IRCCS, National Neurological Institute “C. Mondino,” Via Mondino 2, 27100 Pavia, ItalyLaboratory of Experimental Neurobiology, IRCCS, National Neurological Institute “C. Mondino,” Via Mondino 2, 27100 Pavia, ItalyCopper-zinc superoxide dismutase (SOD1) is a detoxifying enzyme localized in the cytosol, nucleus, peroxisomes, and mitochondria. The discovery that mutations in SOD1 gene cause a subset of familial amyotrophic lateral sclerosis (FALS) has attracted great attention, and studies to date have been mainly focused on discovering mutations in the coding region and investigation at protein level. Considering that changes in SOD1 mRNA levels have been associated with sporadic ALS (SALS), a molecular understanding of the processes involved in the regulation of SOD1 gene expression could not only unravel novel regulatory pathways that may govern cellular phenotypes and changes in diseases but also might reveal therapeutic targets and treatments. This review seeks to provide an overview of SOD1 gene structure and of the processes through which SOD1 transcription is controlled. Furthermore, we emphasize the importance to focus future researches on investigating posttranscriptional mechanisms and their relevance to ALS.http://dx.doi.org/10.1155/2011/458427
spellingShingle Pamela Milani
Stella Gagliardi
Emanuela Cova
Cristina Cereda
SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS
Neurology Research International
title SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS
title_full SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS
title_fullStr SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS
title_full_unstemmed SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS
title_short SOD1 Transcriptional and Posttranscriptional Regulation and Its Potential Implications in ALS
title_sort sod1 transcriptional and posttranscriptional regulation and its potential implications in als
url http://dx.doi.org/10.1155/2011/458427
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