Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.

Oxidative stress is a common etiological feature of neurological disorders, although the pathways that govern defence against reactive oxygen species (ROS) in neurodegeneration remain unclear. We have identified the role of oxidation resistance 1 (Oxr1) as a vital protein that controls the sensitivi...

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Autores principales: Peter L Oliver, Mattéa J Finelli, Benjamin Edwards, Emmanuelle Bitoun, Darcy L Butts, Esther B E Becker, Michael T Cheeseman, Ben Davies, Kay E Davies
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Publicado: Public Library of Science (PLoS) 2011
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Acceso en línea:https://doaj.org/article/bded789a20a14f05bd7a0ff17016ec81
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spelling oai:doaj.org-article:bded789a20a14f05bd7a0ff17016ec812021-11-18T06:19:16ZOxr1 is essential for protection against oxidative stress-induced neurodegeneration.1553-73901553-740410.1371/journal.pgen.1002338https://doaj.org/article/bded789a20a14f05bd7a0ff17016ec812011-10-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/22028674/pdf/?tool=EBIhttps://doaj.org/toc/1553-7390https://doaj.org/toc/1553-7404Oxidative stress is a common etiological feature of neurological disorders, although the pathways that govern defence against reactive oxygen species (ROS) in neurodegeneration remain unclear. We have identified the role of oxidation resistance 1 (Oxr1) as a vital protein that controls the sensitivity of neuronal cells to oxidative stress; mice lacking Oxr1 display cerebellar neurodegeneration, and neurons are less susceptible to exogenous stress when the gene is over-expressed. A conserved short isoform of Oxr1 is also sufficient to confer this neuroprotective property both in vitro and in vivo. In addition, biochemical assays indicate that Oxr1 itself is susceptible to cysteine-mediated oxidation. Finally we show up-regulation of Oxr1 in both human and pre-symptomatic mouse models of amyotrophic lateral sclerosis, indicating that Oxr1 is potentially a novel neuroprotective factor in neurodegenerative disease.Peter L OliverMattéa J FinelliBenjamin EdwardsEmmanuelle BitounDarcy L ButtsEsther B E BeckerMichael T CheesemanBen DaviesKay E DaviesPublic Library of Science (PLoS)articleGeneticsQH426-470ENPLoS Genetics, Vol 7, Iss 10, p e1002338 (2011)
institution DOAJ
collection DOAJ
language EN
topic Genetics
QH426-470
spellingShingle Genetics
QH426-470
Peter L Oliver
Mattéa J Finelli
Benjamin Edwards
Emmanuelle Bitoun
Darcy L Butts
Esther B E Becker
Michael T Cheeseman
Ben Davies
Kay E Davies
Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.
description Oxidative stress is a common etiological feature of neurological disorders, although the pathways that govern defence against reactive oxygen species (ROS) in neurodegeneration remain unclear. We have identified the role of oxidation resistance 1 (Oxr1) as a vital protein that controls the sensitivity of neuronal cells to oxidative stress; mice lacking Oxr1 display cerebellar neurodegeneration, and neurons are less susceptible to exogenous stress when the gene is over-expressed. A conserved short isoform of Oxr1 is also sufficient to confer this neuroprotective property both in vitro and in vivo. In addition, biochemical assays indicate that Oxr1 itself is susceptible to cysteine-mediated oxidation. Finally we show up-regulation of Oxr1 in both human and pre-symptomatic mouse models of amyotrophic lateral sclerosis, indicating that Oxr1 is potentially a novel neuroprotective factor in neurodegenerative disease.
format article
author Peter L Oliver
Mattéa J Finelli
Benjamin Edwards
Emmanuelle Bitoun
Darcy L Butts
Esther B E Becker
Michael T Cheeseman
Ben Davies
Kay E Davies
author_facet Peter L Oliver
Mattéa J Finelli
Benjamin Edwards
Emmanuelle Bitoun
Darcy L Butts
Esther B E Becker
Michael T Cheeseman
Ben Davies
Kay E Davies
author_sort Peter L Oliver
title Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.
title_short Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.
title_full Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.
title_fullStr Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.
title_full_unstemmed Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.
title_sort oxr1 is essential for protection against oxidative stress-induced neurodegeneration.
publisher Public Library of Science (PLoS)
publishDate 2011
url https://doaj.org/article/bded789a20a14f05bd7a0ff17016ec81
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