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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Main Authors: 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
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-10-01
Series:PLoS Genetics
Online Access:https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1002338&type=printable
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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
collection DOAJ
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.
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institution Kabale University
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publishDate 2011-10-01
publisher Public Library of Science (PLoS)
record_format Article
series PLoS Genetics
spelling doaj-art-bded789a20a14f05bd7a0ff17016ec812025-08-20T03:26:39ZengPublic Library of Science (PLoS)PLoS Genetics1553-73901553-74042011-10-01710e100233810.1371/journal.pgen.1002338Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.Peter L OliverMattéa J FinelliBenjamin EdwardsEmmanuelle BitounDarcy L ButtsEsther B E BeckerMichael T CheesemanBen DaviesKay E DaviesOxidative 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.https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1002338&type=printable
spellingShingle 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.
PLoS Genetics
title 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_short Oxr1 is essential for protection against oxidative stress-induced neurodegeneration.
title_sort oxr1 is essential for protection against oxidative stress induced neurodegeneration
url https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1002338&type=printable
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