Amyotrophic lateral sclerosis-like superoxide dismutase 1 proteinopathy is associated with neuronal loss in Parkinson's disease brain.
Identifieur interne : 004843 ( Ncbi/Curation ); précédent : 004842; suivant : 004844Amyotrophic lateral sclerosis-like superoxide dismutase 1 proteinopathy is associated with neuronal loss in Parkinson's disease brain.
Auteurs : Benjamin G. Trist [Australie] ; Katherine M. Davies [Australie] ; Veronica Cottam [Australie] ; Sian Genoud [Australie] ; Richard Ortega [France] ; Stéphane Roudeau [France] ; Asuncion Carmona [France] ; Kasun De Silva [Australie] ; Valerie Wasinger [Australie] ; Simon J G. Lewis [Australie] ; Perminder Sachdev [Australie] ; Bradley Smith [Royaume-Uni] ; Claire Troakes [Royaume-Uni] ; Caroline Vance [Royaume-Uni] ; Christopher Shaw [Royaume-Uni] ; Safa Al-Sarraj [Royaume-Uni] ; Helen J. Ball [Australie] ; Glenda M. Halliday [Australie] ; Dominic J. Hare [Australie] ; Kay L. Double [Australie]Source :
- Acta neuropathologica [ 1432-0533 ] ; 2017.
Abstract
Neuronal loss in numerous neurodegenerative disorders has been linked to protein aggregation and oxidative stress. Emerging data regarding overlapping proteinopathy in traditionally distinct neurodegenerative diseases suggest that disease-modifying treatments targeting these pathological features may exhibit efficacy across multiple disorders. Here, we describe proteinopathy distinct from classic synucleinopathy, predominantly comprised of the anti-oxidant enzyme superoxide dismutase-1 (SOD1), in the Parkinson's disease brain. Significant expression of this pathology closely reflected the regional pattern of neuronal loss. The protein composition and non-amyloid macrostructure of these novel aggregates closely resembles that of neurotoxic SOD1 deposits in SOD1-associated familial amyotrophic lateral sclerosis (fALS). Consistent with the hypothesis that deposition of protein aggregates in neurodegenerative disorders reflects upstream dysfunction, we demonstrated that SOD1 in the Parkinson's disease brain exhibits evidence of misfolding and metal deficiency, similar to that seen in mutant SOD1 in fALS. Our data suggest common mechanisms of toxic SOD1 aggregation in both disorders and a potential role for SOD1 dysfunction in neuronal loss in the Parkinson's disease brain. This shared restricted proteinopathy highlights the potential translation of therapeutic approaches targeting SOD1 toxicity, already in clinical trials for ALS, into disease-modifying treatments for Parkinson's disease.
DOI: 10.1007/s00401-017-1726-6
PubMed: 28527045
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<sourceDesc><biblStruct><analytic><title xml:lang="en">Amyotrophic lateral sclerosis-like superoxide dismutase 1 proteinopathy is associated with neuronal loss in Parkinson's disease brain.</title>
<author><name sortKey="Trist, Benjamin G" sort="Trist, Benjamin G" uniqKey="Trist B" first="Benjamin G" last="Trist">Benjamin G. Trist</name>
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<author><name sortKey="Davies, Katherine M" sort="Davies, Katherine M" uniqKey="Davies K" first="Katherine M" last="Davies">Katherine M. Davies</name>
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<author><name sortKey="Cottam, Veronica" sort="Cottam, Veronica" uniqKey="Cottam V" first="Veronica" last="Cottam">Veronica Cottam</name>
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<author><name sortKey="Troakes, Claire" sort="Troakes, Claire" uniqKey="Troakes C" first="Claire" last="Troakes">Claire Troakes</name>
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<author><name sortKey="Vance, Caroline" sort="Vance, Caroline" uniqKey="Vance C" first="Caroline" last="Vance">Caroline Vance</name>
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<author><name sortKey="Shaw, Christopher" sort="Shaw, Christopher" uniqKey="Shaw C" first="Christopher" last="Shaw">Christopher Shaw</name>
<affiliation wicri:level="1"><nlm:affiliation>Maurice Wohl Clinical Neuroscience Institute, King's College London, Camberwell, London, SE5 9NU, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Maurice Wohl Clinical Neuroscience Institute, King's College London, Camberwell, London, SE5 9NU</wicri:regionArea>
<wicri:noRegion>SE5 9NU</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Al Sarraj, Safa" sort="Al Sarraj, Safa" uniqKey="Al Sarraj S" first="Safa" last="Al-Sarraj">Safa Al-Sarraj</name>
<affiliation wicri:level="1"><nlm:affiliation>Maurice Wohl Clinical Neuroscience Institute, King's College London, Camberwell, London, SE5 9NU, UK.</nlm:affiliation>
<country xml:lang="fr">Royaume-Uni</country>
<wicri:regionArea>Maurice Wohl Clinical Neuroscience Institute, King's College London, Camberwell, London, SE5 9NU</wicri:regionArea>
<wicri:noRegion>SE5 9NU</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Ball, Helen J" sort="Ball, Helen J" uniqKey="Ball H" first="Helen J" last="Ball">Helen J. Ball</name>
<affiliation wicri:level="4"><nlm:affiliation>Biostatistics and Bioinformatics Facility, Bosch Institute, The University of Sydney, Sydney, Australia.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Biostatistics and Bioinformatics Facility, Bosch Institute, The University of Sydney, Sydney</wicri:regionArea>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
<settlement type="city">Sydney</settlement>
</placeName>
<orgName type="university">Université de Sydney</orgName>
</affiliation>
</author>
<author><name sortKey="Halliday, Glenda M" sort="Halliday, Glenda M" uniqKey="Halliday G" first="Glenda M" last="Halliday">Glenda M. Halliday</name>
<affiliation wicri:level="1"><nlm:affiliation>Neuroscience Research Australia, Sydney, NSW, 2031, Australia.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Neuroscience Research Australia, Sydney, NSW, 2031</wicri:regionArea>
<wicri:noRegion>2031</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Hare, Dominic J" sort="Hare, Dominic J" uniqKey="Hare D" first="Dominic J" last="Hare">Dominic J. Hare</name>
<affiliation wicri:level="4"><nlm:affiliation>The Florey Institute of Neuroscience and Mental Health, The University of Melbourne, Parkville, VIC, 3052, Australia.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>The Florey Institute of Neuroscience and Mental Health, The University of Melbourne, Parkville, VIC, 3052</wicri:regionArea>
<orgName type="university">Université de Melbourne</orgName>
<placeName><settlement type="city">Melbourne</settlement>
<region type="état">Victoria (État)</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Double, Kay L" sort="Double, Kay L" uniqKey="Double K" first="Kay L" last="Double">Kay L. Double</name>
<affiliation wicri:level="4"><nlm:affiliation>Discipline of Biomedical Science and Brain and Mind Centre, Sydney Medical School, The University of Sydney, Sydney, NSW, 2006, Australia. kay.double@sydney.edu.au.</nlm:affiliation>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Discipline of Biomedical Science and Brain and Mind Centre, Sydney Medical School, The University of Sydney, Sydney, NSW, 2006</wicri:regionArea>
<orgName type="university">Université de Sydney</orgName>
<placeName><settlement type="city">Sydney</settlement>
<region type="état">Nouvelle-Galles du Sud</region>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">Acta neuropathologica</title>
<idno type="eISSN">1432-0533</idno>
<imprint><date when="2017" type="published">2017</date>
</imprint>
</series>
</biblStruct>
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<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Neuronal loss in numerous neurodegenerative disorders has been linked to protein aggregation and oxidative stress. Emerging data regarding overlapping proteinopathy in traditionally distinct neurodegenerative diseases suggest that disease-modifying treatments targeting these pathological features may exhibit efficacy across multiple disorders. Here, we describe proteinopathy distinct from classic synucleinopathy, predominantly comprised of the anti-oxidant enzyme superoxide dismutase-1 (SOD1), in the Parkinson's disease brain. Significant expression of this pathology closely reflected the regional pattern of neuronal loss. The protein composition and non-amyloid macrostructure of these novel aggregates closely resembles that of neurotoxic SOD1 deposits in SOD1-associated familial amyotrophic lateral sclerosis (fALS). Consistent with the hypothesis that deposition of protein aggregates in neurodegenerative disorders reflects upstream dysfunction, we demonstrated that SOD1 in the Parkinson's disease brain exhibits evidence of misfolding and metal deficiency, similar to that seen in mutant SOD1 in fALS. Our data suggest common mechanisms of toxic SOD1 aggregation in both disorders and a potential role for SOD1 dysfunction in neuronal loss in the Parkinson's disease brain. This shared restricted proteinopathy highlights the potential translation of therapeutic approaches targeting SOD1 toxicity, already in clinical trials for ALS, into disease-modifying treatments for Parkinson's disease.</div>
</front>
</TEI>
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