ParkinsonFranceV1, Hal, Curation, bibRecord, 000784

Parkin deficiency delays motor decline and disease manifestation in a mouse model of synucleinopathy.

Identifieur interne : 000784 ( Hal/Curation ); précédent : 000783; suivant : 000785

Parkin deficiency delays motor decline and disease manifestation in a mouse model of synucleinopathy.

Auteurs : Margot Fournier ; Jérémie Vitte [France] ; Jérôme Garrigue ; Dominique Langui ; Jean-Philippe Dullin ; Françoise Saurini [France] ; Naïma Hanoun [France] ; Fernando Perez-Diaz ; Fabien Cornilleau ; Chantal Joubert [France] ; Héctor Ardila-Osorio ; Sabine Traver ; René Duchateau ; Cécile Goujet-Zalc ; Katerina Paleologou ; Hilal A. Lashuel ; Christian Haass ; Charles Duyckaerts ; Charles Cohen-Salmon ; Philipp J. Kahle ; Michel Hamon [France] ; Alexis Brice [France] ; Olga Corti [France]

Source :

PLoS ONE [ 1932-6203 ] ; 2009.

RBID : Hal:hal-00541956

Abstract

In synucleinopathies, including Parkinson's disease, partially ubiquitylated alpha-synuclein species phosphorylated on serine 129 (P(S129)-alpha-synuclein) accumulate abnormally. Parkin, an ubiquitin-protein ligase that is dysfunctional in autosomal recessive parkinsonism, protects against alpha-synuclein-mediated toxicity in various models.We analyzed the effects of Parkin deficiency in a mouse model of synucleinopathy to explore the possibility that Parkin and alpha-synuclein act in the same biochemical pathway. Whether or not Parkin was present, these mice developed an age-dependent neurodegenerative disorder preceded by a progressive decline in performance in tasks predictive of sensorimotor dysfunction. The symptoms were accompanied by the deposition of P(S129)-alpha-synuclein but not P(S87)-alpha-synuclein in neuronal cell bodies and neuritic processes throughout the brainstem and the spinal cord; activation of caspase 9 was observed in 5% of the P(S129)-alpha-synuclein-positive neurons. As in Lewy bodies, ubiquitin-immunoreactivity, albeit less abundant, was invariably co-localized with P(S129)-alpha-synuclein. During late disease stages, the disease-specific neuropathological features revealed by ubiquitin- and P(S129)-alpha-synuclein-specific antibodies were similar in mice with or without Parkin. However, the proportion of P(S129)-alpha-synuclein-immunoreactive neuronal cell bodies and neurites co-stained for ubiquitin was lower in the absence than in the presence of Parkin, suggesting less advanced synucleinopathy. Moreover, sensorimotor impairment and manifestation of the neurodegenerative phenotype due to overproduction of human alpha-synuclein were significantly delayed in Parkin-deficient mice.These findings raise the possibility that effective compensatory mechanisms modulate the phenotypic expression of disease in parkin-related parkinsonism.

Url:

https://hal.archives-ouvertes.fr/hal-00541956

DOI: 10.1371/journal.pone.0006629

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<author><name sortKey="Corti, Olga" sort="Corti, Olga" uniqKey="Corti O" first="Olga" last="Corti">Olga Corti</name>
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<idno type="DOI">10.1371/journal.pone.0006629</idno>
<series><title level="j">PLoS ONE</title>
<idno type="ISSN">1932-6203</idno>
<imprint><date type="datePub">2009</date>
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<front><div type="abstract" xml:lang="en">In synucleinopathies, including Parkinson's disease, partially ubiquitylated alpha-synuclein species phosphorylated on serine 129 (P(S129)-alpha-synuclein) accumulate abnormally. Parkin, an ubiquitin-protein ligase that is dysfunctional in autosomal recessive parkinsonism, protects against alpha-synuclein-mediated toxicity in various models.We analyzed the effects of Parkin deficiency in a mouse model of synucleinopathy to explore the possibility that Parkin and alpha-synuclein act in the same biochemical pathway. Whether or not Parkin was present, these mice developed an age-dependent neurodegenerative disorder preceded by a progressive decline in performance in tasks predictive of sensorimotor dysfunction. The symptoms were accompanied by the deposition of P(S129)-alpha-synuclein but not P(S87)-alpha-synuclein in neuronal cell bodies and neuritic processes throughout the brainstem and the spinal cord; activation of caspase 9 was observed in 5% of the P(S129)-alpha-synuclein-positive neurons. As in Lewy bodies, ubiquitin-immunoreactivity, albeit less abundant, was invariably co-localized with P(S129)-alpha-synuclein. During late disease stages, the disease-specific neuropathological features revealed by ubiquitin- and P(S129)-alpha-synuclein-specific antibodies were similar in mice with or without Parkin. However, the proportion of P(S129)-alpha-synuclein-immunoreactive neuronal cell bodies and neurites co-stained for ubiquitin was lower in the absence than in the presence of Parkin, suggesting less advanced synucleinopathy. Moreover, sensorimotor impairment and manifestation of the neurodegenerative phenotype due to overproduction of human alpha-synuclein were significantly delayed in Parkin-deficient mice.These findings raise the possibility that effective compensatory mechanisms modulate the phenotypic expression of disease in parkin-related parkinsonism.</div>
</front>
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            <author role="aut">              <persName>                <forename type="first">Margot</forename>
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            <author role="aut">              <persName>                <forename type="first">Jérémie</forename>
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            <editor role="depositor">              <persName>                <forename>Fernando</forename>
                <surname>Pérez-Diaz</surname>
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              <email type="md5">5313d31a7b19ed243cdb15d9eff51a26</email>
              <email type="domain">upmc.fr</email>
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              <date type="whenProduced">2009</date>
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            <idno type="halRefHtml">PLoS ONE, Public Library of Science, 2009, 4 (8), pp.e6629. <10.1371/journal.pone.0006629></idno>
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            <idno type="stamp" n="CNRS">CNRS - Centre national de la recherche scientifique</idno>
            <idno type="stamp" n="UNIV-EVRY">Université d'Evry-Val d'Essonne</idno>
            <idno type="stamp" n="UNIV-TLSE3">Université Paul Sabatier - Toulouse III</idno>
            <idno type="stamp" n="CDF">Collège de France</idno>
            <idno type="stamp" n="UNIV-PARIS7">Université Denis Diderot - Paris VII</idno>
            <idno type="stamp" n="UPMC">Université Pierre et Marie Curie</idno>
            <idno type="stamp" n="ICM" p="UPMC">Institut du Cerveau et de la Moëlle Epinière</idno>
            <idno type="stamp" n="PSL">Paris Sciences et Lettres (PSL) Research University</idno>
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          <sourceDesc>            <biblStruct>              <analytic>                <title xml:lang="en">Parkin deficiency delays motor decline and disease manifestation in a mouse model of synucleinopathy.</title>
                <author role="aut">                  <persName>                    <forename type="first">Margot</forename>
                    <surname>Fournier</surname>
                  </persName>
                  <idno type="halauthorid">553268</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Jérémie</forename>
                    <surname>Vitte</surname>
                  </persName>
                  <idno type="halauthorid">401198</idno>
                  <affiliation ref="#struct-2666"></affiliation>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Jérôme</forename>
                    <surname>Garrigue</surname>
                  </persName>
                  <idno type="halauthorid">553269</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Dominique</forename>
                    <surname>Langui</surname>
                  </persName>
                  <idno type="halauthorid">553270</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Jean-Philippe</forename>
                    <surname>Dullin</surname>
                  </persName>
                  <idno type="halauthorid">309910</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Françoise</forename>
                    <surname>Saurini</surname>
                  </persName>
                  <idno type="halauthorid">295570</idno>
                  <affiliation ref="#struct-24507"></affiliation>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Naïma</forename>
                    <surname>Hanoun</surname>
                  </persName>
                  <idno type="halauthorid">295569</idno>
                  <affiliation ref="#struct-27489"></affiliation>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Fernando</forename>
                    <surname>Perez-Diaz</surname>
                  </persName>
                  <idno type="halauthorid">501780</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Fabien</forename>
                    <surname>Cornilleau</surname>
                  </persName>
                  <idno type="halauthorid">553271</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Chantal</forename>
                    <surname>Joubert</surname>
                  </persName>
                  <idno type="halauthorid">223100</idno>
                  <affiliation ref="#struct-1132"></affiliation>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Héctor</forename>
                    <surname>Ardila-Osorio</surname>
                  </persName>
                  <idno type="halauthorid">553272</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Sabine</forename>
                    <surname>Traver</surname>
                  </persName>
                  <idno type="halauthorid">350356</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">René</forename>
                    <surname>Duchateau</surname>
                  </persName>
                  <idno type="halauthorid">553273</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Cécile</forename>
                    <surname>Goujet-Zalc</surname>
                  </persName>
                  <idno type="halauthorid">202985</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Katerina</forename>
                    <surname>Paleologou</surname>
                  </persName>
                  <idno type="halauthorid">553274</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Hilal A</forename>
                    <surname>Lashuel</surname>
                  </persName>
                  <idno type="halauthorid">553275</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Christian</forename>
                    <surname>Haass</surname>
                  </persName>
                  <idno type="halauthorid">553276</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Charles</forename>
                    <surname>Duyckaerts</surname>
                  </persName>
                  <idno type="halauthorid">168742</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Charles</forename>
                    <surname>Cohen-Salmon</surname>
                  </persName>
                  <idno type="halauthorid">553277</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Philipp J</forename>
                    <surname>Kahle</surname>
                  </persName>
                  <idno type="halauthorid">553278</idno>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Michel</forename>
                    <surname>Hamon</surname>
                  </persName>
                  <idno type="halauthorid">93365</idno>
                  <affiliation ref="#struct-3035"></affiliation>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Alexis</forename>
                    <surname>Brice</surname>
                  </persName>
                  <idno type="halauthorid">183596</idno>
                  <affiliation ref="#struct-81470"></affiliation>
                  <affiliation ref="#struct-129108"></affiliation>
                  <affiliation ref="#struct-16845"></affiliation>
                </author>
                <author role="aut">                  <persName>                    <forename type="first">Olga</forename>
                    <surname>Corti</surname>
                  </persName>
                  <idno type="halauthorid">200494</idno>
                  <affiliation ref="#struct-3037"></affiliation>
                </author>
              </analytic>
              <monogr>                <idno type="halJournalId" status="VALID">28700</idno>
                <idno type="issn">1932-6203</idno>
                <title level="j">PLoS ONE</title>
                <imprint>                  <publisher>Public Library of Science</publisher>
                  <biblScope unit="volume">4</biblScope>
                  <biblScope unit="issue">8</biblScope>
                  <biblScope unit="pp">e6629</biblScope>
                  <date type="datePub">2009</date>
                </imprint>
              </monogr>
              <idno type="doi">10.1371/journal.pone.0006629</idno>
              <idno type="pubmed">19680561</idno>
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          <profileDesc>            <langUsage>              <language ident="en">English</language>
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            <textClass>              <classCode scheme="halDomain" n="scco.psyc">Cognitive science/Psychology</classCode>
              <classCode scheme="halTypology" n="ART">Journal articles</classCode>
            </textClass>
            <abstract xml:lang="en">In synucleinopathies, including Parkinson's disease, partially ubiquitylated alpha-synuclein species phosphorylated on serine 129 (P(S129)-alpha-synuclein) accumulate abnormally. Parkin, an ubiquitin-protein ligase that is dysfunctional in autosomal recessive parkinsonism, protects against alpha-synuclein-mediated toxicity in various models.We analyzed the effects of Parkin deficiency in a mouse model of synucleinopathy to explore the possibility that Parkin and alpha-synuclein act in the same biochemical pathway. Whether or not Parkin was present, these mice developed an age-dependent neurodegenerative disorder preceded by a progressive decline in performance in tasks predictive of sensorimotor dysfunction. The symptoms were accompanied by the deposition of P(S129)-alpha-synuclein but not P(S87)-alpha-synuclein in neuronal cell bodies and neuritic processes throughout the brainstem and the spinal cord; activation of caspase 9 was observed in 5% of the P(S129)-alpha-synuclein-positive neurons. As in Lewy bodies, ubiquitin-immunoreactivity, albeit less abundant, was invariably co-localized with P(S129)-alpha-synuclein. During late disease stages, the disease-specific neuropathological features revealed by ubiquitin- and P(S129)-alpha-synuclein-specific antibodies were similar in mice with or without Parkin. However, the proportion of P(S129)-alpha-synuclein-immunoreactive neuronal cell bodies and neurites co-stained for ubiquitin was lower in the absence than in the presence of Parkin, suggesting less advanced synucleinopathy. Moreover, sensorimotor impairment and manifestation of the neurodegenerative phenotype due to overproduction of human alpha-synuclein were significantly delayed in Parkin-deficient mice.These findings raise the possibility that effective compensatory mechanisms modulate the phenotypic expression of disease in parkin-related parkinsonism.</abstract>
          </profileDesc>
        </hal>
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	La maladie de Parkinson en France (serveur d'exploration)
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La maladie de Parkinson en France (serveur d'exploration)

Parkin deficiency delays motor decline and disease manifestation in a mouse model of synucleinopathy.

Parkin deficiency delays motor decline and disease manifestation in a mouse model of synucleinopathy.

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