La maladie de Parkinson en France (serveur d'exploration)

Attention, ce site est en cours de développement !
Attention, site généré par des moyens informatiques à partir de corpus bruts.
Les informations ne sont donc pas validées.

Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.

Identifieur interne : 000070 ( Hal/Corpus ); précédent : 000069; suivant : 000071

Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.

Auteurs : Anna Migdalska-Richards ; Liam Daly ; Erwan Bezard ; Anthony H V. Schapira

Source :

RBID : Hal:hal-01456949

Abstract

Gaucher disease is caused by mutations in the glucocerebrosidase 1 gene that result in deficiency of the lysosomal enzyme glucocerebrosidase. Both homozygous and heterozygous glucocerebrosidase 1 mutations confer an increased risk for developing Parkinson disease. Current estimates indicate that 10 to 25% of Parkinson patients carry glucocerebrosidase 1 mutations. Ambroxol is a small molecule chaperone that has been shown to increase glucocerebrosidase activity in vitro. This study investigated the effect of ambroxol treatment on glucocerebrosidase activity and on α-synuclein and phosphorylated α-synuclein protein levels in mice.

Url:
DOI: 10.1002/ana.24790.

Links to Exploration step

Hal:hal-01456949

Le document en format XML

<record>
<TEI>
<teiHeader>
<fileDesc>
<titleStmt>
<title xml:lang="en">Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.</title>
<author>
<name sortKey="Migdalska Richards, Anna" sort="Migdalska Richards, Anna" uniqKey="Migdalska Richards A" first="Anna" last="Migdalska-Richards">Anna Migdalska-Richards</name>
<affiliation>
<hal:affiliation type="laboratory" xml:id="struct-95915" status="INCOMING">
<orgName>Department of Clinical Neurosciences</orgName>
<desc>
<address>
<country key="GB"></country>
</address>
</desc>
<listRelation>
<relation active="#struct-300875" type="direct"></relation>
<relation active="#struct-310129" type="direct"></relation>
</listRelation>
<tutelles>
<tutelle active="#struct-300875" type="direct">
<org type="institution" xml:id="struct-300875" status="VALID">
<orgName>University College of London [London]</orgName>
<desc>
<address>
<addrLine>Gower Street, London WC1E 6BT </addrLine>
<country key="GB"></country>
</address>
<ref type="url">http://www.ucl.ac.uk/</ref>
</desc>
</org>
</tutelle>
<tutelle active="#struct-310129" type="direct">
<org type="institution" xml:id="struct-310129" status="INCOMING">
<orgName>Institute of Neurology</orgName>
<desc>
<address>
<country key="FR"></country>
</address>
</desc>
</org>
</tutelle>
</tutelles>
</hal:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Daly, Liam" sort="Daly, Liam" uniqKey="Daly L" first="Liam" last="Daly">Liam Daly</name>
<affiliation>
<hal:affiliation type="laboratory" xml:id="struct-95915" status="INCOMING">
<orgName>Department of Clinical Neurosciences</orgName>
<desc>
<address>
<country key="GB"></country>
</address>
</desc>
<listRelation>
<relation active="#struct-300875" type="direct"></relation>
<relation active="#struct-310129" type="direct"></relation>
</listRelation>
<tutelles>
<tutelle active="#struct-300875" type="direct">
<org type="institution" xml:id="struct-300875" status="VALID">
<orgName>University College of London [London]</orgName>
<desc>
<address>
<addrLine>Gower Street, London WC1E 6BT </addrLine>
<country key="GB"></country>
</address>
<ref type="url">http://www.ucl.ac.uk/</ref>
</desc>
</org>
</tutelle>
<tutelle active="#struct-310129" type="direct">
<org type="institution" xml:id="struct-310129" status="INCOMING">
<orgName>Institute of Neurology</orgName>
<desc>
<address>
<country key="FR"></country>
</address>
</desc>
</org>
</tutelle>
</tutelles>
</hal:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Bezard, Erwan" sort="Bezard, Erwan" uniqKey="Bezard E" first="Erwan" last="Bezard">Erwan Bezard</name>
<affiliation>
<hal:affiliation type="institution" xml:id="struct-467086" status="INCOMING">
<orgName>Univ de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, F-33000 Bordeaux, France.</orgName>
<desc>
<address>
<addrLine>146 rue Léo Saignat - CS 6129233076 Bordeaux cedex</addrLine>
<country key="FR"></country>
</address>
</desc>
</hal:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Schapira, Anthony H V" sort="Schapira, Anthony H V" uniqKey="Schapira A" first="Anthony H V" last="Schapira">Anthony H V. Schapira</name>
<affiliation>
<hal:affiliation type="laboratory" xml:id="struct-95915" status="INCOMING">
<orgName>Department of Clinical Neurosciences</orgName>
<desc>
<address>
<country key="GB"></country>
</address>
</desc>
<listRelation>
<relation active="#struct-300875" type="direct"></relation>
<relation active="#struct-310129" type="direct"></relation>
</listRelation>
<tutelles>
<tutelle active="#struct-300875" type="direct">
<org type="institution" xml:id="struct-300875" status="VALID">
<orgName>University College of London [London]</orgName>
<desc>
<address>
<addrLine>Gower Street, London WC1E 6BT </addrLine>
<country key="GB"></country>
</address>
<ref type="url">http://www.ucl.ac.uk/</ref>
</desc>
</org>
</tutelle>
<tutelle active="#struct-310129" type="direct">
<org type="institution" xml:id="struct-310129" status="INCOMING">
<orgName>Institute of Neurology</orgName>
<desc>
<address>
<country key="FR"></country>
</address>
</desc>
</org>
</tutelle>
</tutelles>
</hal:affiliation>
</affiliation>
</author>
</titleStmt>
<publicationStmt>
<idno type="wicri:source">HAL</idno>
<idno type="RBID">Hal:hal-01456949</idno>
<idno type="halId">hal-01456949</idno>
<idno type="halUri">https://hal.archives-ouvertes.fr/hal-01456949</idno>
<idno type="url">https://hal.archives-ouvertes.fr/hal-01456949</idno>
<idno type="doi">10.1002/ana.24790.</idno>
<date when="2016-11">2016-11</date>
<idno type="wicri:Area/Hal/Corpus">000070</idno>
</publicationStmt>
<sourceDesc>
<biblStruct>
<analytic>
<title xml:lang="en">Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.</title>
<author>
<name sortKey="Migdalska Richards, Anna" sort="Migdalska Richards, Anna" uniqKey="Migdalska Richards A" first="Anna" last="Migdalska-Richards">Anna Migdalska-Richards</name>
<affiliation>
<hal:affiliation type="laboratory" xml:id="struct-95915" status="INCOMING">
<orgName>Department of Clinical Neurosciences</orgName>
<desc>
<address>
<country key="GB"></country>
</address>
</desc>
<listRelation>
<relation active="#struct-300875" type="direct"></relation>
<relation active="#struct-310129" type="direct"></relation>
</listRelation>
<tutelles>
<tutelle active="#struct-300875" type="direct">
<org type="institution" xml:id="struct-300875" status="VALID">
<orgName>University College of London [London]</orgName>
<desc>
<address>
<addrLine>Gower Street, London WC1E 6BT </addrLine>
<country key="GB"></country>
</address>
<ref type="url">http://www.ucl.ac.uk/</ref>
</desc>
</org>
</tutelle>
<tutelle active="#struct-310129" type="direct">
<org type="institution" xml:id="struct-310129" status="INCOMING">
<orgName>Institute of Neurology</orgName>
<desc>
<address>
<country key="FR"></country>
</address>
</desc>
</org>
</tutelle>
</tutelles>
</hal:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Daly, Liam" sort="Daly, Liam" uniqKey="Daly L" first="Liam" last="Daly">Liam Daly</name>
<affiliation>
<hal:affiliation type="laboratory" xml:id="struct-95915" status="INCOMING">
<orgName>Department of Clinical Neurosciences</orgName>
<desc>
<address>
<country key="GB"></country>
</address>
</desc>
<listRelation>
<relation active="#struct-300875" type="direct"></relation>
<relation active="#struct-310129" type="direct"></relation>
</listRelation>
<tutelles>
<tutelle active="#struct-300875" type="direct">
<org type="institution" xml:id="struct-300875" status="VALID">
<orgName>University College of London [London]</orgName>
<desc>
<address>
<addrLine>Gower Street, London WC1E 6BT </addrLine>
<country key="GB"></country>
</address>
<ref type="url">http://www.ucl.ac.uk/</ref>
</desc>
</org>
</tutelle>
<tutelle active="#struct-310129" type="direct">
<org type="institution" xml:id="struct-310129" status="INCOMING">
<orgName>Institute of Neurology</orgName>
<desc>
<address>
<country key="FR"></country>
</address>
</desc>
</org>
</tutelle>
</tutelles>
</hal:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Bezard, Erwan" sort="Bezard, Erwan" uniqKey="Bezard E" first="Erwan" last="Bezard">Erwan Bezard</name>
<affiliation>
<hal:affiliation type="institution" xml:id="struct-467086" status="INCOMING">
<orgName>Univ de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, F-33000 Bordeaux, France.</orgName>
<desc>
<address>
<addrLine>146 rue Léo Saignat - CS 6129233076 Bordeaux cedex</addrLine>
<country key="FR"></country>
</address>
</desc>
</hal:affiliation>
</affiliation>
</author>
<author>
<name sortKey="Schapira, Anthony H V" sort="Schapira, Anthony H V" uniqKey="Schapira A" first="Anthony H V" last="Schapira">Anthony H V. Schapira</name>
<affiliation>
<hal:affiliation type="laboratory" xml:id="struct-95915" status="INCOMING">
<orgName>Department of Clinical Neurosciences</orgName>
<desc>
<address>
<country key="GB"></country>
</address>
</desc>
<listRelation>
<relation active="#struct-300875" type="direct"></relation>
<relation active="#struct-310129" type="direct"></relation>
</listRelation>
<tutelles>
<tutelle active="#struct-300875" type="direct">
<org type="institution" xml:id="struct-300875" status="VALID">
<orgName>University College of London [London]</orgName>
<desc>
<address>
<addrLine>Gower Street, London WC1E 6BT </addrLine>
<country key="GB"></country>
</address>
<ref type="url">http://www.ucl.ac.uk/</ref>
</desc>
</org>
</tutelle>
<tutelle active="#struct-310129" type="direct">
<org type="institution" xml:id="struct-310129" status="INCOMING">
<orgName>Institute of Neurology</orgName>
<desc>
<address>
<country key="FR"></country>
</address>
</desc>
</org>
</tutelle>
</tutelles>
</hal:affiliation>
</affiliation>
</author>
</analytic>
<idno type="DOI">10.1002/ana.24790.</idno>
<series>
<title level="j">Annals of Neurology</title>
<idno type="ISSN">0364-5134</idno>
<imprint>
<date type="datePub">2016-11</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc>
<textClass></textClass>
</profileDesc>
</teiHeader>
<front>
<div type="abstract" xml:lang="0">Gaucher disease is caused by mutations in the glucocerebrosidase 1 gene that result in deficiency of the lysosomal enzyme glucocerebrosidase. Both homozygous and heterozygous glucocerebrosidase 1 mutations confer an increased risk for developing Parkinson disease. Current estimates indicate that 10 to 25% of Parkinson patients carry glucocerebrosidase 1 mutations. Ambroxol is a small molecule chaperone that has been shown to increase glucocerebrosidase activity in vitro. This study investigated the effect of ambroxol treatment on glucocerebrosidase activity and on α-synuclein and phosphorylated α-synuclein protein levels in mice.</div>
</front>
</TEI>
<hal api="V3">
<titleStmt>
<title xml:lang="en">Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.</title>
<author role="aut">
<persName>
<forename type="first">Anna</forename>
<surname>Migdalska-Richards</surname>
</persName>
<idno type="halauthorid">1478353</idno>
<affiliation ref="#struct-95915"></affiliation>
</author>
<author role="aut">
<persName>
<forename type="first">Liam</forename>
<surname>Daly</surname>
</persName>
<idno type="halauthorid">1478354</idno>
<affiliation ref="#struct-95915"></affiliation>
</author>
<author role="aut">
<persName>
<forename type="first">Erwan</forename>
<surname>Bezard</surname>
</persName>
<idno type="halauthorid">537314</idno>
<affiliation ref="#struct-467086"></affiliation>
<affiliation ref="#struct-467087"></affiliation>
</author>
<author role="aut">
<persName>
<forename type="first">Anthony H V</forename>
<surname>Schapira</surname>
</persName>
<idno type="halauthorid">766195</idno>
<affiliation ref="#struct-95915"></affiliation>
</author>
<editor role="depositor">
<persName>
<forename>Chantal</forename>
<surname>GUERIN</surname>
</persName>
<email type="md5">eb4bde61f74652335183b0bd05630b2c</email>
<email type="domain">u-bordeaux.fr</email>
</editor>
</titleStmt>
<editionStmt>
<edition n="v1" type="current">
<date type="whenSubmitted">2017-02-06 10:28:47</date>
<date type="whenModified">2017-02-07 01:00:13</date>
<date type="whenReleased">2017-02-06 10:28:47</date>
<date type="whenProduced">2016-11</date>
</edition>
<respStmt>
<resp>contributor</resp>
<name key="321070">
<persName>
<forename>Chantal</forename>
<surname>GUERIN</surname>
</persName>
<email type="md5">eb4bde61f74652335183b0bd05630b2c</email>
<email type="domain">u-bordeaux.fr</email>
</name>
</respStmt>
</editionStmt>
<publicationStmt>
<distributor>CCSD</distributor>
<idno type="halId">hal-01456949</idno>
<idno type="halUri">https://hal.archives-ouvertes.fr/hal-01456949</idno>
<idno type="halBibtex">migdalskarichards:hal-01456949</idno>
<idno type="halRefHtml">Annals of Neurology, Wiley, 2016, pp.766-775. <10.1002/ana.24790.></idno>
<idno type="halRef">Annals of Neurology, Wiley, 2016, pp.766-775. <10.1002/ana.24790.></idno>
</publicationStmt>
<seriesStmt>
<idno type="stamp" n="CNRS">CNRS - Centre national de la recherche scientifique</idno>
</seriesStmt>
<notesStmt>
<note type="audience" n="2">International</note>
<note type="popular" n="0">No</note>
<note type="peer" n="1">Yes</note>
</notesStmt>
<sourceDesc>
<biblStruct>
<analytic>
<title xml:lang="en">Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.</title>
<author role="aut">
<persName>
<forename type="first">Anna</forename>
<surname>Migdalska-Richards</surname>
</persName>
<idno type="halauthorid">1478353</idno>
<affiliation ref="#struct-95915"></affiliation>
</author>
<author role="aut">
<persName>
<forename type="first">Liam</forename>
<surname>Daly</surname>
</persName>
<idno type="halauthorid">1478354</idno>
<affiliation ref="#struct-95915"></affiliation>
</author>
<author role="aut">
<persName>
<forename type="first">Erwan</forename>
<surname>Bezard</surname>
</persName>
<idno type="halauthorid">537314</idno>
<affiliation ref="#struct-467086"></affiliation>
<affiliation ref="#struct-467087"></affiliation>
</author>
<author role="aut">
<persName>
<forename type="first">Anthony H V</forename>
<surname>Schapira</surname>
</persName>
<idno type="halauthorid">766195</idno>
<affiliation ref="#struct-95915"></affiliation>
</author>
</analytic>
<monogr>
<idno type="halJournalId" status="VALID">10490</idno>
<idno type="issn">0364-5134</idno>
<idno type="eissn">1531-8249</idno>
<title level="j">Annals of Neurology</title>
<imprint>
<publisher>Wiley</publisher>
<biblScope unit="pp">766-775</biblScope>
<date type="datePub">2016-11</date>
</imprint>
</monogr>
<idno type="doi">10.1002/ana.24790.</idno>
<idno type="pubmed">27859541</idno>
</biblStruct>
</sourceDesc>
<profileDesc>
<langUsage>
<language ident="en">English</language>
</langUsage>
<textClass>
<classCode scheme="halDomain" n="sdv">Life Sciences [q-bio]</classCode>
<classCode scheme="halTypology" n="ART">Journal articles</classCode>
</textClass>
<abstract xml:lang="0">Gaucher disease is caused by mutations in the glucocerebrosidase 1 gene that result in deficiency of the lysosomal enzyme glucocerebrosidase. Both homozygous and heterozygous glucocerebrosidase 1 mutations confer an increased risk for developing Parkinson disease. Current estimates indicate that 10 to 25% of Parkinson patients carry glucocerebrosidase 1 mutations. Ambroxol is a small molecule chaperone that has been shown to increase glucocerebrosidase activity in vitro. This study investigated the effect of ambroxol treatment on glucocerebrosidase activity and on α-synuclein and phosphorylated α-synuclein protein levels in mice.</abstract>
<abstract xml:lang="1">Mice were treated with ambroxol for 12 days. After the treatment, glucocerebrosidase activity was measured in the mouse brain lysates. The brain lysates were also analyzed for α-synuclein and phosphorylated α-synuclein protein levels.</abstract>
<abstract xml:lang="2">Ambroxol treatment resulted in increased brain glucocerebrosidase activity in (1) wild-type mice, (2) transgenic mice expressing the heterozygous L444P mutation in the murine glucocerebrosidase 1 gene, and (3) transgenic mice overexpressing human α-synuclein. Furthermore, in the mice overexpressing human α-synuclein, ambroxol treatment decreased both α-synuclein and phosphorylated α-synuclein protein levels.</abstract>
<abstract xml:lang="3">Our work supports the proposition that ambroxol should be further investigated as a potential novel disease-modifying therapy for treatment of Parkinson disease and neuronopathic Gaucher disease to increase glucocerebrosidase activity and decrease α-synuclein and phosphorylated α-synuclein protein levels. Ann Neurol 2016;80:766-775.</abstract>
</profileDesc>
</hal>
</record>

Pour manipuler ce document sous Unix (Dilib)

EXPLOR_STEP=$WICRI_ROOT/Wicri/Sante/explor/ParkinsonFranceV1/Data/Hal/Corpus
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000070 | SxmlIndent | more

Ou

HfdSelect -h $EXPLOR_AREA/Data/Hal/Corpus/biblio.hfd -nk 000070 | SxmlIndent | more

Pour mettre un lien sur cette page dans le réseau Wicri

{{Explor lien
   |wiki=    Wicri/Sante
   |area=    ParkinsonFranceV1
   |flux=    Hal
   |étape=   Corpus
   |type=    RBID
   |clé=     Hal:hal-01456949
   |texte=   Ambroxol effects in glucocerebrosidase and α-synuclein transgenic mice.
}}

Wicri

This area was generated with Dilib version V0.6.29.
Data generation: Wed May 17 19:46:39 2017. Site generation: Mon Mar 4 15:48:15 2024