Afficher la notice abrégée

dc.rights.licenseopenen_US
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorMAURIAC, Stephanie A.
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorHIEN, Yeri E.
dc.contributor.authorBIRD, Jonathan E.
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorDOS SANTOS CARVALHO, Steve
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorPEYROUTOU, Ronan
dc.contributor.authorLEE, Sze Chim
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorMOREAU, Maite M.
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorBLANC, Jean-Michel
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorGEZER, Aysegul
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorMEDINA, Chantal
hal.structure.identifierInterdisciplinary Institute for Neuroscience [Bordeaux] [IINS]
dc.contributor.authorTHOUMINE, Olivier
dc.contributor.authorBEER-HAMMER, Sandra
dc.contributor.authorFRIEDMAN, Thomas B.
dc.contributor.authorRUTTIGER, Lukas
dc.contributor.authorFORGE, Andrew
dc.contributor.authorNURNBERG, Bernd
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorSANS, Nathalie
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorMONTCOUQUIOL, Mireille
dc.date.accessioned2022-07-15T12:33:33Z
dc.date.available2022-07-15T12:33:33Z
dc.date.issued2017-04-07
dc.identifier.issn2041-1723en_US
dc.identifier.urioai:crossref.org:10.1038/ncomms14907
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/140504
dc.description.abstractEnMutations in GPSM2 cause Chudley-McCullough syndrome (CMCS), an autosomal recessive neurological disorder characterized by early-onset sensorineural deafness and brain anomalies. Here, we show that mutation of the mouse orthologue of GPSM2 affects actin-rich stereocilia elongation in auditory and vestibular hair cells, causing deafness and balance defects. The G-protein subunit Gαi3, a well-documented partner of Gpsm2, participates in the elongation process, and its absence also causes hearing deficits. We show that Gpsm2 defines an ∼200 nm nanodomain at the tips of stereocilia and this localization requires the presence of Gαi3, myosin 15 and whirlin. Using single-molecule tracking, we report that loss of Gpsm2 leads to decreased outgrowth and a disruption of actin dynamics in neuronal growth cones. Our results elucidate the aetiology of CMCS and highlight a new molecular role for Gpsm2/Gαi3 in the regulation of actin dynamics in epithelial and neuronal tissues.
dc.description.sponsorshipDéveloppment d'une infrastructure française distribuée coordonnéeen_US
dc.description.sponsorshipBordeaux Region Aquitaine Initiative for Neuroscience - ANR-10-LABX-0043en_US
dc.description.sponsorshipPathophysiologie du complexe Gai/mPins dans l'oreille interneen_US
dc.language.isoENen_US
dc.rightsAttribution 3.0 United States*
dc.rights.urihttp://creativecommons.org/licenses/by/3.0/us/*
dc.sourcecrossref
dc.title.enDefective Gpsm2/Gαi3 signalling disrupts stereocilia development and growth cone actin dynamics in Chudley-McCullough syndrome
dc.title.alternativeNat Communen_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1038/ncomms14907en_US
dc.subject.halSciences du Vivant [q-bio]/Neurosciences [q-bio.NC]en_US
dc.identifier.pubmed28387217en_US
bordeaux.journalNature Communicationsen_US
bordeaux.page14907en_US
bordeaux.volume8en_US
bordeaux.hal.laboratoriesNeurocentre Magendie - UMR-S 1215en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.institutionINSERMen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
bordeaux.import.sourcedissemin
hal.identifierhal-03724003
hal.version1
hal.date.transferred2022-07-15T12:33:43Z
hal.exporttrue
workflow.import.sourcedissemin
dc.rights.ccCC BYen_US
bordeaux.COinSctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Nature%20Communications&rft.date=2017-04-07&rft.volume=8&rft.spage=14907&rft.epage=14907&rft.eissn=2041-1723&rft.issn=2041-1723&rft.au=MAURIAC,%20Stephanie%20A.&HIEN,%20Yeri%20E.&BIRD,%20Jonathan%20E.&DOS%20SANTOS%20CARVALHO,%20Steve&PEYROUTOU,%20Ronan&rft.genre=article


Fichier(s) constituant ce document

Thumbnail
Thumbnail

Ce document figure dans la(les) collection(s) suivante(s)

Afficher la notice abrégée