Defective Gpsm2/Gαi3 signalling disrupts stereocilia development and growth cone actin dynamics in Chudley-McCullough syndrome
| dc.rights.license | open | en_US |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | MAURIAC, Stephanie A. | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | HIEN, Yeri E. | |
| dc.contributor.author | BIRD, Jonathan E. | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | DOS SANTOS CARVALHO, Steve | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | PEYROUTOU, Ronan | |
| dc.contributor.author | LEE, Sze Chim | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | MOREAU, Maite M. | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | BLANC, Jean-Michel | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | GEZER, Aysegul | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | MEDINA, Chantal | |
| hal.structure.identifier | Interdisciplinary Institute for Neuroscience [Bordeaux] [IINS] | |
| dc.contributor.author | THOUMINE, Olivier | |
| dc.contributor.author | BEER-HAMMER, Sandra | |
| dc.contributor.author | FRIEDMAN, Thomas B. | |
| dc.contributor.author | RUTTIGER, Lukas | |
| dc.contributor.author | FORGE, Andrew | |
| dc.contributor.author | NURNBERG, Bernd | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | SANS, Nathalie | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | MONTCOUQUIOL, Mireille | |
| dc.date.accessioned | 2022-07-15T12:33:33Z | |
| dc.date.available | 2022-07-15T12:33:33Z | |
| dc.date.issued | 2017-04-07 | |
| dc.identifier.issn | 2041-1723 | en_US |
| dc.identifier.uri | oai:crossref.org:10.1038/ncomms14907 | |
| dc.identifier.uri | https://oskar-bordeaux.fr/handle/20.500.12278/140504 | |
| dc.description.abstractEn | Mutations 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.sponsorship | Développment d'une infrastructure française distribuée coordonnée | en_US |
| dc.description.sponsorship | Bordeaux Region Aquitaine Initiative for Neuroscience - ANR-10-LABX-0043 | en_US |
| dc.description.sponsorship | Pathophysiologie du complexe Gai/mPins dans l'oreille interne - ANR-14-CE13-0013 | en_US |
| dc.language.iso | EN | en_US |
| dc.rights | Attribution 3.0 United States | * |
| dc.rights.uri | http://creativecommons.org/licenses/by/3.0/us/ | * |
| dc.source | crossref | |
| dc.title.en | Defective Gpsm2/Gαi3 signalling disrupts stereocilia development and growth cone actin dynamics in Chudley-McCullough syndrome | |
| dc.title.alternative | Nat Commun | en_US |
| dc.type | Article de revue | en_US |
| dc.identifier.doi | 10.1038/ncomms14907 | en_US |
| dc.subject.hal | Sciences du Vivant [q-bio]/Neurosciences [q-bio.NC] | en_US |
| dc.identifier.pubmed | 28387217 | en_US |
| bordeaux.journal | Nature Communications | en_US |
| bordeaux.page | 14907 | en_US |
| bordeaux.volume | 8 | en_US |
| bordeaux.hal.laboratories | Neurocentre Magendie - UMR-S 1215 | en_US |
| bordeaux.institution | Université de Bordeaux | en_US |
| bordeaux.institution | INSERM | en_US |
| bordeaux.peerReviewed | oui | en_US |
| bordeaux.inpress | non | en_US |
| bordeaux.import.source | dissemin | |
| hal.identifier | hal-03724003 | |
| hal.version | 1 | |
| hal.date.transferred | 2022-07-15T12:33:43Z | |
| hal.export | true | |
| workflow.import.source | dissemin | |
| dc.rights.cc | CC BY | en_US |
| bordeaux.COinS | ctx_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 |
