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Cellular microenvironments reveal defective mechanosensing responses and elevated YAP signaling in LMNA-mutated muscle precursors

dc.contributor.authorBERTRAND, Anne
hal.structure.identifierDepartment of Mechanical Engineering and Materials Science
dc.contributor.authorZIAEI, Simindokht
hal.structure.identifierBioingénierie tissulaire [BIOTIS]
dc.contributor.authorEHRET, Camille
dc.contributor.authorDUCHEMIN, Hélène
hal.structure.identifierSorbonne Université [SU]
dc.contributor.authorMAMCHAOUI, Kamel
hal.structure.identifierSorbonne Université [SU]
dc.contributor.authorBIGOT, Anne
dc.contributor.authorMAYER, Michèle
hal.structure.identifierHôpital Raymond Poincaré [AP-HP]
dc.contributor.authorQUIJANO-ROY, Susana
hal.structure.identifierService de neurologie pédiatrique [CHU Necker]
dc.contributor.authorDESGUERRE, Isabelle
hal.structure.identifierInstitut de Myologie
dc.contributor.authorLAINÉ, Jeanne
hal.structure.identifierCHU Pitié-Salpêtrière [AP-HP]
dc.contributor.authorYAOU, Rabah
hal.structure.identifierCentre de recherche en myologie
dc.contributor.authorBONNE, Gisèle
hal.structure.identifierCentre de recherche en myologie
dc.contributor.authorCOIRAULT, Catherine
dc.date.accessioned2021-06-10T07:04:48Z
dc.date.available2021-06-10T07:04:48Z
dc.date.issued2014-06-30
dc.identifier.issn0021-9533
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/78996
dc.description.abstractEnThe mechanisms underlying the cell response to mechanical forces are crucial for muscle development and functionality. We aim to determine whether mutations of the LMNA gene (which encodes lamin A/C) causing congenital muscular dystrophy impair the ability of muscle precursors to sense tissue stiffness and to respond to mechanical challenge. We found that LMNA-mutated myoblasts embedded in soft matrix did not align along the gel axis, whereas control myoblasts did. LMNA-mutated myoblasts were unable to tune their cytoskeletal tension to the tissue stiffness as attested by inappropriate cell-matrix adhesion sites and cytoskeletal tension in soft versus rigid substrates or after mechanical challenge. Importantly, in soft two-dimensional (2D) and/or static three-dimensional (3D) conditions, LMNA-mutated myoblasts showed enhanced activation of the yes-associated protein (YAP) signaling pathway that was paradoxically reduced after cyclic stretch. siRNA-mediated downregulation of YAP reduced adhesion and actin stress fibers in LMNA myoblasts. This is the first demonstration that human myoblasts with LMNA mutations have mechanosensing defects through a YAP-dependent pathway. In addition, our data emphasize the crucial role of biophysical attributes of cellular microenvironment to the response of mechanosensing pathways in LMNA-mutated myoblasts.
dc.language.isoen
dc.publisherCompany of Biologists
dc.subject.enYes-associated protein
dc.subject.enMuscular dystrophy
dc.subject.enLMNA
dc.subject.enCell microenvironment
dc.subject.enMechanosensitivity
dc.title.enCellular microenvironments reveal defective mechanosensing responses and elevated YAP signaling in LMNA-mutated muscle precursors
dc.typeArticle de revue
dc.identifier.doi10.1242/jcs.144907
dc.subject.halSciences du Vivant [q-bio]
dc.subject.halSciences du Vivant [q-bio]/Biologie cellulaire
dc.subject.halSciences du Vivant [q-bio]/Biotechnologies
bordeaux.journalJournal of Cell Science
bordeaux.page2873-2884
bordeaux.volume127
bordeaux.hal.laboratoriesBioingénierie Tissulaire (BioTis) - U1026*
bordeaux.issue13
bordeaux.institutionCNRS
bordeaux.institutionINSERM
bordeaux.institutionCHU de Bordeaux
bordeaux.institutionInstitut Bergonié
bordeaux.peerReviewedoui
hal.identifierinserm-02426468
hal.version1
hal.origin.linkhttps://hal.archives-ouvertes.fr//inserm-02426468v1
bordeaux.COinSctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Journal%20of%20Cell%20Science&rft.date=2014-06-30&rft.volume=127&rft.issue=13&rft.spage=2873-2884&rft.epage=2873-2884&rft.eissn=0021-9533&rft.issn=0021-9533&rft.au=BERTRAND,%20Anne&ZIAEI,%20Simindokht&EHRET,%20Camille&DUCHEMIN,%20H%C3%A9l%C3%A8ne&MAMCHAOUI,%20Kamel&rft.genre=article


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