Role of Mental Retardation-Associated Dystrophin-Gene Product Dp71 in Excitatory Synapse Organization, Synaptic Plasticity and Behavioral Functions 英文参考文献.docVIP
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Role of Mental Retardation-Associated Dystrophin-Gene Product Dp71 in Excitatory Synapse Organization, Synaptic Plasticity and Behavioral Functions 英文参考文献
RoleofMentalRetardation-AssociatedDystrophin-Gene
ProductDp71inExcitatorySynapseOrganization,
SynapticPlasticityandBehavioralFunctions
FatmaDaoud1,2,AuroraCandelario-Mart?′nez3,Jean-MarieBillard4,AviAvital5,6,MalikKhelfaoui1,2 ,Yael
Rozenvald7,MaryvonneGuegan8,9,DominiqueMornet10,DanielleJaillard11,UriNudel7,JamelChelly1,2,
DalilaMart?′nez-Rojas3,SergeLaroche8,9,DavidYaffe7,CyrilleVaillend8,9*
1Institut Cochin, Universite′ Paris-Descartes, CNRS, UMR 8104, Paris, France, 2INSERM U 567, Paris, France, 3CINVESTAV, Physiology, Biophysics and Neurosciences,
Mexico City, Mexico, 4Centre de Psychiatrie et Neurosciences, INSERM, UMR 894, Universite′ Paris Descartes, Faculte′ de Me′decine Rene′ Descartes, Paris, France,
5DepartmentofPsychologyandTheCenterforPsychobiologicalResearch,YezreelValleyCollege,EmekYezreel,Israel,6Neurobiology,WeizmannInstituteofScience,
Rehovot,Israel,7MolecularCellBiology,WeizmannInstituteofScience,Rehovot,Israel,8CNRS,Neurobiologiedel’Apprentissage,delaMe′moireetdelaCommunication,
UMR8620,Orsay,France,9UnivParis-Sud,Orsay,France, 10INSERM,EquipeESPRI25Muscleet Pathologies,Universite′ deMontpellierI,UFRdeMe′decine,EA4202,
Montpellier,France,11UnivParis-Sud,CNRS,UMR8080,CentreCommundeMicroscopieElectronique(CCME),Orsay,France
Abstract
Background:Duchennemusculardystrophy(DMD)iscausedbydeficientexpressionofthecytoskeletalprotein,dystrophin.
OnethirdofDMDpatientsalsohavementalretardation(MR),likelyduetomutationspreventingexpressionofdystrophin
andotherbrainproductsoftheDMDgeneexpressedfromdistinctinternalpromoters.LossofDp71,themajorDMD-gene
productinbrain,isthoughttocontributetotheseverityofMR;however,thespecificfunctionofDp71ispoorlyunderstood.
Methodology/PrincipalFindings:ComplementaryapproacheswereusedtoexploretheroleofDp71inneuronalfunction
andidentifymechanismsbywhichDp71lossmayimpairneuronalandcognitivefunctions.Besidesthenormalexpression
of Dp71 in a subpopulation of astrocytes, we found that a pool of Dp71 colocalizes with synaptic
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