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1、HMGAdvanceAccesspublishedMarch23,20151PerinatalGjb2genetransferrescueshearinginamousemodelofhereditarydeafness1122TakashiIizuka,KazusakuKamiya,SatoruGotoh,YoshinobuSuginani,MasaakiDownloadedfrom32,42,41,Suzuki,TetsuoNoda,OsamuMinowaandKatsuhisaIkeda*1DepartmentofOtorhinolaryngology,JuntendoUniv
2、ersityFacultyofMedicine,Tokyohttp://hmg.oxfordjournals.org/113-84212DepartmentofCellBiology,JapaneseFoundationforCancerResearch,CancerInstitute,Tokyo135-8550atChinaUniversityofGeosciencesWuhanCampusonMay18,20153DepartmentofOtolaryngology,TeikyoUniversityChibaMedicalCenter,Ichihara299-01114Teamf
3、orAdvancedDevelopmentandEvaluationofHumanDiseaseModels,RIKENBioResourceCenter,Tsukuba305-0074,Japan*Towhomcorrespondenceshouldbeaddressedto(ike@juntendo.ac.jp),DepartmentofOtorhinolaryngology,JuntendoUniversityFacultyofMedicine,2-1-1Hongo,Bunkyo-ku,Tokyo113-8421,Japan.Tel:+81-3-5802-1094;Fax:+8
4、1-3-5689-0547;Email:ike@juntendo.ac.jp©TheAuthor2015.PublishedbyOxfordUniversityPress.Allrightsreserved.ForPermissions,pleaseemail:journals.permissions@oup.com2ABSTRACTHearinglossisthemostwidespreadsensorydisorder,withanincidenceofcongenitalgeneticdeafnessof1in1,600children.Formanyethnicpopulat
5、ions,themostprevalentformDownloadedfromofgeneticdeafnessiscausedbyrecessivemutationsinthegenegapjunctionprotein,beta2,26kDa(GJB2),whichisalsoknownasconnexin26(Cx26).Despitethisknowledge,existinghttp://hmg.oxfordjournals.org/treatmentstrategiesdonotcompletelyrecoverspeechperception.Hereweusedage
6、nedeliverysystemtorescuehearinginamousemodelofGjb2deletion.MicelackingCx26arecharacterizedbyprofounddeafnessfrombirthandimproperdevelopmentofcochlearcells.atChinaUniversityofGeosciencesWuhanCampusonMay18,2015CochleardeliveryofGjb2usinganadeno-associatedvirus(AAV)significantlyimprovedtheauditory
7、responsesanddevelopmentofthecochlearstructure.UsinggenereplacementtorestorehearinginanewmousemodelofGjb2-relateddeafnessmayleadtothedevelopmentoftherapiesforhumanhereditarydeafness.3INTRODUCTIONSeveretoprofoundgenetichearinglossaf
