ArticleDefectsintheAlternativeSplicing-DependentRegulationofRESTCauseDeafnessGraphicalAbstractHighlightsdAlternativesplicingofRESTexon4regulatesgeneexpressioninhaircellsdARESTmutationisassociatedwithdefectiveexon4splicinganddeafnessinhumansdGeneticdeletionofexon4ofmouseRestcausessensoryhaircelllossanddeafnessdTheHDACinhibitorSAHA(Vorinostat)rescueshearinginRestmutantmiceAuthorsYokoNakano,MichaelC.Kelly,AtteeqU.Rehman,...,MatthewW.Kelley,ThomasB.Friedman,BotondBa´nfiCorrespondencebotond-banfi@uiowa.eduInBriefARESTmutationimpactingitsalternativesplicingdisruptsgeneexpressioninauditoryhaircells,leadingtodeafness,andtargetingREST’spartnerswithHDACinhibitorscanrescuehearinginRestmutantmice.Nakanoetal.,2018,Cell174,536–548July26,2018ª2018ElsevierInc.https://doi.org/10.1016/j.cell.2018.06.004ArticleDefectsintheAlternativeSplicing-DependentRegulationofRESTCauseDeafnessYokoNakano,1,2MichaelC.Kelly,3,9AtteeqU.Rehman,4,5,9ErichT.Boger,6RobertJ.Morell,6MatthewW.Kelley,3ThomasB.Friedman,4andBotondBa´nfi1,2,7,8,10,*1DepartmentofAnatomyandCellBiology,CarverCollegeofMedicine,UniversityofIowa,IowaCity,IA52242,USA2InflammationProgram,CarverCollegeofMedicine,UniversityofIowa,IowaCity,IA52242,USA3LaboratoryofCochlearDevelopment,NationalInstituteonDeafnessandOtherCommunicationDisorders,NIH,Bethesda,MD20892,USA4LaboratoryofMolecularGenetics,NationalInstituteonDeafnessandOtherCommunicationDisorders,NIH,Bethesda,MD20892,USA5DepartmentofMolecularandHumanGenetics,BaylorCollegeofMedicine,Houston,TX77030,USA6GenomicsandComputationalBiologyCore,NationalInstituteonDeafnessandOtherCommunicationDisorders,NIH,Bethesda,MD20892,USA7DepartmentofOtolaryngology—HeadandNeckSurgery,CarverCollegeofMedicine,UniversityofIowa,IowaCity,IA52242,USA8DepartmentofInternalMedicine,CarverCollegeofMedicine,UniversityofIowa,IowaCity,IA52242,USA9Theseauthorscontributedequally10LeadContact*Correspondence:botond-banfi@uiowa.eduhttps://doi.org/10.1016/j.cell.2018.06.004SUMMARYTheDNA-bindingproteinRESTformscomplex...
