Membrane repair defects in muscular dystrophy

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1、SupplementalMaterialcanbefoundat:http://www.jbc.org/content/suppl/2009/04/22/M109.009589.DC1.htmlhttp://www.jbc.org/content/suppl/2009/05/28/284.23.15894.DC1.htmlTHEJOURNALOFBIOLOGICALCHEMISTRYVOL.284,NO.23,pp.15894–15902,June5,2009©2009byTheAmericanSocietyforBiochemistryandMolecularBiology,Inc.Pri

2、ntedintheU.S.A.MembraneRepairDefectsinMuscularDystrophyAreLinkedtoAlteredInteractionbetweenMG53,Caveolin-3,andDysferlin*□SReceivedforpublication,February19,2009,andinrevisedform,April17,2009Published,JBCPapersinPress,April20,2009,DOI10.1074/jbc.M109.009589‡1‡1‡§¶
ChuanxiCai,NoahWeisleder,Jae-KyunK

3、o,ShinjiKomazaki,YoshihideSunada,MiyukiNishi,
‡2HiroshiTakeshima,andJianjieMa**‡FromtheDepartmentsofPhysiologyandBiophysicsand**Medicine,RobertWoodJohnsonMedicalSchool,§Piscataway,NewJersey08854,theDepartmentofAnatomy,SaitamaMedicalSchool,Saitama350-0495,Japan,¶
theDepartmentofInternalMedicine,Kawa

4、sakiMedicalSchool,Kurashiki701-0192,Japan,andtheDepartmentofBiologicalChemistry,KyotoUniversityGraduateSchoolofPharmaceuticalSciences,Kyoto606-8501,JapanDownloadedfromDefectivemembranerepaircancontributetotheprogressionlarvesiclestoinjurysites(4,5).Oneproteinthathasbeenlinkedofmusculardystrophy.Alt

5、houghmutationsincaveolin-3tomembranerepairinskeletalmuscleisdysferlin(6,7),which(Cav3)anddysferlinarelinkedtomusculardystrophyinhumanisthoughttofuseintracellularvesiclestopatchthedamagedpatients,themolecularmechanismunderlyingthefunctionalmembraneandrestoresarcolemmalintegrityfollowingmuscleinterpl

6、aybetweenCav3anddysferlininmembranerepairofinjury.Likedysferlin,caveolin-3(Cav3)3isamuscle-specificwww.jbc.orgmusclephysiologyanddiseasehasnotbeenfullyresolved.Weprotein,andmanymutationsinCav3,includingP104L,R26Q,recentlydiscoveredthatmitsugumin53(MG53),amuscle-spe-andC71W,havebeenlinkedtomusculard

7、ystrophy(8–11).cificTRIM(Tri-partitemotif)familyprotein(TRIM72),con-DespiteextensiveresearcheffortsonCav3anddysferlin(12–14),atFUDANUNIVERSITYSCHOOFPHARMACY,onNovember11,2012tributestointracellularvesicletr