Role of Disulfide Cross-Linking of Mutant SOD1 in the Formation of Inclusion-Body-Like Structures 英文参考文献.docVIP

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Role of Disulfide Cross-Linking of Mutant SOD1 in the Formation of Inclusion-Body-Like Structures 英文参考文献.doc

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Role of Disulfide Cross-Linking of Mutant SOD1 in the Formation of Inclusion-Body-Like Structures 英文参考文献

RoleofDisulfideCross-LinkingofMutantSOD1inthe FormationofInclusion-Body-LikeStructures BrittanyL.T.Roberts.,KinareePatel.,HildaH.Brown,DavidR.Borchelt* DepartmentofNeuroscience,CenterforTranslationalResearchinNeurodegenerativeDisease,SantaFeHealthCareAlzheimer’sDiseaseResearchCenter,McKnightBrain Institute,UniversityofFlorida,Gainesville,Florida,UnitedStatesofAmerica Abstract Background:Pathologicaggregatesofsuperoxidedismutase1(SOD1)harboringmutationslinkedtofamilialamyotrophic lateral sclerosis (fALS) have been shown to contain aberrant intermolecular disulfide cross-links. In prior studies, we observed that intermolecular bonding was not necessary in the formation of detergent- insoluble SOD1 complexes by mutant SOD1, but we were unable to assess whether this type of bonding may be important for pathologic inclusion formation. In the present study, we visually assess the formation of large inclusions by fusing mutant SOD1 to yellow fluorescentprotein(YFP). Methodology/PrincipalFindings:ExperimentalconstructspossessingmutationsatallcysteineresiduesinSOD1(sites6,57, 111,and146toF,S,Y,RorG,S,Y,R,respectively)wereshowntomaintainahighpropensityofinclusionformationdespitethe inability to form disulfide cross-links. Interestingly, although aggregates form when all cysteines were mutated, double mutantsoftheALSmutationC6GwithanexperimentalmutationC111Sexhibitedlowaggregationpropensity. Conclusions/Significance: Overall, this study is an extension of previous work demonstrating that cysteine residues in mutantSOD1playaroleinmodulatingaggregationandthatintermoleculardisulfidebondsarenotrequiredtoproduce largeintracellularinclusion-likestructures. Citation: Roberts BLT, Patel K, Brown HH, Borchelt DR (2012) Role of Disulfide Cross-Linking of Mutant SOD1 in the Formation of Inclusion-Body-Like Structures.PLoSONE7(10):e47838.doi:10.1371/journal.pone.0047838 Editor:HuaibinCai,NationalInstituteofHealth,UnitedStatesofAmerica ReceivedAugust9,2012;AcceptedSeptember21,2012;Publishe