SMN Requirement for Synaptic Vesicle, Active Zone and Microtubule Postnatal Organization in Motor Nerve Terminals 英文参考文献.docVIP

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SMN Requirement for Synaptic Vesicle, Active Zone and Microtubule Postnatal Organization in Motor Nerve Terminals 英文参考文献.doc

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SMN Requirement for Synaptic Vesicle, Active Zone and Microtubule Postnatal Organization in Motor Nerve Terminals 英文参考文献

SMNRequirementforSynapticVesicle,ActiveZoneand MicrotubulePostnatalOrganizationinMotorNerve Terminals LauraTorres-Benito,MargretFeodoraNeher,RaquelCano,RocioRuiz,LuciaTabares* DepartmentofMedicalPhysiologyandBiophysics,SchoolofMedicine,UniversityofSeville,Seville,Spain Abstract Low levels of the Survival Motor Neuron (SMN) protein produce Spinal Muscular Atrophy (SMA), a severe monogenetic diseaseininfantscharacterizedbymuscleweaknessandimpairedsynaptictransmission.Wereporthereseverestructural andfunctionalalterationsintheorganizationoftheorganellesandthecytoskeletonofmotornerveterminalsinamouse model of SMA. The decrease in SMN levels resulted in the clustering of synaptic vesicles (SVs) and Active Zones (AZs), reductioninthesizeofthereadilyreleasablepool(RRP),andtherecyclingpool(RP)ofsynapticvesicles,adecreaseinactive mitochondriaandlimitingofneurofilamentandmicrotubulematuration.WeproposethatSMNisessentialforthenormal postnatalmaturationofmotornerveterminalsandthatSMNdeficiencydisruptsthepresynapticorganizationleadingto neurodegeneration. Citation:Torres-BenitoL,NeherMF,CanoR,RuizR,TabaresL(2011)SMNRequirementforSynapticVesicle,ActiveZoneandMicrotubulePostnatalOrganization inMotorNerveTerminals.PLoSONE6(10):e26164.doi:10.1371/journal.pone.0026164 Editor:MelB.Feany,BrighamandWomen’sHospital,HarvardMedicalSchool,UnitedStatesofAmerica ReceivedAugust2,2011;AcceptedSeptember21,2011;PublishedOctober12,2011 Copyright:?2011Torres-Benitoetal.Thisisanopen-accessarticledistributedunderthetermsoftheCreativeCommonsAttributionLicense,whichpermits unrestricteduse,distribution,andreproductioninanymedium,providedtheoriginalauthorandsourcearecredited. Funding: The financial support of the following research grants is gratefully acknowledged: FUNDAME (Gename Project), Spanish Ministry of Science and InnovationBFU2010-21648,andJuntadeAndaluc?′aP09-CVI-4862.Thefundershadnoroleinstudydesign,datacollectionandanalysis,decisiontopublish,or preparationofthemanuscript. CompetingInterests:T