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dc.creatorTorres Benito, Lauraes
dc.creatorFeodora Neher, Margretes
dc.creatorCano, Raqueles
dc.creatorTabares, Lucíaes
dc.date.accessioned2021-04-20T11:45:12Z
dc.date.available2021-04-20T11:45:12Z
dc.date.issued2011-10-12
dc.identifier.citationTorres Benito, L., Feodora Neher, M., Cano, R. y Tabares Domínguez, L. (2011). SMN Requirement for Synaptic Vesicle, Active Zone and Microtubule Postnatal Organization in Motor Nerve Terminals. PLoS ONE, 6 (10)
dc.identifier.issn1932-6203 (electrónico)es
dc.identifier.urihttps://hdl.handle.net/11441/107461
dc.description.abstractLow levels of the Survival Motor Neuron (SMN) protein produce Spinal Muscular Atrophy (SMA), a severe monogenetic disease in infants characterized by muscle weakness and impaired synaptic transmission. We report here severe structural and functional alterations in the organization of the organelles and the cytoskeleton of motor nerve terminals in a mouse model of SMA. The decrease in SMN levels resulted in the clustering of synaptic vesicles (SVs) and Active Zones (AZs), reduction in the size of the readily releasable pool (RRP), and the recycling pool (RP) of synaptic vesicles, a decrease in active mitochondria and limiting of neurofilament and microtubule maturation. We propose that SMN is essential for the normal postnatal maturation of motor nerve terminals and that SMN deficiency disrupts the presynaptic organization leading to neurodegeneration.es
dc.formatapplication/pdfes
dc.format.extent16 p.es
dc.language.isoenges
dc.relation.ispartofPLoS ONE, 6 (10)
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subjectSMNes
dc.subjectSynaptic Vesiclees
dc.subjectMicrotubule Postnatales
dc.subjectSpinal Muscular Atrophyes
dc.subjectSMAes
dc.titleSMN Requirement for Synaptic Vesicle, Active Zone and Microtubule Postnatal Organization in Motor Nerve Terminalses
dc.typeinfo:eu-repo/semantics/articlees
dcterms.identifierhttps://ror.org/03yxnpp24
dc.type.versioninfo:eu-repo/semantics/publishedVersiones
dc.rights.accessRightsinfo:eu-repo/semantics/openAccesses
dc.contributor.affiliationUniversidad de Sevilla. Departamento de Fisiología Médica y Biofísicaes
dc.relation.publisherversionhttps://doi.org/10.1371/journal.pone.0026164es
dc.identifier.doi10.1371/journal.pone.0026164es
dc.journaltitlePLoS ONEes
dc.publication.volumen6es
dc.publication.issue10es

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Attribution-NonCommercial-NoDerivatives 4.0 Internacional
Except where otherwise noted, this item's license is described as: Attribution-NonCommercial-NoDerivatives 4.0 Internacional