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dc.contributor.authorStillwell, Elizabethen_US
dc.contributor.authorVitale, Josephen_US
dc.contributor.authorZhao, Qingshien_US
dc.contributor.authorBeck, Amandaen_US
dc.contributor.authorSchneider, Joelen_US
dc.contributor.authorKhadim, Farahen_US
dc.contributor.authorElson, Genieen_US
dc.contributor.authorAltaf, Aneelaen_US
dc.contributor.authorYehia, Ghassanen_US
dc.contributor.authorDong, Jia-huien_US
dc.contributor.authorLiu, Jingen_US
dc.contributor.authorMark, Willieen_US
dc.contributor.authorBhaumik, Mantuen_US
dc.contributor.authorGrange, Roberten_US
dc.contributor.authorFraidenraich, Diegoen_US
dc.date.accessioned2018-11-15T14:43:30Z
dc.date.available2018-11-15T14:43:30Z
dc.date.issued2009-03-11en_US
dc.identifier.othere4759en_US
dc.identifier.urihttp://hdl.handle.net/10919/85853
dc.description.abstractDuchenne muscular dystrophy (DMD) is an incurable neuromuscular degenerative disease, caused by a mutation in the dystrophin gene. Mdx mice recapitulate DMD features. Here we show that injection of wild-type (WT) embryonic stem cells (ESCs) into mdx blastocysts produces mice with improved pathology and function. A small fraction of WT ESCs incorporates into the mdx mouse nonuniformly to upregulate protein levels of dystrophin in the skeletal muscle. The chimeric muscle shows reduced regeneration and restores dystrobrevin, a dystrophin-related protein, in areas with high and with low dystrophin content. WT ESC injection increases the amount of fat in the chimeras to reach WT levels. ESC injection without dystrophin does not prevent the appearance of phenotypes in the skeletal muscle or in the fat. Thus, dystrophin supplied by the ESCs reverses disease in mdx mice globally in a dose-dependent manner.en_US
dc.format.mimetypeapplication/pdfen_US
dc.language.isoen_USen_US
dc.publisherPLOSen_US
dc.rightsCreative Commons Attribution 4.0 Internationalen_US
dc.rights.urihttps://creativecommons.org/licenses/by/4.0en_US
dc.titleBlastocyst Injection of Wild Type Embryonic Stem Cells Induces Global Corrections in Mdx Miceen_US
dc.typeArticle - Refereeden_US
dc.description.versionPeer Revieweden_US
dc.title.serialPLOS ONEen_US
dc.identifier.doihttps://doi.org/10.1371/journal.pone.0004759en_US
dc.identifier.volume4en_US
dc.identifier.issue3en_US
dc.type.dcmitypeTexten_US
dc.identifier.pmid19277212en_US
dc.identifier.eissn1932-6203en_US


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Creative Commons Attribution 4.0 International
License: Creative Commons Attribution 4.0 International