Blastocyst Injection of Wild Type Embryonic Stem Cells Induces Global Corrections in Mdx Mice

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dc.contributor.authorStillwell, Elizabethen
dc.contributor.authorVitale, Josephen
dc.contributor.authorZhao, Qingshien
dc.contributor.authorBeck, Amandaen
dc.contributor.authorSchneider, Joel S.en
dc.contributor.authorKhadim, Farahen
dc.contributor.authorElson, Genieen
dc.contributor.authorAltaf, Aneelaen
dc.contributor.authorYehia, Ghassanen
dc.contributor.authorDong, Jia-huien
dc.contributor.authorLiu, Jingen
dc.contributor.authorMark, Willieen
dc.contributor.authorBhaumik, Mantuen
dc.contributor.authorGrange, Robert W.en
dc.contributor.authorFraidenraich, Diegoen
dc.contributor.departmentHuman Nutrition, Foods, and Exerciseen
dc.date.accessioned2018-11-15T14:43:30Zen
dc.date.available2018-11-15T14:43:30Zen
dc.date.issued2009-03-11en
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
dc.description.versionPublished versionen
dc.format.mimetypeapplication/pdfen
dc.identifier.doihttps://doi.org/10.1371/journal.pone.0004759en
dc.identifier.eissn1932-6203en
dc.identifier.issue3en
dc.identifier.othere4759en
dc.identifier.pmid19277212en
dc.identifier.urihttp://hdl.handle.net/10919/85853en
dc.identifier.volume4en
dc.language.isoenen
dc.publisherPLOSen
dc.rightsCreative Commons Attribution 4.0 Internationalen
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.titleBlastocyst Injection of Wild Type Embryonic Stem Cells Induces Global Corrections in Mdx Miceen
dc.title.serialPLOS ONEen
dc.typeArticle - Refereeden
dc.type.dcmitypeTexten

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