Differential Requirement for Utrophin in the Induced Pluripotent Stem Cell Correction of Muscle versus Fat in Muscular Dystrophy Mice

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dc.contributor.authorBeck, Amanda J.en
dc.contributor.authorVitale, Joseph M.en
dc.contributor.authorZhao, Qingshien
dc.contributor.authorSchneider, Joel S.en
dc.contributor.authorChang, Coreyen
dc.contributor.authorAltaf, Aneelaen
dc.contributor.authorMichaels, Jenniferen
dc.contributor.authorBhaumik, Mantuen
dc.contributor.authorGrange, Robert W.en
dc.contributor.authorFraidenraich, Diegoen
dc.contributor.departmentHuman Nutrition, Foods, and Exerciseen
dc.date.accessioned2018-11-07T18:25:54Zen
dc.date.available2018-11-07T18:25:54Zen
dc.date.issued2011-05-16en
dc.description.abstractDuchenne muscular dystrophy (DMD) is an incurable degenerative muscle disorder. We injected WT mouse induced pluripotent stem cells (iPSCs) into mdx and mdx∶utrophin mutant blastocysts, which are predisposed to develop DMD with an increasing degree of severity (mdx <<< mdx∶utrophin). In mdx chimeras, iPSC-dystrophin was supplied to the muscle sarcolemma to effect corrections at morphological and functional levels. Dystrobrevin was observed in dystrophin-positive and, at a lesser extent, utrophin-positive areas. In the mdx∶utrophin mutant chimeras, although iPSC-dystrophin was also supplied to the muscle sarcolemma, mice still displayed poor skeletal muscle histopathology, and negligible levels of dystrobrevin in dystrophin- and utrophin-negative areas. Not only dystrophin-expressing tissues are affected by iPSCs. Mdx and mdx∶utrophin mice have reduced fat/body weight ratio, but iPSC injection normalized this parameter in both mdx and mdx∶utrophin chimeras, despite the fact that utrophin was compromised in the mdx∶utrophin chimeric fat. The results suggest that the presence of utrophin is required for the iPSC-corrections in skeletal muscle. Furthermore, the results highlight a potential (utrophin-independent) non-cell autonomous role for iPSC-dystrophin in the corrections of non-muscle tissue like fat, which is intimately related to the muscle.en
dc.description.versionPublished versionen
dc.format.mimetypeapplication/pdfen
dc.identifier.doihttps://doi.org/10.1371/journal.pone.0020065en
dc.identifier.eissn1932-6203en
dc.identifier.issue5en
dc.identifier.othere20065en
dc.identifier.pmid21603573en
dc.identifier.urihttp://hdl.handle.net/10919/85783en
dc.identifier.volume6en
dc.language.isoenen
dc.publisherPLOSen
dc.rightsCreative Commons Attribution 4.0 Internationalen
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.titleDifferential Requirement for Utrophin in the Induced Pluripotent Stem Cell Correction of Muscle versus Fat in Muscular Dystrophy Miceen
dc.title.serialPLOS ONEen
dc.typeArticle - Refereeden
dc.type.dcmitypeTexten

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