Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice
Duchenne 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
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| Veröffentlicht in: | PloS one 2011-05, Vol.6 (5), p.e20065-e20065 |
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| creator | Beck, Amanda J Vitale, Joseph M Zhao, Qingshi Schneider, Joel S Chang, Corey Altaf, Aneela Michaels, Jennifer Bhaumik, Mantu Grange, Robert Fraidenraich, Diego |
| description | Duchenne 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 |
| doi_str_mv | 10.1371/journal.pone.0020065 |
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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.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0020065</identifier><identifier>PMID: 21603573</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Adipocytes ; Adipose Tissue ; Animal tissues ; Animals ; Artificial chromosomes ; Biology ; Blastocyst ; Blastocysts ; Body Composition ; Body Weight ; Chimeras ; Dentistry ; Duchenne's muscular dystrophy ; Dystrobrevin ; Dystrophin ; Dystrophy ; Genes ; Histopathology ; Induced Pluripotent Stem Cells - transplantation ; Mechanical properties ; Medical schools ; Medicine ; Mice ; Muscle, Skeletal ; Muscular dystrophy ; Muscular Dystrophy, Animal - therapy ; Musculoskeletal system ; Mutation ; Physiology ; Pluripotency ; Proteins ; Rodents ; Sarcolemma ; Skeletal muscle ; Stem cell transplantation ; Stem Cell Transplantation - methods ; Stem cells ; Utrophin ; Utrophin - administration & dosage ; Utrophin - pharmacology ; Weight reduction</subject><ispartof>PloS one, 2011-05, Vol.6 (5), p.e20065-e20065</ispartof><rights>COPYRIGHT 2011 Public Library of Science</rights><rights>2011 Beck et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License: https://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>Beck et al. 2011</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c691t-b2f80b142dfd884f3413e05589513c42aa3fb68d4c16249856d132274d080ba93</citedby><cites>FETCH-LOGICAL-c691t-b2f80b142dfd884f3413e05589513c42aa3fb68d4c16249856d132274d080ba93</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3095639/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3095639/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,864,885,2102,2928,23866,27924,27925,53791,53793,79472,79473</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21603573$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Lee, Se-Jin</contributor><creatorcontrib>Beck, Amanda J</creatorcontrib><creatorcontrib>Vitale, Joseph M</creatorcontrib><creatorcontrib>Zhao, Qingshi</creatorcontrib><creatorcontrib>Schneider, Joel S</creatorcontrib><creatorcontrib>Chang, Corey</creatorcontrib><creatorcontrib>Altaf, Aneela</creatorcontrib><creatorcontrib>Michaels, Jennifer</creatorcontrib><creatorcontrib>Bhaumik, Mantu</creatorcontrib><creatorcontrib>Grange, Robert</creatorcontrib><creatorcontrib>Fraidenraich, Diego</creatorcontrib><title>Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice</title><title>PloS one</title><addtitle>PLoS One</addtitle><description>Duchenne 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.</description><subject>Adipocytes</subject><subject>Adipose Tissue</subject><subject>Animal tissues</subject><subject>Animals</subject><subject>Artificial chromosomes</subject><subject>Biology</subject><subject>Blastocyst</subject><subject>Blastocysts</subject><subject>Body Composition</subject><subject>Body Weight</subject><subject>Chimeras</subject><subject>Dentistry</subject><subject>Duchenne's muscular dystrophy</subject><subject>Dystrobrevin</subject><subject>Dystrophin</subject><subject>Dystrophy</subject><subject>Genes</subject><subject>Histopathology</subject><subject>Induced Pluripotent Stem Cells - transplantation</subject><subject>Mechanical properties</subject><subject>Medical schools</subject><subject>Medicine</subject><subject>Mice</subject><subject>Muscle, Skeletal</subject><subject>Muscular dystrophy</subject><subject>Muscular Dystrophy, Animal - therapy</subject><subject>Musculoskeletal system</subject><subject>Mutation</subject><subject>Physiology</subject><subject>Pluripotency</subject><subject>Proteins</subject><subject>Rodents</subject><subject>Sarcolemma</subject><subject>Skeletal muscle</subject><subject>Stem cell transplantation</subject><subject>Stem Cell Transplantation - methods</subject><subject>Stem cells</subject><subject>Utrophin</subject><subject>Utrophin - administration & dosage</subject><subject>Utrophin - pharmacology</subject><subject>Weight reduction</subject><issn>1932-6203</issn><issn>1932-6203</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>DOA</sourceid><recordid>eNqNk9tq3DAQhk1padJt36C0gkJLL3ark2XpJhDSUyAQ6OlWyJa0q2Bbjg6h-wB978rJJmRLLooNksff_0ua0VTVSwRXiDTow4XPYVT9avKjWUGIIWT1o-oQCYKXDEPy-N78oHoW4wWENeGMPa0OMGKQ1A05rP58dNaaYMbkVA-CucwumKF8AusDyCn4aeNGUN60MWXQuTMaTH0ObvJp5mIyA-hM34POh2C65PwIvAVDjl1vwJUJMUdgVZpN5mDuVQB6G6-9t2BwnXlePbGqj-bFblxUPz9_-nHydXl2_uX05Phs2TGB0rLFlsMWUayt5pxaQhExsK65qBHpKFaK2JZxTTvEMBW8ZhoRjBuqYdEpQRbV6xvfqfdR7jIYJcJCQCQwawpxekNory7kFNygwlZ65eR1wIe1VCG5cjKJuSYtbaFASlNIFK8pNdwyYVuNeKOL19FutdwORnclW0H1e6b7f0a3kWt_JQkUNSPzdt_tDIK_zCYmObg4p1qNxucoOeOkEbgUeFG9-Yd8-HA7aq3K_t1ofVm2mz3lMW0YF6hmqFCrB6jyaFNqVW6bdSW-J3i_JyhMMr_TWuUY5en3b__Pnv_aZ9_eYzdG9WkTfZ_nGxb3QXoDdsHHGIy9yzGCcm6W22zIuVnkrlmK7NX9-tyJbruD_AUicRED</recordid><startdate>20110516</startdate><enddate>20110516</enddate><creator>Beck, Amanda J</creator><creator>Vitale, Joseph M</creator><creator>Zhao, Qingshi</creator><creator>Schneider, Joel S</creator><creator>Chang, Corey</creator><creator>Altaf, Aneela</creator><creator>Michaels, Jennifer</creator><creator>Bhaumik, Mantu</creator><creator>Grange, Robert</creator><creator>Fraidenraich, Diego</creator><general>Public Library of Science</general><general>Public Library of Science (PLoS)</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>IOV</scope><scope>ISR</scope><scope>3V.</scope><scope>7QG</scope><scope>7QL</scope><scope>7QO</scope><scope>7RV</scope><scope>7SN</scope><scope>7SS</scope><scope>7T5</scope><scope>7TG</scope><scope>7TM</scope><scope>7U9</scope><scope>7X2</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABJCF</scope><scope>ABUWG</scope><scope>AEUYN</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>ATCPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>D1I</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB.</scope><scope>KB0</scope><scope>KL.</scope><scope>L6V</scope><scope>LK8</scope><scope>M0K</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>M7S</scope><scope>NAPCQ</scope><scope>P5Z</scope><scope>P62</scope><scope>P64</scope><scope>PATMY</scope><scope>PDBOC</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PTHSS</scope><scope>PYCSY</scope><scope>RC3</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope></search><sort><creationdate>20110516</creationdate><title>Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice</title><author>Beck, Amanda J ; Vitale, Joseph M ; Zhao, Qingshi ; Schneider, Joel S ; Chang, Corey ; Altaf, Aneela ; Michaels, Jennifer ; Bhaumik, Mantu ; Grange, Robert ; Fraidenraich, Diego</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c691t-b2f80b142dfd884f3413e05589513c42aa3fb68d4c16249856d132274d080ba93</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Adipocytes</topic><topic>Adipose Tissue</topic><topic>Animal tissues</topic><topic>Animals</topic><topic>Artificial chromosomes</topic><topic>Biology</topic><topic>Blastocyst</topic><topic>Blastocysts</topic><topic>Body Composition</topic><topic>Body Weight</topic><topic>Chimeras</topic><topic>Dentistry</topic><topic>Duchenne's muscular dystrophy</topic><topic>Dystrobrevin</topic><topic>Dystrophin</topic><topic>Dystrophy</topic><topic>Genes</topic><topic>Histopathology</topic><topic>Induced Pluripotent Stem Cells - transplantation</topic><topic>Mechanical properties</topic><topic>Medical schools</topic><topic>Medicine</topic><topic>Mice</topic><topic>Muscle, Skeletal</topic><topic>Muscular dystrophy</topic><topic>Muscular Dystrophy, Animal - therapy</topic><topic>Musculoskeletal system</topic><topic>Mutation</topic><topic>Physiology</topic><topic>Pluripotency</topic><topic>Proteins</topic><topic>Rodents</topic><topic>Sarcolemma</topic><topic>Skeletal muscle</topic><topic>Stem cell transplantation</topic><topic>Stem Cell Transplantation - methods</topic><topic>Stem cells</topic><topic>Utrophin</topic><topic>Utrophin - administration & dosage</topic><topic>Utrophin - pharmacology</topic><topic>Weight reduction</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Beck, Amanda J</creatorcontrib><creatorcontrib>Vitale, Joseph M</creatorcontrib><creatorcontrib>Zhao, Qingshi</creatorcontrib><creatorcontrib>Schneider, Joel S</creatorcontrib><creatorcontrib>Chang, Corey</creatorcontrib><creatorcontrib>Altaf, Aneela</creatorcontrib><creatorcontrib>Michaels, Jennifer</creatorcontrib><creatorcontrib>Bhaumik, Mantu</creatorcontrib><creatorcontrib>Grange, Robert</creatorcontrib><creatorcontrib>Fraidenraich, Diego</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Gale In Context: Opposing Viewpoints</collection><collection>Gale In Context: Science</collection><collection>ProQuest Central (Corporate)</collection><collection>Animal Behavior Abstracts</collection><collection>Bacteriology Abstracts (Microbiology B)</collection><collection>Biotechnology Research Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Ecology Abstracts</collection><collection>Entomology Abstracts (Full archive)</collection><collection>Immunology Abstracts</collection><collection>Meteorological & Geoastrophysical Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Agricultural Science Collection</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>Technology Research Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Technology Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>Materials Science & Engineering Collection</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest One Sustainability</collection><collection>ProQuest Central UK/Ireland</collection><collection>Advanced Technologies & Aerospace Collection</collection><collection>Agricultural & Environmental Science Collection</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Technology Collection</collection><collection>Natural Science Collection</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>ProQuest Materials Science Collection</collection><collection>ProQuest Central Korea</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Materials Science Database</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Meteorological & Geoastrophysical Abstracts - 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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.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>21603573</pmid><doi>10.1371/journal.pone.0020065</doi><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1932-6203 |
| ispartof | PloS one, 2011-05, Vol.6 (5), p.e20065-e20065 |
| issn | 1932-6203 1932-6203 |
| language | eng |
| recordid | cdi_plos_journals_1299019267 |
| source | MEDLINE; DOAJ Directory of Open Access Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Public Library of Science (PLoS); PubMed Central; Free Full-Text Journals in Chemistry |
| subjects | Adipocytes Adipose Tissue Animal tissues Animals Artificial chromosomes Biology Blastocyst Blastocysts Body Composition Body Weight Chimeras Dentistry Duchenne's muscular dystrophy Dystrobrevin Dystrophin Dystrophy Genes Histopathology Induced Pluripotent Stem Cells - transplantation Mechanical properties Medical schools Medicine Mice Muscle, Skeletal Muscular dystrophy Muscular Dystrophy, Animal - therapy Musculoskeletal system Mutation Physiology Pluripotency Proteins Rodents Sarcolemma Skeletal muscle Stem cell transplantation Stem Cell Transplantation - methods Stem cells Utrophin Utrophin - administration & dosage Utrophin - pharmacology Weight reduction |
| title | Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-08T02%3A27%3A32IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_plos_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Differential%20requirement%20for%20utrophin%20in%20the%20induced%20pluripotent%20stem%20cell%20correction%20of%20muscle%20versus%20fat%20in%20muscular%20dystrophy%20mice&rft.jtitle=PloS%20one&rft.au=Beck,%20Amanda%20J&rft.date=2011-05-16&rft.volume=6&rft.issue=5&rft.spage=e20065&rft.epage=e20065&rft.pages=e20065-e20065&rft.issn=1932-6203&rft.eissn=1932-6203&rft_id=info:doi/10.1371/journal.pone.0020065&rft_dat=%3Cgale_plos_%3EA476891561%3C/gale_plos_%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1299019267&rft_id=info:pmid/21603573&rft_galeid=A476891561&rft_doaj_id=oai_doaj_org_article_28d3b4b091ad403a8544e8f69fbd187d&rfr_iscdi=true |