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Article: A human iPSC model of Hutchinson Gilford Progeria reveals vascular smooth muscle and mesenchymal stem cell defects

TitleA human iPSC model of Hutchinson Gilford Progeria reveals vascular smooth muscle and mesenchymal stem cell defects
Authors
Issue Date2011
PublisherCell Press. The Journal's web site is located at http://www.cellstemcell.com
Citation
Cell Stem Cell, 2011, v. 8 n. 1, p. 31-45 How to Cite?
AbstractThe segmental premature aging disease Hutchinson-Gilford Progeria syndrome (HGPS) is caused by a truncated and farnesylated form of Lamin A called progerin. HGPS affects mesenchymal lineages, including the skeletal system, dermis, and vascular smooth muscle (VSMC). To understand the underlying molecular pathology of HGPS, we derived induced pluripotent stem cells (iPSCs) from HGPS dermal fibroblasts. The iPSCs were differentiated into neural progenitors, endothelial cells, fibroblasts, VSMCs, and mesenchymal stem cells (MSCs). Progerin levels were highest in MSCs, VSMCs, and fibroblasts, in that order, with these lineages displaying increased DNA damage, nuclear abnormalities, and HGPS-VSMC accumulating numerous calponin-staining inclusion bodies. Both HGPS-MSC and -VSMC viability was compromised by stress and hypoxia in vitro and in vivo (MSC). Because MSCs reside in low oxygen niches in vivo, we propose that, in HGPS, this causes additional depletion of the MSC pool responsible for replacing differentiated cells lost to progerin toxicity.
Persistent Identifierhttp://hdl.handle.net/10722/163353
ISSN
2015 Impact Factor: 22.387
2015 SCImago Journal Rankings: 13.121
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorZhang, Jen_US
dc.contributor.authorLian, Qen_US
dc.contributor.authorZhu, Gen_US
dc.contributor.authorZhou, Fen_US
dc.contributor.authorSui, Len_US
dc.contributor.authorTan, Cen_US
dc.contributor.authorMutalif, RAen_US
dc.contributor.authorNavasankari, Ren_US
dc.contributor.authorZhang, Yen_US
dc.contributor.authorTse, HFen_US
dc.contributor.authorStewart, CLen_US
dc.contributor.authorColman, Aen_US
dc.date.accessioned2012-09-05T05:30:27Z-
dc.date.available2012-09-05T05:30:27Z-
dc.date.issued2011en_US
dc.identifier.citationCell Stem Cell, 2011, v. 8 n. 1, p. 31-45en_US
dc.identifier.issn1934-5909en_US
dc.identifier.urihttp://hdl.handle.net/10722/163353-
dc.description.abstractThe segmental premature aging disease Hutchinson-Gilford Progeria syndrome (HGPS) is caused by a truncated and farnesylated form of Lamin A called progerin. HGPS affects mesenchymal lineages, including the skeletal system, dermis, and vascular smooth muscle (VSMC). To understand the underlying molecular pathology of HGPS, we derived induced pluripotent stem cells (iPSCs) from HGPS dermal fibroblasts. The iPSCs were differentiated into neural progenitors, endothelial cells, fibroblasts, VSMCs, and mesenchymal stem cells (MSCs). Progerin levels were highest in MSCs, VSMCs, and fibroblasts, in that order, with these lineages displaying increased DNA damage, nuclear abnormalities, and HGPS-VSMC accumulating numerous calponin-staining inclusion bodies. Both HGPS-MSC and -VSMC viability was compromised by stress and hypoxia in vitro and in vivo (MSC). Because MSCs reside in low oxygen niches in vivo, we propose that, in HGPS, this causes additional depletion of the MSC pool responsible for replacing differentiated cells lost to progerin toxicity.en_US
dc.languageengen_US
dc.publisherCell Press. The Journal's web site is located at http://www.cellstemcell.comen_US
dc.relation.ispartofCell Stem Cellen_US
dc.subject.meshAging, Premature - metabolismen_US
dc.subject.meshFibroblasts - pathologyen_US
dc.subject.meshInduced Pluripotent Stem Cells - cytology - metabolismen_US
dc.subject.meshMesenchymal Stem Cells - cytology - pathologyen_US
dc.subject.meshMuscle, Smooth, Vascular - cytology - pathologyen_US
dc.titleA human iPSC model of Hutchinson Gilford Progeria reveals vascular smooth muscle and mesenchymal stem cell defectsen_US
dc.typeArticleen_US
dc.identifier.emailLian, Q: qzlian@hkucc.hku.hken_US
dc.identifier.emailTse, HF: hftse@hkucc.hku.hken_US
dc.identifier.emailStewart, CL: colin.stewart@imb.a-star.edu.sg-
dc.identifier.emailColman, A: alan.colman@imb.a-star.edu.sg-
dc.identifier.authorityLian, Q=rp00267en_US
dc.identifier.authorityTse, HF=rp00428en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1016/j.stem.2010.12.002en_US
dc.identifier.pmid21185252-
dc.identifier.scopuseid_2-s2.0-78650995671en_US
dc.identifier.hkuros204428-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-78650995671&selection=ref&src=s&origin=recordpageen_US
dc.identifier.volume8en_US
dc.identifier.issue1en_US
dc.identifier.spage31en_US
dc.identifier.epage45en_US
dc.identifier.eissn1875-9777-
dc.identifier.isiWOS:000286786200009-
dc.publisher.placeUnited Statesen_US
dc.identifier.f10007761957-
dc.identifier.scopusauthoridColman, A=35406098300en_US
dc.identifier.scopusauthoridStewart, CL=7401937591en_US
dc.identifier.scopusauthoridTse, HF=7006070805en_US
dc.identifier.scopusauthoridZhang, Y=35785466900en_US
dc.identifier.scopusauthoridNavasankari, R=36656200500en_US
dc.identifier.scopusauthoridMutalif, RA=36470730500en_US
dc.identifier.scopusauthoridTan, C=41862423400en_US
dc.identifier.scopusauthoridSui, L=41862397200en_US
dc.identifier.scopusauthoridZhou, F=41862956500en_US
dc.identifier.scopusauthoridZhu, G=41862922800en_US
dc.identifier.scopusauthoridLian, Q=7003399023en_US
dc.identifier.scopusauthoridZhang, J=7601360461en_US
dc.identifier.citeulike9386858-

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