Modeling Supravalvular Aortic Stenosis Syndrome With Human Induced Pluripotent Stem Cells
Ge X, Ren Y, Bartulos O, Lee MY, Yue Z, Kim KY, Li W, Amos PJ, Bozkulak EC, Iyer A, Zheng W, Zhao H, Martin KA, Kotton DN, Tellides G, Park IH, Yue L, Qyang Y. Modeling Supravalvular Aortic Stenosis Syndrome With Human Induced Pluripotent Stem Cells. Circulation 2012, 126: 1695-1704. PMID: 22914687, PMCID: PMC3586776, DOI: 10.1161/circulationaha.112.116996.Peer-Reviewed Original ResearchConceptsActin filament bundlesSmooth muscle αSmooth muscle cellsExtracellular signal-regulated kinase 1/2Muscle αFilament bundlesSignal-regulated kinase 1/2Four-nucleotide insertionDisease mechanismsContractile smooth muscle cellsStem cell linesPluripotent stem cellsPluripotent stem cell linePlatelet-derived growth factorRhoA signalingVascular smooth muscle cellsRecombinant proteinsKinase 1/2Elastin geneELN geneWilliams-Beuren syndromeBrdU analysisSupravalvular aortic stenosisStem cellsHigh proliferation rate