2012
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 rateMutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability
Bilican B, Serio A, Barmada SJ, Nishimura AL, Sullivan GJ, Carrasco M, Phatnani HP, Puddifoot CA, Story D, Fletcher J, Park IH, Friedman BA, Daley GQ, Wyllie DJ, Hardingham GE, Wilmut I, Finkbeiner S, Maniatis T, Shaw CE, Chandran S. Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability. Proceedings Of The National Academy Of Sciences Of The United States Of America 2012, 109: 5803-5808. PMID: 22451909, PMCID: PMC3326463, DOI: 10.1073/pnas.1202922109.Peer-Reviewed Original ResearchConceptsAmyotrophic lateral sclerosisTDP-43 proteinopathyTDP-43Human neuronsTransactive response DNA binding proteinFrontotemporal lobar degenerationFamilial amyotrophic lateral sclerosisFunctional motor neuronsCell-specific vulnerabilityTDP-43 proteinDNA binding proteinPI3K pathwayCell-autonomous phenotypeMotor neuronsLateral sclerosisStem cell linesPluripotent stem cellsGeneration of iPSCsIdentification of mutationsPluripotent stem cell lineMutant neuronsNeuronsK pathwayElevated levelsDisease mechanisms
2011
Neuronal maturation defect in induced pluripotent stem cells from patients with Rett syndrome
Kim KY, Hysolli E, Park IH. Neuronal maturation defect in induced pluripotent stem cells from patients with Rett syndrome. Proceedings Of The National Academy Of Sciences Of The United States Of America 2011, 108: 14169-14174. PMID: 21807996, PMCID: PMC3161557, DOI: 10.1073/pnas.1018979108.Peer-Reviewed Original ResearchMeSH KeywordsAdultAmino Acid SequenceBase SequenceBiomarkersCell DifferentiationChildChild, PreschoolChromosomes, Human, XEmbryonic Stem CellsFemaleFibroblastsGene Expression RegulationHumansInduced Pluripotent Stem CellsKruppel-Like Factor 4Methyl-CpG-Binding Protein 2Molecular Sequence DataNeuronsRett SyndromeX Chromosome InactivationConceptsX chromosomePluripotent stem cellsSingle active X chromosomeRett syndromeActive X chromosomePathophysiology of RTTX-chromosome inactivationStem cellsInduced pluripotent stem cellsRTT fibroblastsMurine genetic modelsMolecular dissectionChromosome inactivationFactors OCT4Methyl-CpGRTT phenotypeNeuronal differentiationChromosomesPurposeful hand movementsNormal developmentRTT modelModel of RTTProtein 2Maturation defectsNeuronal maturation
2009
Cardiomyocyte Differentiation of Human Induced Pluripotent Stem Cells
Zwi L, Caspi O, Arbel G, Huber I, Gepstein A, Park IH, Gepstein L. Cardiomyocyte Differentiation of Human Induced Pluripotent Stem Cells. Circulation 2009, 120: 1513-1523. PMID: 19786631, DOI: 10.1161/circulationaha.109.868885.Peer-Reviewed Original ResearchConceptsTranscription factorsHiPS cellsCardiomyocyte differentiationCardiac-specific transcription factorsInduced pluripotent stem cellsGene expression studiesHuman induced pluripotent stem cellsStem cell linesPluripotent stem cellsCardiomyocyte differentiation potentialPluripotent stem cell lineStructural geneContracting embryoid bodiesCardiomyocyte differentiation processTranslational cardiovascular researchEmbryoid bodiesExpression studiesDifferentiation systemDifferentiation processCardiovascular regenerative medicineSarcomeric proteinsDifferentiation potentialIon channelsAdult fibroblastsFunctional propertiesGeneration of induced pluripotent stem cells from human blood
Loh YH, Agarwal S, Park IH, Urbach A, Huo H, Heffner GC, Kim K, Miller JD, Ng K, Daley GQ. Generation of induced pluripotent stem cells from human blood. Blood 2009, 113: 5476-5479. PMID: 19299331, PMCID: PMC2689048, DOI: 10.1182/blood-2009-02-204800.Peer-Reviewed Original ResearchConceptsPluripotent stem cellsPluripotent cell-specific genesPatient-specific stem cellsTranscription factorsStem cellsHuman embryonic stem cellsPluripotency-associated transcription factorsEmbryonic stem cellsCell-specific genesInduced pluripotent stem cellsDNA methylation statusEctopic expressionHematopoietic lineagesRetroviral transductionMethylation statusSomatic mutationsHuman dermal fibroblastsHuman bloodDermal fibroblastsCellsHuman peripheral blood cellsExpressionPluripotencyPeripheral blood cellsLineages
2007
Reprogramming of human somatic cells to pluripotency with defined factors
Park IH, Zhao R, West JA, Yabuuchi A, Huo H, Ince TA, Lerou PH, Lensch MW, Daley GQ. Reprogramming of human somatic cells to pluripotency with defined factors. Nature 2007, 451: 141-146. PMID: 18157115, DOI: 10.1038/nature06534.Peer-Reviewed Original ResearchMeSH KeywordsAdultAnimalsCell DifferentiationCell ShapeCells, CulturedDNA MethylationDNA-Binding ProteinsEmbryonic Stem CellsFetusFibroblastsGene Expression ProfilingHMGB ProteinsHomeodomain ProteinsHumansInfant, NewbornKruppel-Like Factor 4Kruppel-Like Transcription FactorsMiceNanog Homeobox ProteinOctamer Transcription Factor-3Pluripotent Stem CellsPromoter Regions, GeneticProto-Oncogene Proteins c-mycSOXB1 Transcription FactorsTeratomaTranscription FactorsTransplantation, HeterologousConceptsEmbryonic stem cellsStem cellsIPS cellsHuman somatic cellsInduced pluripotent stem cellsHuman iPS cellsPluripotent stem cellsHuman primary cellsPatient-specific cellsEarly embryosTranscription factorsSomatic cellsEctopic expressionPluripotencyGene expressionHuman cellsMurine fibroblastsDefined factorsPrimary cellsCell linesDermal fibroblastsCellsInvaluable toolFibroblastsExpression