2013
Modelling human disease with pluripotent stem cells.
Siller R, Greenhough S, Park IH, Sullivan GJ. Modelling human disease with pluripotent stem cells. Current Gene Therapy 2013, 13: 99-110. PMID: 23444871, PMCID: PMC3785403, DOI: 10.2174/1566523211313020004.Peer-Reviewed Original ResearchConceptsPluripotent stem cellsStem cellsAffected cell typesCellular reprogrammingEndodermal lineagesPluripotent cellsHuman diseasesCell typesGenetic diseasesDisease phenotypeDisease mechanismsDisease modellingTissue of interestPatient tissuesCellsLimitless supplyReprogrammingLineagesRecent progressProgenyPhenotypeTissueTherapeutic interventionsHigh levelsCell technology
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
2010
Directed differentiation of hematopoietic precursors and functional osteoclasts from human ES and iPS cells
Grigoriadis AE, Kennedy M, Bozec A, Brunton F, Stenbeck G, Park IH, Wagner EF, Keller GM. Directed differentiation of hematopoietic precursors and functional osteoclasts from human ES and iPS cells. Blood 2010, 115: 2769-2776. PMID: 20065292, PMCID: PMC2854424, DOI: 10.1182/blood-2009-07-234690.Peer-Reviewed Original ResearchConceptsPluripotent stem cellsInduced pluripotent stem cellsStem cellsPrimitive streak-like populationHuman pluripotent stem cellsHuman cell typesEmbryonic bone developmentEmbryoid bodiesBone-resorbing osteoclastsMacrophage colony-stimulating factorIPS cellsHematopoietic cytokinesCell typesMolecular analysisCathepsin KHuman ESHematopoietic precursorsPrecursor populationAlphavbeta3 integrinBone developmentConfocal microscopyAbsence of RANKLNuclear factor-kappaB ligandDisease mechanismsSerum-free medium