2023
Generation of ventralized human thalamic organoids with thalamic reticular nucleus
Kiral F, Cakir B, Tanaka Y, Kim J, Yang W, Wehbe F, Kang Y, Zhong M, Sancer G, Lee S, Xiang Y, Park I. Generation of ventralized human thalamic organoids with thalamic reticular nucleus. Cell Stem Cell 2023, 30: 677-688.e5. PMID: 37019105, PMCID: PMC10329908, DOI: 10.1016/j.stem.2023.03.007.Peer-Reviewed Original ResearchConceptsHuman embryonic stem cellsSingle-cell RNA sequencingReceptor tyrosine protein kinaseTyrosine protein kinaseEmbryonic stem cellsDisease-associated genesLineage developmentRNA sequencingHuman brain developmentOrganoid systemsStem cellsHuman brain organoidsNeuronal functionBrain organoidsOrganoidsBrain organoid systemsDistinct nucleiBrain developmentThalamic developmentPTCHD1NucleusKinaseGenesSequencingMutations in the transcriptional regulator MeCP2 severely impact key cellular and molecular signatures of human astrocytes during maturation
Sun J, Osenberg S, Irwin A, Ma L, Lee N, Xiang Y, Li F, Wan Y, Park I, Maletic-Savatic M, Ballas N. Mutations in the transcriptional regulator MeCP2 severely impact key cellular and molecular signatures of human astrocytes during maturation. Cell Reports 2023, 42: 111942. PMID: 36640327, PMCID: PMC10857774, DOI: 10.1016/j.celrep.2022.111942.Peer-Reviewed Original ResearchConceptsMECP2 mutationsTranscriptional regulator MeCP2Rett syndromeTranscriptional landscapeTranscriptional changesDysfunctional mitochondriaHuman astrocytesAstrocyte gene expressionGene expressionMECP2 geneMolecular signaturesMutationsPost-natal maturationMaturationDevelopmental maturationBrain bioenergeticsMolecular featuresFunctional maturationStellate morphologyMature morphologyMetabolic aberrationsHuman-based modelsAstrocytesKey roleNeurodevelopmental disorders
2021
Regional specification and complementation with non-neuroectodermal cells in human brain organoids
Tanaka Y, Park IH. Regional specification and complementation with non-neuroectodermal cells in human brain organoids. Journal Of Molecular Medicine 2021, 99: 489-500. PMID: 33651139, PMCID: PMC8026433, DOI: 10.1007/s00109-021-02051-9.Peer-Reviewed Original ResearchAnimalsBrainCellular Reprogramming TechniquesEmbryoid BodiesEndothelial CellsFetusFibroblast Growth Factor 2Hedgehog ProteinsHuman Umbilical Vein Endothelial CellsHumansInduced Pluripotent Stem CellsIntercellular Signaling Peptides and ProteinsMicrogliaNeuronsOrgan SpecificityOrganogenesisOrganoidsRetinaSpinal CordWnt Signaling Pathway
2020
Deconstructing and reconstructing the human brain with regionally specified brain organoids
Xiang Y, Cakir B, Park IH. Deconstructing and reconstructing the human brain with regionally specified brain organoids. Seminars In Cell And Developmental Biology 2020, 111: 40-51. PMID: 32553582, DOI: 10.1016/j.semcdb.2020.05.023.Peer-Reviewed Original Research
2015
Ethanol Upregulates NMDA Receptor Subunit Gene Expression in Human Embryonic Stem Cell-Derived Cortical Neurons
Xiang Y, Kim KY, Gelernter J, Park IH, Zhang H. Ethanol Upregulates NMDA Receptor Subunit Gene Expression in Human Embryonic Stem Cell-Derived Cortical Neurons. PLOS ONE 2015, 10: e0134907. PMID: 26266540, PMCID: PMC4534442, DOI: 10.1371/journal.pone.0134907.Peer-Reviewed Original ResearchConceptsCortical neuronsReceptor subunit gene expressionNeuron-specific biomarkerReverse transcription-quantitative polymerase chain reactionNMDA receptor subunit gene expressionChronic alcohol consumptionHuman brain cellsAlcohol-responsive genesNMDA receptor genesCalcium channel activityLive human brainQuantitative polymerase chain reactionSubunit gene expressionWithdrawal treatmentPolymerase chain reactionExpression changesEthanol exposureAlcohol abuseMultiple comparison correctionBrain cellsGene expression alterationsAlcohol consumptionNeuronal functionAlcohol metabolismNeurons
2013
Trivalent Chromatin Marks the Way iN
Hysolli E, Park IH. Trivalent Chromatin Marks the Way iN. Cell Stem Cell 2013, 13: 510-512. PMID: 24209756, PMCID: PMC4665996, DOI: 10.1016/j.stem.2013.10.007.Peer-Reviewed Original ResearchTherapeutic Potential of Human Induced Pluripotent Stem Cells in Experimental Stroke
Chang DJ, Lee N, Park IH, Choi C, Jeon I, Kwon J, Oh SH, Shin DA, Tae J, Lee DR, Lee H, Hong K, Daley G, Song J, Moon H. Therapeutic Potential of Human Induced Pluripotent Stem Cells in Experimental Stroke. Cell Transplantation 2013, 22: 1427-1440. PMID: 23044029, DOI: 10.3727/096368912x657314.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsApoptosisBehavior, AnimalCell DifferentiationCell LineCell TrackingDisease Models, AnimalGliosisHumansInduced Pluripotent Stem CellsInfarction, Middle Cerebral ArteryInflammationMagnetic Resonance ImagingMaleMiceNeural Stem CellsNeurogenesisNeuronsRatsRats, Sprague-DawleyStem Cell TransplantationStrokeConceptsMiddle cerebral artery occlusionNeural precursor cellsNeural stem cellsStroke-induced inflammatory responseTherapeutic potentialMCAO stroke modelCerebral artery occlusionPeri-infarct areaTreatment of strokeLimited therapeutic optionsStem cellsAutologous cell therapyEndogenous neurogenesisExperimental strokePluripotent stem cellsArtery occlusionIschemic strokeBehavioral recoveryTherapeutic optionsNeurological functionInflammatory responseRobust therapeutic potentialStroke modelMRI resultsAnimal modelsMeCP2 Regulates the Synaptic Expression of a Dysbindin-BLOC-1 Network Component in Mouse Brain and Human Induced Pluripotent Stem Cell-Derived Neurons
Larimore J, Ryder PV, Kim KY, Ambrose LA, Chapleau C, Calfa G, Gross C, Bassell GJ, Pozzo-Miller L, Smith Y, Talbot K, Park IH, Faundez V. MeCP2 Regulates the Synaptic Expression of a Dysbindin-BLOC-1 Network Component in Mouse Brain and Human Induced Pluripotent Stem Cell-Derived Neurons. PLOS ONE 2013, 8: e65069. PMID: 23750231, PMCID: PMC3672180, DOI: 10.1371/journal.pone.0065069.Peer-Reviewed Original ResearchConceptsMutant miceRett syndrome patientsBDNF contentDeficient miceSyndrome patientsInduced pluripotent stem cell-derived neuronsHuman Induced Pluripotent Stem Cell-Derived NeuronsPluripotent stem cell-derived neuronsStem cell-derived neuronsAutism spectrum disorderNormal human hippocampusCell-derived neuronsHuman inducible pluripotent stem cellsAsymmetric synapsesQuantitative real-time PCRHippocampal samplesReal-time PCRMouse hippocampusHuman neuronsPathogenic mechanismsQuantitative qRT-PCRQuantitative immunohistochemistryExpression of componentsSynaptic expressionInducible pluripotent stem cells
2012
Quantitative proteomic analysis of induced pluripotent stem cells derived from a human Huntington's disease patient
Chae JI, Kim DW, Lee N, Jeon YJ, Jeon I, Kwon J, Kim J, Soh Y, Lee DS, Seo KS, Choi NJ, Park BC, Kang SH, Ryu J, Oh SH, Shin DA, Lee DR, Tae J, Park IH, Daley GQ, Song J. Quantitative proteomic analysis of induced pluripotent stem cells derived from a human Huntington's disease patient. Biochemical Journal 2012, 446: 359-371. PMID: 22694310, DOI: 10.1042/bj20111495.Peer-Reviewed Original ResearchConceptsHD-iPSCsProteomic analysisCellular disease-modelling systemsHD-iPSCComparative proteomic analysisQuantitative proteomic analysisStress-related proteinsDifferent biological processesP53-mediated apoptotic pathwayInduced pluripotent stem cellsOxidative stress-related proteinsExpression of cytoskeletonPluripotent stem cellsDisease model systemsOxidative stressPrx familyProteomic profilesUndifferentiated stageBiological processesApoptotic pathwayNeuronal differentiationCell deathNeurodegenerative genetic disorderNeurodegeneration mechanismsProteinNeuronal Properties, In Vivo Effects, and Pathology of a Huntington's Disease Patient‐Derived Induced Pluripotent Stem Cells
Jeon I, Lee N, Li J, Park I, Park KS, Moon J, Shim SH, Choi C, Chang D, Kwon J, Oh S, Shin DA, Kim HS, Tae J, Lee DR, Kim M, Kang K, Daley GQ, Brundin P, Song J. Neuronal Properties, In Vivo Effects, and Pathology of a Huntington's Disease Patient‐Derived Induced Pluripotent Stem Cells. Stem Cells 2012, 30: 2054-2062. PMID: 22628015, DOI: 10.1002/stem.1135.Peer-Reviewed Original ResearchConceptsHD-iPSCHD pathologyHuntington's diseaseDisease patientsNeuronal propertiesUnilateral excitotoxic striatal lesionExcitotoxic striatal lesionsSignificant behavioral recoveryStem cellsGABAergic striatal neuronsHuntington's disease patientsCAG repeatsNeuronal cell typesPluripotent stem cellsBehavioral recoveryGABAergic neuronsStriatal lesionsStriatal neuronsRat modelNeonatal brainNovel cell therapiesVivo effectsHD phenotypeCell therapyNovel therapeutics
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 maturationInduced pluripotent stem cell models from X‐linked adrenoleukodystrophy patients
Jang J, Kang H, Kim H, Kim JY, Huh YJ, Kim D, Yoo J, Lee J, Lim B, Lee J, Yoon T, Park I, Hwang D, Daley GQ, Kim D. Induced pluripotent stem cell models from X‐linked adrenoleukodystrophy patients. Annals Of Neurology 2011, 70: 402-409. PMID: 21721033, DOI: 10.1002/ana.22486.Peer-Reviewed Original ResearchMeSH KeywordsAdrenoleukodystrophyATP Binding Cassette Transporter, Subfamily DATP-Binding Cassette TransportersBrainCell DifferentiationDNAExcitatory Postsynaptic PotentialsFatty Acids, NonesterifiedHematopoietic Stem Cell TransplantationHumansHydroxymethylglutaryl-CoA Reductase InhibitorsInduced Pluripotent Stem CellsLovastatinNeuronsOligodendrogliaPhenotypePhenylbutyratesReverse Transcriptase Polymerase Chain ReactionConceptsChildhood cerebral ALDX-ALDVLCFA accumulationLong chain fatty acid levelsAppropriate animal model systemsSevere clinical manifestationsFatty acid levelsAnimal model systemsDisease-relevant phenotypesClinical manifestationsCerebral ALDABCD1 mutationsAdrenoleukodystrophy patientsDevelopment of therapeuticsHuman oligodendrocytesUnique cellular modelDisease subtypesAbnormal accumulationAccurate diagnosisPluripotent stem cell modelsOligodendrocytesNew therapeuticsAcid levelsOligodendrocyte differentiationStem cell model
2010
Robust Enhancement of Neural Differentiation from Human ES and iPS Cells Regardless of their Innate Difference in Differentiation Propensity
Kim DS, Lee JS, Leem JW, Huh YJ, Kim JY, Kim HS, Park IH, Daley GQ, Hwang DY, Kim DW. Robust Enhancement of Neural Differentiation from Human ES and iPS Cells Regardless of their Innate Difference in Differentiation Propensity. Stem Cell Reviews And Reports 2010, 6: 270-281. PMID: 20376579, DOI: 10.1007/s12015-010-9138-1.Peer-Reviewed Original ResearchConceptsDifferentiation propensityStem cell linesCell lineagesNeural differentiationHuman embryonic stem cell linesEmbryonic stem cell linesCell typesActivin/NodalHuman pluripotent stem cell linesDesirable cell typesSpecific cell typesCell linesModulation of intracellularPluripotent stem cellsPluripotent stem cell lineBMP pathwaySpecific lineagesIPS cellsUndifferentiated cellsLineagesDifferentiation potentialHuman ESStem cellsNeural cellsDifferentiation