2020
Implantation of the clinical‐grade human neural stem cell line, CTX0E03, rescues the behavioral and pathological deficits in the quinolinic acid‐lesioned rodent model of Huntington's disease
Yoon Y, Kim HS, Jeon I, Noh J, Park HJ, Lee S, Park I, Stevanato L, Hicks C, Corteling R, Barker RA, Sinden JD, Song J. Implantation of the clinical‐grade human neural stem cell line, CTX0E03, rescues the behavioral and pathological deficits in the quinolinic acid‐lesioned rodent model of Huntington's disease. Stem Cells 2020, 38: 936-947. PMID: 32374064, PMCID: PMC7496241, DOI: 10.1002/stem.3191.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCell Line, TumorDisease Models, AnimalHumansHuntington DiseaseMiceNeoplasm GradingNeural Stem CellsQuinolinic AcidConceptsMedium spiny neuronsNeural stem cell lineHuntington's diseaseQuinolinic acid (QA) lesion rat modelChronic ischemic stroke patientsStriatal medium spiny neuronsCell linesImmortalized neural stem cell linesIschemic stroke patientsDisease-modifying therapiesSignals of efficacyGlial scar formationHost brain tissueHuman neural stem cell lineSignificant behavioral improvementAutosomal dominant neurodegenerative diseaseCTX0E03 cellsEndogenous neurogenesisBDNF expressionGABAergic neuronsHD patientsStroke patientsFluoro-GoldRetrograde labelSpiny neurons
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