Search results for " STEM CELLS"

showing 10 items of 881 documents

Homozygous variants in the gene SCAPER cause syndromic intellectual disability

2019

The S-Phase Cyclin A Associated Protein In The ER (SCAPER) gene is a ubiquitously expressed gene with unknown function in the brain. Recently, biallelic SCAPER variants were described in four patients from three families with retinitis pigmentosa (RP) and intellectual disability (ID). Here, we expand the spectrum of pathogenic variants in SCAPER and report on 10 further patients from four families with ID, RP, and additional dysmorphic features carrying homozygous variants in SCAPER. The variants found comprise frameshift, nonsense, and missense variants as well as an intragenic homozygous deletion, which spans SCAPER exons 15 and 16 and introduces a frameshift and a premature stop codon. A…

AdultMale0301 basic medicineAdolescentmedia_common.quotation_subjectCyclin ANonsenseGene Expression030105 genetics & heredityFrameshift mutationConsanguinityMice03 medical and health sciencesExonNeural Stem CellsIntellectual DisabilityRetinitis pigmentosaGene expressionGeneticsmedicineAnimalsHumansMissense mutationFamilyChildGeneGenetics (clinical)media_commonCerebral CortexNeuronsGeneticsbiologyHomozygoteSyndromemedicine.diseasePedigree030104 developmental biologyMutationbiology.proteinFemaleCarrier ProteinsRetinitis PigmentosaAmerican Journal of Medical Genetics Part A
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Adipose tissue-derived mesenchymal stromal cells as part of therapy for chronic graft-versus-host disease: A phase I/II study

2017

Abstract Background aims Despite the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT), the procedure is still associated with high toxicity in patients with refractory graft-versus-host disease (GvHD). Mesenchymal stromal cells (MSCs) are a new mode of therapy in the context of allo-HSCT. The objective of this study was to evaluate the safety and feasibility of the use of adipose tissue–derived MSCs (AT-MSCs) in patients with chronic GvHD. Methods Fourteen patients with moderate (n = 7) or severe (n = 7) chronic GvHD received 1 × 106/kg (group A, n = 9) or 3 × 106/kg (group B, n = 5) AT-MSCs with cyclosporine and prednisone as first-line therapy. Results Ten of the…

AdultMale0301 basic medicineCancer Researchmedicine.medical_specialtymedicine.medical_treatmentImmunologyGraft vs Host DiseaseAdipose tissueContext (language use)DiseaseHematopoietic stem cell transplantationMesenchymal Stem Cell TransplantationGastroenterology03 medical and health sciencesPrednisoneInternal medicinemedicineHumansImmunology and AllergyGenetics (clinical)TransplantationTumor Necrosis Factor-alphabusiness.industryMesenchymal stem cellMesenchymal Stem CellsCell BiologyMiddle Agedmedicine.diseaseKiller Cells NaturalTreatment Outcome030104 developmental biologyGraft-versus-host diseaseAdipose TissueOncologyToxicityImmunologyCyclosporinePrednisoneFemalebusinessImmunosuppressive Agentsmedicine.drugCytotherapy
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Comparison of diffusion, cytotoxicity and tissue inflammatory reactions of four commercial bleaching products against human dental pulp stem cells

2018

AbstractMultiple side effects related to bleaching were found to occur in the dental pulp tissue, including decreased cell metabolism and viability. In this work we evaluated the in vitro diffusion capacity, cytotoxicity and biocompatibility of four commercial bleaching products on stem cells from human dental pulp (hDPSCs). Two commercial bleaching gels hydrogen peroxide-based (HP), Norblanc Office 37.5% (Nor-HP) and Opalescence Boost 40% (Opal-HP) were applied for 30 min to enamel/dentine discs. Another two gels from the same manufacturers, 16% carbamide peroxide-based (CP), Norblanc Home (Nor-CP) and Opalescence CP 16% (Opal-CP), were applied for 90 min. The diffusion of HP was analysed …

AdultMale0301 basic medicineNecrosisBiocompatibilityScienceCarbamide PeroxideArticleDiffusionTooth whiteningYoung Adult03 medical and health scienceschemistry.chemical_compoundDental biomaterials0302 clinical medicineAnti-Infective Agentsstomatognathic systemDental pulp stem cellsTooth BleachingmedicineAnimalsHumansRats WistarTooth Bleaching AgentsHydrogen peroxideCytotoxicityDental PulpInflammationMultidisciplinaryEnamel paintStem CellsQRHydrogen PeroxideMolecular biologyPeroxidesRatsstomatognathic diseases030104 developmental biologychemistryvisual_artToxicityMicroscopy Electron Scanningvisual_art.visual_art_mediumMedicinePulp (tooth)Femalemedicine.symptom030217 neurology & neurosurgeryScientific Reports
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Role of p16INK4a and BMI-1 in oxidative stress-induced premature senescence in human dental pulp stem cells

2017

Human dental pulp stem cells (hDPSCs) are a source for cell therapy. Before implantation, an in vitro expansion step is necessary, with the inconvenience that hDPSCs undergo senescence following a certain number of passages, loosing their stemness properties. Long-term in vitro culture of hDPSCs at 21% (ambient oxygen tension) compared with 3–6% oxygen tension (physiological oxygen tension) caused an oxidative stress-related premature senescence, as evidenced by increased β-galactosidase activity and increased lysil oxidase expression, which is mediated by p16INK4a pathway. Furthermore, hDPSCs cultured at 21% oxygen tension underwent a downregulation of OCT4, SOX2, KLF4 and c-MYC factors, w…

AdultMale0301 basic medicineSenescenceAginghDPSCs human dental pulp stem cellsMSC mesenchymal stem cellsAdolescentCellular differentiationClinical BiochemistryCell Culture TechniquesOSKM OCT4 SOX2 KLF4 and c-MYCBiologymedicine.disease_causeBiochemistryCell therapyKruppel-Like Factor 4Young Adult03 medical and health sciencesDental pulp stem cellsmedicineHumansOxygen tensionlcsh:QH301-705.5SIPS stress-induced premature senescenceCells CulturedCellular SenescenceCyclin-Dependent Kinase Inhibitor p16Dental PulpMDA malondialdehydePolycomb Repressive Complex 1lcsh:R5-920Stem CellsOrganic ChemistryCell DifferentiationOxygen tensionCell biologyOxygenOxidative Stress030104 developmental biologylcsh:Biology (General)Cell cultureRegenerative medicineImmunologyFemaleStem celllcsh:Medicine (General)Oxidative stressResearch PaperRedox Biology
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Direct pericyte-to-neuron reprogramming via unfolding of a neural stem cell-like program

2018

Ectopic expression of defined transcription factors can force direct cell-fate conversion from one lineage to another in the absence of cell division. Several transcription factor cocktails have enabled successful reprogramming of various somatic cell types into induced neurons (iNs) of distinct neurotransmitter phenotype. However, the nature of the intermediate states that drive the reprogramming trajectory toward distinct iN types is largely unknown. Here we show that successful direct reprogramming of adult human brain pericytes into functional iNs by Ascl1 and Sox2 encompasses transient activation of a neural stem cell-like gene expression program that precedes bifurcation into distinct…

AdultMale0301 basic medicineSomatic cellCellular differentiationBasic Helix-Loop-Helix Transcription FactorSOXB1 Transcription FactorBiologyArticleYoung Adult03 medical and health sciences0302 clinical medicineNeural Stem CellsSOX2Basic Helix-Loop-Helix Transcription FactorsHumansCell LineageNeural Stem CellAgedPericyteNeuronsSOXB1 Transcription FactorsGeneral NeuroscienceCell DifferentiationMiddle AgedNeuronCellular ReprogrammingNeural stem cellASCL1030104 developmental biologyGene Expression RegulationFemaleEctopic expressionPericytesNeural developmentReprogrammingNeuroscience030217 neurology & neurosurgeryHuman
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Hematopoietic peripheral circulating blood stem cells as an independent marker of good transfusion management in patients with β-thalassemia: results…

2015

Beyond hemoglobin (Hb) levels and performance status, further surrogate markers of appropriate transfusion management should improve the quality of thalassemia care. We investigated the levels of peripheral circulating CD34+ stem cells as an independent marker of appropriate hematopoietic balance in patients with thalassemia.Peripheral circulating CD34+ stem cells, colony-forming unitgranulocyte, erythrocyte, macrophage, magakaryocyte (CF-GEMM), colony-forming unitgranulocyte/macrophage (CFU-GM), and erythroidburst-forming units (BFU-E) were assayed, according to standard procedures. Patients with thalassemia major (TM) and thalassemia intermedia (TI) were tested and compared to healthy con…

AdultMaleAdolescentbeta-ThalassemiaHematopoietic Stem Cell TransplantationPilot ProjectsMiddle AgedHematopoietic Stem CellsPrognosisSettore MED/15 - Malattie Del SangueYoung AdultPeripheral CD34+ stem cells beta-thalassemiaTreatment OutcomeCase-Control StudiesHumansFemaleBiomarkersAgedFollow-Up Studies
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Temporal dynamics of hippocampal neurogenesis in chronic neurodegeneration.

2014

Increased neurogenesis has been reported in neurodegenerative disease, but its significance is unclear. In a mouse model of prion disease, Gomez-Nicola et al. detect increased neurogenesis in the dentate gyrus that partially counteracts neuronal loss. Targeting neurogenesis may have therapeutic potential.

AdultMaleAntimetabolites AntineoplasticPatch-Clamp TechniquesTime FactorsPrionsNeurogenesisGenetic VectorsHippocampusTissue BanksBiologyHippocampal formationHippocampusCreutzfeldt-Jakob SyndromePrion DiseasesMiceYoung AdultNeural Stem CellsAlzheimer Diseasevariant CJDNeural PathwaysmedicineAnimalsHumansAgedCell ProliferationDentate gyrusNeurogenesisNeurodegenerationCytarabineNeurodegenerative DiseasesOriginal ArticlesMiddle Agedmedicine.diseaseNeural stem cellMice Inbred C57BLNeuroanatomical Tract-Tracing Techniquesadult neurogenesisDisease Models AnimalChronic DiseaseDentate GyrusMossy Fibers HippocampalDisease ProgressionFemaleNeurology (clinical)Alzheimer's diseaseNeuroscienceNeural developmentAlzheimer’s diseaseBrain : a journal of neurology
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CONSISTENT BONE MARROW-DERIVED CELL MOBILIZATION FOLLOWING REPEATED SHORT COURSES OF GRANULOCYTE-COLONY-STIMULATING FACTOR IN PATIENTS WITH AMYOTROPH…

2009

Background and aims. The aim of this study was to evaluate and characterize the feasibility and safety of bone marrow-derived cell (BMC) mobilization following repeated courses of granulocyte-colony stimulating factor (G-CSF) in patients with amyotrophic lateral sclerosis (ALS). Methods. Between January 2006 and March 2007, 26 ALS patients entered a multicenter trial that included four courses of BMC mobilization at 3-month intervals. In each course, G-CSF (5 mu g/kg b.i.d.) was administered for four consecutive days; 18% mannitol was also given. Mobilization was monitored by flow cytometry analysis of circulating CD34(+) cells and by in vitro colony assay for clonogenic progenitors. Co-exp…

AdultMaleCancer Researchmedicine.medical_specialtySLa - trial clinico - C-GSFImmunologyAntigens CD34Bone Marrow CellsDrug Administration ScheduleColony-Forming Units AssayCell MovementInternal medicineMulticenter trialmedicineImmunology and AllergyHumansCell LineageProspective StudiesAmyotrophic lateral sclerosisProspective cohort studyGenetics (clinical)Hematopoietic Stem Cell MobilizationNeuronsTransplantationMobilizationbusiness.industryStem CellsAmyotrophic Lateral SclerosisGranulocyte-Macrophage Colony-Stimulating FactorCell DifferentiationCell BiologyMiddle Agedmedicine.diseaseHematopoietic Stem CellsBone Marrow-Derived CellHematopoietic Stem Cell MobilizationSurgeryGranulocyte colony-stimulating factorNerve RegenerationSettore MED/26 - NEUROLOGIAGranulocyte macrophage colony-stimulating factorTreatment OutcomeOncologyBiological MarkersFemalebusinessNeurogliaBiomarkersmedicine.drug
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Successful treatment of resistant thrombotic thrombocytopenic purpura/hemolytic uremic syndrome with autologous peripheral blood stem and progenitor …

1999

The first-line treatment of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS syndrome) induces a response and survival rate of approximately 85%, even if a considerable number of patients relapse; nevertheless, a number of these patients are resistant to conventional management. Immunoablation followed by stem cell transplantation has been shown to be capable of inducing remissions in a large spectrum of experimental autoimmune disorders. We report here the case of a 20-year-old male patient with the TTP-HUS syndrome who was resistant to conventional treatment and was transplanted with autologous immunoselected CD34+ PBPC after conditioning with cyclosphosphamide, anti…

AdultMaleHemolytic anemiaLymphocyteThrombotic thrombocytopenic purpuraAntigens CD34Transplantation AutologousPrednisonehemic and lymphatic diseasesHumansMedicineProgenitor cellSurvival rateTransplantationPurpura Thrombotic Thrombocytopenicbusiness.industryHematopoietic Stem Cell TransplantationHematologyHematopoietic Stem Cellsmedicine.diseaseTransplantationmedicine.anatomical_structureHemolytic-Uremic SyndromeImmunologyStem cellbusinessmedicine.drugBone Marrow Transplantation
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Effect of Age and Lipoperoxidation in Rat and Human Adipose Tissue-Derived Stem Cells

2020

A wide range of clinical applications in regenerative medicine were opened decades ago with the discovery of adult stem cells. Highly promising adult stem cells are mesenchymal stem/stromal cells derived from adipose tissue (ADSCs), primarily because of their abundance and accessibility. These cells have multipotent properties and have been used extensively to carry out autologous transplants. However, the biology of these cells is not entirely understood. Among other factors, the regeneration capacity of these cells will depend on both their capacity of proliferation/differentiation and the robustness of the biochemical pathways that allow them to survive under adverse conditions like thos…

AdultMaleHomeobox protein NANOGAgingTime FactorsStromal cellArticle SubjectApoptosisBiologyRegenerative MedicineBiochemistryRegenerative medicineCell therapyAMP-Activated Protein Kinase KinasesPeptide Elongation Factor 2Sirtuin 1SOX2AnimalsHumansRats WistarLipoperoxidation.Cell ProliferationQH573-671SOXB1 Transcription FactorsStem CellsMesenchymal stem cellAge FactorsCell DifferentiationMesenchymal Stem CellsNanog Homeobox ProteinCell BiologyGeneral MedicineMiddle AgedRatsCell biologyOxidative StressAdipose TissueageFemaleLipid PeroxidationStem cellCytologyProtein KinasesResearch ArticleHeLa CellsAdult stem cell
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