grant
TGF-ß-SOX18-Collagen metabolism in pulmonary vascular disease associated with CHD
Organization FLORIDA INTERNATIONAL UNIVERSITYLocation MIAMI, UNITED STATESPosted 10 Aug 2020Deadline 31 Jul 2030
NIHUS FederalResearch GrantFY20250-11 years old0-4 weeks old21+ years oldAdultAdult HumanAnabolismAnimal ModelAnimal Models and Related StudiesApoptoticAttentionAutomobile DrivingBasal Transcription FactorBasal transcription factor genesBindingBiomechanicsBirthBlood VesselsBlood flowBone-Derived Transforming Growth FactorCardiac MalformationCardiac defectCardiopulmonaryCell Communication and SignalingCell IsolationCell SegregationCell SeparationCell Separation TechnologyCell SignalingCellular Metabolic ProcessChildChild YouthChildren (0-21)ChronicCollagenCongenital Cardiac DefectsCongenital Heart DefectsDataDepositDepositionDevelopmentDiseaseDisorderDrugsDysfunctionEndotheliumEnhancersExhibitsFamilyFunctional disorderGene Down-RegulationGene ExpressionGeneral Transcription Factor GeneGeneral Transcription FactorsGenesHeart MalformationIncidenceInfantInfantile HemangiomaInitiation FactorsIntermediary MetabolismIntervention StrategiesIntracellular Communication and SignalingJuvenile HemangiomaL-ProlineLeftLinkLive BirthLungLung Respiratory SystemMalignant CellMeCP-2 proteinMeCP2MeCP2 proteinMediatingMedicalMedicationMetabolicMetabolic ProcessesMetabolismMethyl CpG binding protein MeCP2Methyl-CpG-Binding Protein 2Methyl-DNA binding protein MECP2Milk Growth FactorModelingMolecular InteractionMorbidityMorbidity - disease rateMorphologyMuscle CellsMyocytesNewborn InfantNewbornsOperative ProceduresOperative Surgical ProceduresPPAR gammaPPAR-gPPAR-γPPARgammaPPARγParturitionPathogenesisPathway interactionsPeptide Initiation FactorsPeroxisome Proliferative Activated Receptor GammaPeroxisome Proliferator-Activated Receptor gammaPeroxisome Proliferator-Activated Receptor γPharmaceutical PreparationsPhenotypePhysiologyPhysiopathologyPlatelet Transforming Growth FactorPrecision therapeuticsPreventionProcessProductionProlinePropanololPropranololPulmonary HypertensionRepressionRight Ventricular DysfunctionRight heart dysfunctionRight ventricle dysfunctionRight-sided heart dysfunctionRoleSOX18SOX18 geneSRY-Box 18ShuntShunt DeviceSignal TransductionSignal Transduction SystemsSignalingSiteStrawberry AngiomaStrawberry BirthmarkStrawberry HemangiomaStrawberry MarkStrawberry nevusSurgicalSurgical InterventionsSurgical ProcedureTGF BTGF-betaTGF-βTGFbetaTGFβTestingTherapeuticTherapeutic InterventionThiazolidinedione ReceptorTranscription Factor Proto-OncogeneTranscription RepressionTranscription RepressorTranscription factor genesTranscriptional RepressorTransforming Growth Factor betaTransforming Growth Factor-Beta Family GeneTranslation Initiation FactorTranslational Initiation FactorVascular remodelingWorkabnormal heart developmentadulthoodadvanced diseaseadvanced illnessaerobic glycolysisage associatedage correlatedage dependentage linkedage relatedage specificangiogenesisarteriolebiological signal transductionbiomechanicalbiosynthesiscancer cellcell metabolismcell sortingcellular metabaolismclinical relevanceclinically relevantcongenital cardiac abnormalitycongenital cardiac anomaliescongenital cardiac diseasecongenital cardiac disordercongenital cardiac malformationcongenital heart abnormalitycongenital heart anomalycongenital heart diseasecongenital heart disordercongenital heart malformationdesigndesigningdevelopmentaldrivingdrug/agentfatty acid oxidationfeasibility testinggene repressiongenetic repressorheart defecthemodynamicsimpaired pulmonary vascularizationimprovedin vivoinhibitorinsightintervention therapykidslung vascular diseaselung vascular remodelingmechanical forcemitochondrial metabolismmodel of animalmortalitynew approachesnewborn childnewborn childrennovelnovel approachesnovel strategiesnovel strategypathophysiologypathwayprecision therapiesprecision treatmentpulmonarypulmonary arterial endothelial cellpulmonary arterial stiffeningpulmonary arterial stiffnesspulmonary artery endothelial cellpulmonary artery stiffeningpulmonary artery stiffnesspulmonary vascular diseasepulmonary vascular disorderpulmonary vascular dysfunctionpulmonary vascular remodelingpulmonary vasculopathyresponseshuntssmall molecular inhibitorsmall moleculesmall molecule inhibitorsocial rolesurgerytherapeutic targettranscription factorvascularvascular abnormalityyoungster
Description preview
ABSTRACT
Children born with common congenital heart defects that cause increased pulmonary blood flow (PBF) develop
abnormal pulmonary vascular reactivity and, if untreated, pulmonary hypertension (PH). Vascular morphology
studies in lambs born with surgically created left-to-right shunts (Shunt) demonstrate a >2-fold increase in
pulmonary…
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