Search results for " TGF-B"

showing 5 items of 5 documents

Bi-allelic LoF NRROS Variants Impairing Active TGF-β1 Delivery Cause a Severe Infantile-Onset Neurodegenerative Condition with Intracranial Calcifica…

2020

Negative regulator of reactive oxygen species (NRROS) is a leucine-rich repeat-containing protein that uniquely associates with latent transforming growth factor beta-1 (TGF- β1) and anchors it on the cell surface; this anchoring is required for activation of TGF-β1 in macrophages and microglia. We report six individuals from four families with bi-allelic variants in NRROS. All affected individuals had neurodegenerative disease with refractory epilepsy, developmental regression, and reduced white matter volume with delayed myelination. The clinical course in affected individuals began with normal development or mild developmental delay, and the onset of seizures occurred within the first ye…

Male0301 basic medicineInflammationBiologyintracranial calcificationneuroinflammationTransforming Growth Factor beta103 medical and health sciences0302 clinical medicineReportTGF-β1NRROSGeneticsmedicineHumansAllelesGenetics (clinical)NeuroinflammationBrain DiseasesMicrogliaMacrophagesNeurodegenerationneurodegenerationCalcinosisGenetic VariationInfantNeurodegenerative Diseasesmedicine.diseaseNFKB1Latent TGF-beta binding proteinHEK293 Cells030104 developmental biologymedicine.anatomical_structureLatent TGF-beta Binding ProteinsImmunologyKnockout mouseFemaleMicrogliamutationmedicine.symptomDevelopmental regression030217 neurology & neurosurgeryThe American Journal of Human Genetics
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Leptin and leptin receptor expression in asthma.

2009

Background The adipokine leptin is a potential new mediator for bronchial epithelial homeostasis. Asthma is a chronic inflammatory disease characterized by airway remodeling that might affect disease chronicity and severity. TGF-β is a tissue growth factor the dysregulation of which is associated with airway remodeling. Objective We sought to determine whether a bronchial epithelial dysfunction of the leptin/leptin receptor pathway contributes to asthma pathogenesis and severity. Methods We investigated in vitro the presence of leptin/leptin receptor on human bronchial epithelial cells. Then we studied the effect of TGF-β and fluticasone propionate on leptin receptor expression. Finally, th…

AdultLeptinMalemedicine.medical_specialtyPyridinesMorpholinesImmunologyAdipokineBronchiRespiratory MucosaSettore MED/10 - Malattie Dell'Apparato RespiratorioSettore BIO/09 - FisiologiaCell LinePathogenesisTransforming Growth Factor beta1Leptin leptin receptor severe asthma epithelium TGF-b remodelingInternal medicineImmunology and AllergyMedicineHumansEnzyme InhibitorsReceptorCell ProliferationLeptin receptorbusiness.industryTumor Necrosis Factor-alphaLeptindigestive oral and skin physiologyImidazolesMiddle AgedEpitheliumAsthmaRecombinant Proteinsrespiratory tract diseasesAndrostadienesmedicine.anatomical_structureEndocrinologyChromonesImmunologyFluticasoneReceptors LeptinFemalebusinesshormones hormone substitutes and hormone antagonistsEx vivoTransforming growth factorThe Journal of allergy and clinical immunology
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Mongersen, an oral SMAD7 antisense oligonucleotide, and crohn's disease

2015

Crohn's disease-related inflammation is characterized by reduced activity of the immunosuppressive cytokine transforming growth factor β1 (TGF-β1) due to high levels of SMAD7, an inhibitor of TGF-β1 signaling. Preclinical studies and a phase 1 study have shown that an oral SMAD7 antisense oligonucleotide, mongersen, targets ileal and colonic SMAD7.In a double-blind, placebo-controlled, phase 2 trial, we evaluated the efficacy of mongersen for the treatment of persons with active Crohn's disease. Patients were randomly assigned to receive 10, 40, or 160 mg of mongersen or placebo per day for 2 weeks. The primary outcomes were clinical remission at day 15, defined as a Crohn's Disease Activit…

MaleSMAD7 antisense oligonucleotidemedicine.medical_treatmentOligonucleotidesPharmacologyPLACEBO-CONTROLLED TRIALTHERAPYGastroenterologylaw.inventionACTIVATIONImmunosuppressive AgentGlucocorticoidRandomized controlled trialCrohn DiseaselawOligonucleotideMedicineYoung adultCrohn's diseaseSettore MED/12 - GastroenterologiabiologyINDUCTIONMedicine (all)Remission InductionGeneral MedicineMiddle AgedCrohn's diseaseCytokineC-Reactive ProteinCombinationDrug Therapy CombinationFemaleDrugImmunosuppressive AgentsCOLITISHumanAdultmedicine.medical_specialtyAdolescentINFLAMMATORY-BOWEL-DISEASE PLACEBO-CONTROLLED TRIAL NECROSIS-FACTOR-ALPHA TGF-BETA-1-MEDIATED SUPPRESSION COLITIS INDUCTION ACTIVATION EFFICACY THERAPY MICEPlaceboSmad7 ProteinDose-Response RelationshipYoung AdultPharmacotherapyDouble-Blind MethodDrug TherapyInternal medicineHumansAntisenseGlucocorticoidsAgedDose-Response Relationship Drugbusiness.industryC-reactive proteinNECROSIS-FACTOR-ALPHAOligonucleotides AntisenseTGF-BETA-1-MEDIATED SUPPRESSIONEFFICACYmedicine.diseaseClinical trialMICEbiology.proteinbusinessAdolescent; Adult; Aged; C-Reactive Protein; Crohn Disease; Dose-Response Relationship Drug; Double-Blind Method; Drug Therapy Combination; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Middle Aged; Oligonucleotides; Oligonucleotides Antisense; Remission Induction; Smad7 Protein; Young Adult; Medicine (all)INFLAMMATORY-BOWEL-DISEASE
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TGF-β Signaling Pathways in Different Compartments of the Lower Airways of Patients With Stable COPD

2017

Background: The expression and localization of transforming growth factor-β (TGF-β) pathway proteins in different compartments of the lower airways of patients with stable COPD is unclear. We aimed to determine TGF-β pathway protein expression in patients with stable COPD. Methods: The expression and localization of TGF-β pathway components was measured in the bronchial mucosa and peripheral lungs of patients with stable COPD (n = 44), control smokers with normal lung function (n = 24), and control nonsmoking subjects (n = 11) using immunohistochemical analysis. Results: TGF-β1, TGF-β3, and connective tissue growth factor expression were significantly decreased in the bronchiolar epithelium…

MaleCCN2 connective tissue growth factorSmad Proteinsairway inflammationCritical Care and Intensive Care MedicineTRAP-1 transforming growth factor-β receptor-associated binding proteinPulmonary Disease Chronic ObstructiveLAP latency-associated peptideSMAD small mother against decapentaplegicBAMBI CTGF SMAD TGF-B airway inflammation autoimmunityLungTGF transforming growth factorLLC large latent complexBAMBI CTGF SMAD TGF-β Airway Inflammation AutoimmunityautoimmunityMiddle Agedrespiratory systemLTBP latent transforming growth factor-β binding proteinImmunohistochemistryTGIF 5′-TG-3′-interacting factorECM extracellular matrixTGFBI transforming growth factor-β-induced proteinFemaleCardiology and Cardiovascular MedicinePI3K phosphoinositide 3-kinaseSignal TransductionTGF-βPulmonary and Respiratory MedicineTGF-βR TGF-β receptorSocio-culturaleBronchiRespiratory MucosaArticleTGF-BTransforming Growth Factor beta1Transforming Growth Factor beta3Macrophages AlveolarHumansAgedBAMBI; CTGF; SMAD; TGF-β; airway inflammation; autoimmunityBAMBIMembrane ProteinsCTGFBMP bone morphogenetic proteinBAMBI; CTG; SMAD; TGF-β; airway inflammation; autoimmunityCTGBAMBI bone morphogenetic proteins and activin membrane-bound inhibitorrespiratory tract diseasesairway inflammation; autoimmunity; BAMBI; CTGF; SMAD; TGF-β; Pulmonary and Respiratory Medicine; Critical Care and Intensive Care Medicine; Cardiology and Cardiovascular MedicineCase-Control StudiesBiomarkersMAPK mitogen-activated protein kinaseSMAD
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Analysis of molecular mechanisms and anti-tumoural effects of zoledronic acid in breast cancer cells

2012

Zoledronic acid (ZOL) is the most potent nitrogen-containing bisphosphonate (N-BPs) that strongly binds to bone mineral and acts as a powerful inhibitor of bone resorption, already clinically available for the treatment of patients with osteolytic metastases. Recent data also suggest that ZOL, used in breast cancer, may provide more than just supportive care modifying the course of the disease, though the possible molecular mechanism of action is still unclear. As breast cancer is one of the primary tumours with high propensity to metastasize to the bone, we investigated, for the first time, differential gene expression profile on Michigan Cancer Foundation-7 (MCF-7) breast cancer cells tre…

medicine.medical_specialtyAngiogenesismedicine.medical_treatmentBlotting WesternAngiogenesis InhibitorsAntineoplastic AgentsBreast NeoplasmsBiologyReal-Time Polymerase Chain ReactionZoledronic AcidZOL FN1 TGF-b1 THBS-1 invasion breast cancerBone resorptionThrombospondin 1Transforming Growth Factor beta1breast cancerBreast cancerTGF-β1Internal medicineThrombospondin 1medicineHumansBone ResorptionCell ProliferationMatrigelDiphosphonatesFN1Gene Expression ProfilingImidazolesCancerOriginal ArticlesCell BiologyZOLBisphosphonateMicroarray Analysisinvasionmedicine.diseaseFibronectinsUp-RegulationGene Expression Regulation NeoplasticEndocrinologyZoledronic acidTHBS-1MCF-7 CellsCancer researchMolecular MedicineFemalemedicine.drug
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