Search results for "Forming"

showing 10 items of 1719 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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Sequential BMP7/TGF-β1 signaling and microbiota instruct mucosal Langerhans cell differentiation

2018

Capucha et al. demonstrate that mucosal Langerhans cell (LC) differentiation from pre–dendritic cells and monocytes involves consecutive BMP7 and TGF-β1 signaling in separate anatomical locations. Moreover, mucosal microbiota regulates the development of LCs that in turn shape microbial and immunological homeostasis.

Male0301 basic medicineLangerhans cellBone Morphogenetic Protein 7ImmunologyReceptor Transforming Growth Factor-beta Type IBiologyArticle311Transforming Growth Factor beta1Mice03 medical and health sciencesDownregulation and upregulation319Langerhans cell differentiationmedicineAnimalsHumansImmunology and AllergyLectins C-TypeImmunity MucosalResearch ArticlesBone Morphogenetic Protein Receptors Type IMice KnockoutLamina propriaintegumentary systemMicrobiotaStem CellsMouth MucosaMucous membraneCell DifferentiationEpitheliumUp-RegulationCell biologyMice Inbred C57BLBone morphogenetic protein 7Mannose-Binding Lectins030104 developmental biologymedicine.anatomical_structureLangerhans CellsAntigens SurfaceSignal transductionTranscriptomeSignal TransductionJournal of Experimental Medicine
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The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema

2018

Hereditary angioedema (HAE) is a rare autosomic-dominant disorder characterized by a deficiency of C1 esterase inhibitor which causes episodic swellings of subcutaneous tissues, bowel walls and upper airways that are disabling and potentially life-threatening. We evaluated n = 17 patients with confirmed HAE diagnosis during attack and remission state and n = 19 healthy subjects. The samples were tested for a panel of IL (Interleukin)-17-type cytokines (IL-1β, IL-6, IL-10, granulocyte–macrophage colony stimulating factor (GM-CSF), IL-17, IL-21, IL-22, IL-23) and transforming growth factor-beta (TGF-β) subtypes. Data indicate that there are variations of cytokine levels in HAE subjects compar…

Male0301 basic medicinemedicine.medical_treatmentC1 esterase inhibitorInterleukin-23chemistry.chemical_compoundSubcutaneous TissueTransforming Growth Factor betaChildHereditary angioedemaHematologyInterleukin-17InterleukinGeneral MedicineMiddle AgedIntestinesCytokineHereditary angioedemaFemaleInterleukin 17medicine.symptomComplement C1 Inhibitor ProteinAdultmedicine.medical_specialtyAdolescentBradykininBronchiBradykininGeneral Biochemistry Genetics and Molecular Biology03 medical and health sciencesInternal medicinemedicineHumansCytokineAgedBiochemistry Genetics and Molecular Biology (all)Angioedemabusiness.industryInterleukinsAngioedemas HereditaryGranulocyte-Macrophage Colony-Stimulating Factormedicine.diseaseColony-stimulating factor030104 developmental biologyGene Expression RegulationchemistryCase-Control StudiesImmunologyTh17 CellsbusinessClinical and Experimental Medicine
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Vascular‐derived TGF‐β increases in the stem cell niche and perturbs neurogenesis during aging and following irradiation in the adult mouse brain

2012

Neurogenesis decreases during aging and following cranial radiotherapy, causing a progressive cognitive decline that is currently untreatable. However, functional neural stem cells remained present in the subventricular zone of high dose-irradiated and aged mouse brains. We therefore investigated whether alterations in the neurogenic niches are perhaps responsible for the neurogenesis decline. This hypothesis was supported by the absence of proliferation of neural stem cells that were engrafted into the vascular niches of irradiated host brains. Moreover, we observed a marked increase in TGF-β1 production by endothelial cells in the stem cell niche in both middle-aged and irradiated mice. I…

MaleAgingNeurogenesisStem cell theory of agingSubventricular zoneBiologyMice03 medical and health sciences0302 clinical medicineNeural Stem CellsTransforming Growth Factor betamedicineAnimalsHumansTGF-betaStem Cell NicheProgenitor cellResearch ArticlesCell Proliferation030304 developmental biology0303 health sciencesirradiationNeurogenesisBrainEndothelial CellsNeural stem cellCell biologyMice Inbred C57BLEndothelial stem cellNeuroepithelial cellmedicine.anatomical_structureImmunologyMolecular Medicine030217 neurology & neurosurgerySignal TransductionAdult stem cellEMBO Molecular Medicine
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SARS CoV2 infection _The longevity study perspectives

2021

Graphical abstract

MaleAgingssRNA single-stranded RNARFLP restriction fragment length polymorphismHSPs heat shock proteinsReviewPTMs post-translational modificationsSevere Acute Respiratory SyndromeBiochemistryHIV-1 human immunodeficiency virus-1TNF-α tumor necrosis factor-αEC endothelial cells0302 clinical medicineFluAV influenza A virusI insertionMedicineIFN-γ interferon-γDIC disseminated intravascular coagulationPCR Polymerase Chain Reactionmedia_commonAged 80 and overLongevityRBD receptor-binding domainNeurologyLongevity modelMI myocardial infarctionNK natural killerhPIV2 human parainfluenza virus type 2media_common.quotation_subjectResearching genetic basis of resistance and potential pharmacological targetsLongevityDBP diastolic blood pressureNF-Kb nuclear transcription factor kBRANTES regulated upon activation normal T cell expressed and secretedMphi human macrophages03 medical and health sciencesCox 2 cyclooxygenase 2ORF open reading framePT prothrombin timeSettore MED/05 - Patologia ClinicaHumansMolecular BiologyInflammatory genesARDS acute respiratory distress syndromeNO nitric oxideD deletionCpGIs CpG islandsT2DM type 2 diabetes mellitusmedicine.diseaseFDP fibrin degradation products030104 developmental biologySARS CoV2 severe acute respiratory syndrome Coronavirus 2 virusImmunologyBMI body max indexItalian nonagenarians/centenariansRSV respiratory syncytial virusComplication030217 neurology & neurosurgeryMAPK mitogen-activated protein kinaseIP-10 IFN-γ -Inducible Protein 1040301 basic medicineAT1R activity of angiotensin 1 receptorsDCs dentritic cellsSSCP single strand conformation polymorphismACE/DD polymorphism of the angiotensin converting enzymeFGF21 fibroblast growth factor 21TLR4 toll-like receptor 4NAD nicotinamide adenine dinucleotideACE angiotensin-I converting enzymeAT2R activity of angiotensin 2 receptorsCOVID-19 Coronavirus disease 2019Respiratory distressACE2 angiotensin converting enzyme 2MKP-1 mitogen-activated protein kinase phosphatase-1 ()PD protease domainSNP single nucleotide polymorphismEH essential hypertensionTNFR tumor necrosis factor receptorINR international normalized ratio of the prothrombin timePAI-1 plasminogen activator inhibitor-1Ang angiotensinLPS lipopolysaccharideMCP1 monocyte chemoattractant protein-1medicine.symptomaPTT partial thromboplastin timeBiotechnologyDUSP1 dual specificity phosphatase 1Coronavirus disease 2019 (COVID-19)PC prostate cancerRAS renin-angiotensin aldosterone systemCCR5Δ32 genetic variant of chemokine receptorCOVID-19 Researching genetic basis of resistance and potential pharmacological targets Italian nonagenarians/centenarians Longevity modelAsymptomaticSARS-1 severe acute respiratory syndrome virus 1SIRT-1 Sirtuin 1Th1 t-helper lymphocyte type 1Immune systemROS reactive oxygen speciesTGF-β transforming growth factor betaET-1 endothelin-1ComputingMethodologies_COMPUTERGRAPHICSADAM-17 metallopeptidase domain 17business.industrySARS-CoV-2SBP systolic blood pressureCOVID-19HDACs histone deacetylasesComorbidityImmune Systembusiness5-LO lipoxygenase 5Ageing Research Reviews
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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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Activin A circulating levels in patients with bone metastasis from breast or prostate cancer

2006

Recent studies have highlighted that Activin A, a member of the transforming growth factor-beta (TGF-beta) superfamily, may be involved in the regulation of osteoblastic activity and in osteoclast differentiation. Therefore, we have investigated the clinical significance of its circulating levels in patients with bone metastasis. Activin A serum concentrations were determined, by a commercially available enzyme-linked immunosorbent assay kit, in 72 patients with breast cancer (BC) or prostatic cancer (PC) with (BM+) or without (BM-) bone metastases, in 15 female patients with age-related osteoporosis (OP), in 20 patients with benign prostatic hypertrophy (BPH) and in 48 registered healthy b…

MaleCancer Researchmedicine.medical_specialtyBone diseaseOsteoporosisProstatic HyperplasiaBone NeoplasmsBreast NeoplasmsActivin A; Benign prostatic hypertrophy; Bone metastasis; Breast cancer; Neoplasm; > Osteoporosis; Prostate cancer; Transforming growth factor ¦Â; Tumor markersSensitivity and SpecificityGastroenterologyProstate cancerBreast cancerBreast cancerOsteoclastInternal medicineBiomarkers TumormedicineHumansAgedAged 80 and overHematologyProstate cancerbusiness.industryOsteoporosiProstatic NeoplasmsBone metastasisCancerGeneral MedicineMiddle AgedActivin ATransforming growth factor ¦Âmedicine.diseaseBenign prostatic hypertrophyActivinsmedicine.anatomical_structureEndocrinologyOncologyBone metastasiTumor markersOsteoporosisNeoplasmFemalebusiness
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Serum follistatin in patients with prostate cancer metastatic to the bone

2010

The clinical significance of circulating fol- listatin (FLST), an inhibitor of the multifunctional cytokine activin A (Act A), was investigated in patients with prostate cancer (PCa). The serum concentrations of this molecule were determined by an enzyme-linked immunosorbent assay (ELISA) in PCa patients with (M?) or without (M0) bone metastases, in patients with benign prostate hyperplasia (BPH) and in healthy sub- jects (HS). The effectiveness of FLST in detecting PCa patients with skeletal metastases was determined by the receiver operating characteristic (ROC) curve analysis. Serum FLST was significantly higher in PCa patients than in BPH patients (P = 0.001) or HS (P = 0.011). Converse…

MaleCancer Researchmedicine.medical_specialtyFollistatinBone diseaseSettore MED/06 - Oncologia MedicaBone metastasis cancer follistatin prostate cancer transforming growth factor beta tumor markersBone Neoplasmsurologic and male genital diseasesGastroenterologySensitivity and SpecificityProstate cancerInternal medicineMedicineHumansClinical significanceAgedHematologybusiness.industryCancerBone metastasisProstatic NeoplasmsGeneral MedicineHyperplasiaMiddle AgedProstate-Specific Antigenmedicine.diseaseActivinsProstate-specific antigenEndocrinologyOncologySettore BIO/14 - Farmacologiabusiness
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Follistatin as potential therapeutic target in prostate cancer

2012

Follistatin is a single-chain glycosylated protein whose primary function consists in binding and neutralizing some members of the transforming growth factor-β superfamily such as activin and bone morphogenic proteins. Emerging evidence indicates that this molecule may also play a role in the malignant progression of several human tumors including prostate cancer. In particular, recent findings suggest that, in this tumor, follistatin may also contribute to the formation of bone metastasis through multiple mechanisms, some of which are not related to its specific activin or bone morphogenic proteins' inhibitory activity. This review provides insight into the most recent advances in understa…

MaleFollistatinendocrine systemCancer Researchmedicine.medical_specialtyanimal structuresGlycosylated proteinProstate cancerInternal medicinemedicineAnimalsHumansPharmacology (medical)Molecular Targeted TherapyActivin Bone metastasis.Cancer . Follistatin Prostate cancerbiologybusiness.industryProstatic NeoplasmsCancerBone metastasisSUPERFAMILYmedicine.diseaseEndocrinologyOncologyembryonic structuresDisease Progressionbiology.proteinCancer researchbusinesshormones hormone substitutes and hormone antagonistsFunction (biology)FollistatinTransforming growth factor
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Hepatocellular expression of a dominant-negative mutant TGF-β type II receptor accelerates chemically induced hepatocarcinogenesis

2001

The potent growth-inhibitory activity of cytokines of the transforming growth factor-beta (TGF-beta) superfamily and their widespread expression in epithelia suggest that they may play an important role in the maintenance of epithelial homeostasis. To analyse TGF-beta mediated tumor suppressor activity in the liver, we generated transgenic mice overexpressing a dominant negative type II TGF-beta receptor in hepatocytes under control of the regulatory elements of the human C-reactive protein gene promoter. Transgenic animals exhibited constitutive and liver-specific transgene expression. The functional inactivation of the TGF-beta signaling pathway in transgenic hepatocytes was shown by redu…

MaleGenetically modified mouseCancer Researchmedicine.medical_specialtyCarcinoma HepatocellularTransgeneMice TransgenicProtein Serine-Threonine KinasesBiologymedicine.disease_causeMiceLiver Neoplasms ExperimentalTransforming Growth Factor betaInternal medicineGeneticsmedicineAnimalsRNA MessengerMolecular BiologyCells CulturedTissue homeostasisDNA synthesisReceptor Transforming Growth Factor-beta Type IICell biologyC-Reactive ProteinEndocrinologymedicine.anatomical_structureHepatocyteMutationHepatocytesSignal transductionCarcinogenesisReceptors Transforming Growth Factor betaTransforming growth factorOncogene
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