Search results for " fibroblast"

showing 10 items of 140 documents

Ataluren for the Treatment of Usher Syndrome 2A Caused by Nonsense Mutations

2019

The identification of genetic defects that underlie inherited retinal diseases (IRDs) paves the way for the development of therapeutic strategies. Nonsense mutations caused approximately 12% of all IRD cases, resulting in a premature termination codon (PTC). Therefore, an approach that targets nonsense mutations could be a promising pharmacogenetic strategy for the treatment of IRDs. Small molecules (translational read-through inducing drugs

0301 basic medicinepatient-derived fibroblastsUsher syndromechemistry.chemical_compound0302 clinical medicineMedicineTRIDSpectroscopyCells CulturedExtracellular Matrix ProteinsOxadiazolesGeneral MedicinePhenotypeImmunohistochemistryComputer Science ApplicationsRetinitis pigmentosaCodon Nonsenseocular therapyUsher syndromeUsher SyndromesNonsense mutationModels BiologicalCatalysisArticleInorganic Chemistry03 medical and health sciencesStructure-Activity RelationshipAtalurenCiliogenesisparasitic diseasesRetinitis pigmentosaHumansGenetic Predisposition to DiseasePhysical and Theoretical ChemistryMolecular BiologyGenetranslational read-throughbusiness.industryOrganic ChemistryHEK 293 cellsFibroblastsmedicine.diseaseAtaluren030104 developmental biologyHEK293 CellschemistryProtein BiosynthesisMutationCancer researchbusiness030217 neurology & neurosurgeryInternational Journal of Molecular Sciences
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Positive effects of an extracellular matrix hydrogel on rat anterior cruciate ligament fibroblast proliferation and collagen mRNA expression

2015

Summary Background/Objective We have previously shown that an extracellular matrix (ECM) bioscaffold derived from porcine small intestine submucosa (SIS) enhanced the healing of a gap injury of the medial collateral ligament as well as the central third defect of the patellar tendon. With the addition of a hydrogel form of SIS, we found that a transected goat anterior cruciate ligament (ACL) could also be healed. The result begs the research question of whether SIS hydrogel has positive effects on ACL fibroblasts (ACLFs) and thus facilitates ACL healing. Methods In the study, ECM-SIS hydrogel was fabricated from the digestion of decellularised and sterilised sheets of SIS derived from αGal-…

ACL fibroblasts; Fibre morphology; Growth factors; Hydrogel; Porcine small intestine submucosa; Orthopedics and Sports Medicinelcsh:Diseases of the musculoskeletal systemfibre morphologyAnterior cruciate ligamentExtracellular matrixchemistry.chemical_compoundgrowth factorsmedicineOrthopedics and Sports MedicineBovine serum albuminFibroblastporcine small intestine submucosabiologyCell growthAnatomyGrowth factorACL fibroblastsACL fibroblastMolecular biologymedicine.anatomical_structurechemistrySelf-healing hydrogelsbiology.proteinOriginal Articlelcsh:RC925-935hydrogelBromodeoxyuridineExplant cultureJournal of Orthopaedic Translation
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CAF-like state in primary skin fibroblasts with constitutional BRCA1 epimutation sheds new light on tumor suppressor deficiency-related changes in he…

2016

Constitutive epimutations of tumor suppressor genes are increasingly considered as cancer predisposing factors equally to sequence mutations. In light of the emerging role of the microenvironment for cancer predisposition, initiation, and progression, we aimed to characterize the consequences of a BRCA1 epimutation in cells of mesenchymal origin. We performed a comprehensive molecular and cellular comparison of primary dermal fibroblasts taken from a monozygous twin pair discordant for recurrent cancers and BRCA1 epimutation, whose exceptional clinical case we previously reported in this journal. Comparative transcriptome analysis identified differential expression of extracellular matrix-r…

Adult0301 basic medicineCancer ResearchTwinsHaploinsufficiencyKetone BodiesExtracellular matrixTranscriptome03 medical and health sciencesCell Line TumormedicineHumansGenes Tumor SuppressorMolecular BiologyPDPNCells CulturedOligonucleotide Array Sequence AnalysisSkinExtracellular Matrix ProteinsbiologyBRCA1 ProteinCell growthGenes HomeoboxCancerDNA MethylationFibroblastsmedicine.diseaseGene Expression Regulation Neoplastic030104 developmental biologyCulture Media ConditionedMutationDNA methylationImmunologyCancer researchbiology.proteinCytokinesCancer-Associated FibroblastsFemaleNeoplasm Recurrence LocalACTA2TranscriptomeResearch PaperEpigenetics
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Multicenter Phase I Study of Erdafitinib (JNJ-42756493), Oral Pan-Fibroblast Growth Factor Receptor Inhibitor, in Patients with Advanced or Refractor…

2018

AbstractPurpose:Here, we report results of the first phase I study of erdafitinib, a potent, oral pan-FGFR inhibitor.Patients and Methods:Patients age ≥18 years with advanced solid tumors for which standard antineoplastic therapy was no longer effective were enrolled (NCT01703481). Parts 2 to 4 employed molecular screening for activating FGFR genomic alterations. In patients with such alterations, two selected doses/schedules identified during part 1 dose-escalation [9 mg once daily and 10 mg intermittently (7 days on/7 days off), as previously published (Tabernero JCO 2015;33:3401-8)] were tested.Results:The study included 187 patients. The most common treatment-related adverse events were…

AdultMale0301 basic medicineCancer Researchmedicine.medical_specialtyAdministration OralAntineoplastic AgentsDrug resistanceGastroenterologyYoung Adult03 medical and health sciencesHyperphosphatemia0302 clinical medicineRefractoryErdafitinibNeoplasmsQuinoxalinesInternal medicinemedicineHumansNeoplasmDosingAdverse effectProtein Kinase InhibitorsAgedNeoplasm StagingAged 80 and overbusiness.industryGenetic VariationMiddle AgedPrognosismedicine.diseaseReceptors Fibroblast Growth FactorTreatment Outcome030104 developmental biologyOncologyTolerabilityDrug Resistance Neoplasm030220 oncology & carcinogenesisRetreatmentPyrazolesFemalebusinessClinical Cancer Research
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Dermatofibrosarcoma protuberans: clinical, pathological, and genetic (COL1A1-PDGFB ) study with therapeutic implications.

2009

Aims:  To analyse the presence of collagen type I alpha 1–platelet-derived growth factor beta (COL1A1–PDGFB) transcripts in 20 cases of dermatofibrosarcoma protuberans (DFSP) and to assess the relationship between COL1A1 breakpoints and clinical and histopathological variables. Methods and results:  Multiplex reverse transcriptase-polymerase chain reaction was carried out using frozen tissue. Our series contained 14 men and six women. Histologically, most cases were of conventional type (n = 9), followed by fibrosarcoma (n = 4), Bednar tumour (n = 2), sclerosing (n = 2), myoid (n = 1) and atrophic (n = 1) DFSP, and giant cell fibroblastoma (n = 1). Immunohistochemistry revealed CD34 express…

AdultMalePathologymedicine.medical_specialtyHistologySkin NeoplasmsAdolescentCD34Antineoplastic AgentsBiologyCollagen Type IPiperazinesPathology and Forensic MedicineYoung AdultDermatofibrosarcoma protuberansmedicineHumansAgedDNA PrimersAged 80 and overPDGFBBase SequenceDermatofibrosarcomaGeneral MedicineGiant-cell fibroblastomaMiddle Agedmedicine.diseaseMohs SurgeryCollagen Type I alpha 1 ChainImatinib mesylatePyrimidinesFusion transcriptCOL1A1/PDGFB Fusion GeneBenzamidesImatinib MesylateFemaleGene FusionDermatofibrosarcomaGenes sisHistopathology
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Serum VEGF and b-FGF profiles after tension-free or conventional hernioplasty.

2005

Angiogenesis is strongly influenced by vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (b-FGF), whose production is also regulated by interferon (IFN)-gamma and interleukin (IL)-10. The aim of this study was to evaluate the modifications of serum VEGF, b-FGF, IFN-gamma and IL-10 levels in patients with inguinal hernia undergoing hernioplasty with the Lichtenstein technique (LH) using polypropylene mesh or with Bassini open conventional inguinal hernia repair (BH). MATERIALS AND METHODS: Randomly, 16 patients underwent BH, and 16 were treated with the LH technique using polypropylene mesh. Blood samples were collected 24 h prior to surgery and then 6, 24, 48 and …

AdultMaleVascular Endothelial Growth Factor Amedicine.medical_specialtyAngiogenesisBasic fibroblast growth factorUrologyEnzyme-Linked Immunosorbent AssayHernia InguinalStatistics Nonparametricchemistry.chemical_compoundInterferon-gammaMedicineHumansIFN-γMeshAnalysis of Variancebusiness.industryInterleukinMiddle AgedSurgical Meshmedicine.diseaseVEGFSurgeryInterleukin-10Vascular endothelial growth factorInterleukin 10Inguinal herniachemistryHernioplastyIL-10SurgeryCytokine secretionFibroblast Growth Factor 2b-FGFbusinessAbdominal surgery
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Endothelium-derived factors in microalbuminuric and nonmicroalbuminuric essential hypertensives

2000

Previous evidence has demonstrated a relationship between growth factors and cardiovascular diseases. This study was aimed at evaluating levels of some endothelium-derived growth factors, and their relationship with microalbuminuria (MAU), in essential hypertension. Ninety-nine mild-moderate essential hypertensives (EH) and 25 healthy controls were studied. All patients underwent 24-h blood pressure monitoring, serum endothelin-1 (ET-1), basic fibroblast growth factor (bFGF) and platelet-derived growth factor (PDGF), and 24-h MAU assays. Later, EH were divided into two subsets consisting of microalbuminurics (MAU >11 microg/min) and nonmicroalbuminurics (MAU <11 microg/min). In microalbumin…

AdultMalemedicine.hormonemedicine.medical_specialtyPlatelet-derived growth factorBasic fibroblast growth factorBlood PressureEnzyme-Linked Immunosorbent AssayEssential hypertensionEndothelinschemistry.chemical_compoundInternal medicineInternal MedicinemedicineAlbuminuriaHumansPlatelet-Derived Growth FactorCreatinineProteinuriaEndothelin-1business.industryBlood Pressure Monitoring Ambulatorymedicine.diseaseEndocrinologyBlood pressurechemistrySpectrophotometryCreatinineHypertensionFemaleFibroblast Growth Factor 2MicroalbuminuriaEndothelium Vascularmedicine.symptombusinessBiomarkersAmerican Journal of Hypertension
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The FGF-2/FGFRs neurotrophic system promotes neurogenesis in the adult brain.

2009

Neurogenesis occurs in two regions of the adult brain, namely, the subventricular zone (SVZ) throughout the wall of the lateral ventricle and the subgranular zone (SGZ) of the dentate gyrus (DG) in hippocampal formation. Adult neurogenesis requires several neurotrophic factors to sustain and regulate the proliferation and differentiation of the adult stem cell population. In the present review, we examine the cellular and functional aspects of a trophic system mediated by fibroblast growth factor-2 (FGF-2) and its receptors (FGFRs) related to neurogenesis in the SVZ and SGZ of the adult rat brain. In the SVZ, FGF-2 is expressed in GFAP-positive cells of SVZ but is not present in proliferati…

AgingNeurogenesisSubventricular zoneCerebral VentriclesSubgranular zoneNeurotrophic factorsPrecursor cellmedicineAnimalsHumansBiological PsychiatrybiologyDentate gyrusNeurogenesisBrainReceptors Fibroblast Growth FactorPsychiatry and Mental healthmedicine.anatomical_structurenervous systemNeurologybiology.proteinFibroblast Growth Factor 2Neurology (clinical)NeurosciencePrecursor cells Fibroblast growth factor-2 (FGF-2) Fibroblast growth factor receptor 1 (FGFR-1) Fibroblast growth factor receptor 2 (FGFR-2) Subgranular zone (SGZ) Subventricular zone (SVZ) NeurogenesisSignal TransductionAdult stem cellNeurotrophin
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Agonist-induced formation of FGFR1 homodimers and signaling differ among members of the FGF family

2011

Fibroblast growth factor receptor 1 (FGFR1) is known to be activated by homodimerization in the presence of both the FGF agonist ligand and heparan sulfate glycosaminoglycan. FGFR1 homodimers in turn trigger a variety of downstream signaling cascades via autophosphorylation of tyrosine residues in the cytoplasmic domain of FGFR1. By means of Bioluminescence Energy Resonance Transfer (BRET) as a sign of FGFR1 homodimerization, we evaluated in HEK293T cells the effects of all known FGF agonist ligands on homodimer formation. A significant correlation between BRET(2) signaling and ERK1/2 phosphorylation was observed, leading to a further characterization of the binding and signaling properties…

AgonistMAPK/ERK pathwaymedicine.drug_classBiophysicsSettore BIO/11 - Biologia MolecolareBiologyLigandsFibroblast growth factorSettore BIO/09 - FisiologiaBiochemistrychemistry.chemical_compoundFluorescence Resonance Energy TransfermedicineHumansReceptor Fibroblast Growth Factor Type 1Molecular BiologyMitogen-Activated Protein Kinase 1Mitogen-Activated Protein Kinase 3Fibroblast growth factor receptor 1HEK 293 cellsAutophosphorylationCell BiologyHeparan sulfateFibroblast growth factors FGFR1 Homodimerization BRET MAPKCell biologyFibroblast Growth Factorsstomatognathic diseasesHEK293 CellschemistrySettore BIO/14 - FarmacologiaPhosphorylationHeparitin SulfateProtein MultimerizationBiochemical and Biophysical Research Communications
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Enhancement of the FGFR1 signaling in the FGFR1-5-HT1A heteroreceptor complex in midbrain raphe 5-HT neuron systems. Relevance for neuroplasticity an…

2015

New findings show existence of FGFR1-5-HT1A heteroreceptor complexes in 5-HT nerve cells of the dorsal and median raphe nuclei of the rat midbrain and hippocampus. Synergistic receptor-receptor interactions in these receptor complexes indicated their enhancing role in hippocampal plasticity. The existence of FGFR1-5-HT1A heteroreceptor complexes also in midbrain raphe 5-HT nerve cells open up the possibility that antidepressant drugs by increasing extracellular 5-HT levels can cause an activation of the FGF-2/FGFR1 mechanism in these nerve cells as well. Therefore, the agonist modulation of the FGFR1-5-HT1A heteroreceptor complexes and their specific role is now determined in rat medullary …

AgonistSerotoninmedicine.medical_specialtymedicine.drug_classCellular differentiationBiophysicsHeteroreceptor complexBiologyHeteroreceptorBiochemistrySettore BIO/09 - FisiologiaCell LineMidbrainDorsal raphe nucleusMesencephalonInternal medicinemedicineAnimalsSerotonin 5-HT1A receptorReceptor Fibroblast Growth Factor Type 1Protein Interaction MapsPhosphorylationExtracellular Signal-Regulated MAP KinasesMolecular BiologyNeurons8-Hydroxy-2-(di-n-propylamino)tetralinNeuronal PlasticityRapheDepressionAnimalExtracellular Signal-Regulated MAP KinaseCell BiologySerotonin 5-HT1 Receptor AgonistsNeuronFibroblast growth factor receptorRatsEndocrinologymedicine.anatomical_structurenervous systemReceptor Serotonin 5-HT1AAutoreceptorRatFibroblast Growth Factor 2Serotonin 5-HT1 Receptor AgonistNeuronDimerizationNeuroscienceDepression; Dimerization; Fibroblast growth factor receptor; Heteroreceptor complex; Neuronal plasticity; Serotonin 5-HT1A receptor; 8-Hydroxy-2-(di-n-propylamino)tetralin; Animals; Cell Line; Extracellular Signal-Regulated MAP Kinases; Fibroblast Growth Factor 2; Mesencephalon; Neurons; Phosphorylation; Rats; Receptor Fibroblast Growth Factor Type 1; Receptor Serotonin 5-HT1A; Serotonin; Serotonin 5-HT1 Receptor Agonists; Neuronal Plasticity; Protein Interaction Maps
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