Search results for "Repo"

showing 10 items of 2753 documents

Cutis verticis gyrata and Noonan syndrome: report of two cases with pathogenetic variant in SOS1 gene

2022

Abstract Background Noonan and Noonan-like syndromes are multisystem genetic disorders, mainly with autosomal dominant trasmission, caused by mutations in several genes. Missense pathogenetic variants of SOS1 gene are the second most common cause of Noonan syndrome (NS) and account approximately for 13% to 17% of cases. Subjects carrying a pathogenetic variant in SOS1 gene tend to exhibit a distinctive phenotype that is characterized by ectodermal abnormalities. Cutis verticis gyrata (CVG) is a rare disease, congenital or acquired, characterized by the redundancy of skin on scalp, forming thick skin folds and grooves of similar aspect to cerebral cortex gyri. Several references in the liter…

Rare DiseasesScalpCutis verticis gyrataCase reportHumansNoonan syndromeGeneral MedicineSOS1K170EItalian Journal of Pediatrics
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Requirement of Retinoic Acid Receptor Isotypes α, β, and γ during the Initial Steps of Neural Differentiation of PCC7 Cells

2005

Retinoic acid (RA) is indispensable for morphogenesis and differentiation of several tissues, including the nervous system. The requirement of the RA receptor (RAR) isotypes alpha, beta, and gamma and the putative role of retinoid X receptor-(RXR) signaling in RA-induced neural differentiation, was analyzed. For this compound-selective retinoids and the murine embryonal carcinoma cell line PCC7, a model system for RA-dependent neural differentiation was used. The present paper shows that proliferating PCC7 cells primarily express RXRalpha and RARalpha, lower levels of RXRbeta, and barely detectable amounts of RARbeta, RARgamma, and RXRgamma. At receptor-selective concentrations, only a RARa…

Receptors Retinoic AcidRetinoic acidReceptors Cytoplasmic and NuclearApoptosisLigandsMicechemistry.chemical_compoundEndocrinologyGenes ReporterNuclear Receptor Subfamily 6 Group A Member 1Protein IsoformsRetinoidReceptorGlutathione TransferaseNeuronsCell DeathReverse Transcriptase Polymerase Chain ReactionCell DifferentiationGeneral MedicineImmunohistochemistryUp-RegulationCell biologyDNA-Binding ProteinsBiochemistrySignal transductionPlasmidsProtein BindingSignal Transductionmedicine.drugTranscriptional ActivationDNA Complementarymedicine.drug_classRecombinant Fusion ProteinsBlotting WesternDown-RegulationTretinoinRetinoid X receptorBiologyTransfectionCell LineTretinoinCell Line TumormedicineAnimalsHumansMolecular BiologyCell ProliferationKineticsRetinoic acid receptorRetinoid X ReceptorschemistryNuclear receptorRNAOctamer Transcription Factor-3Transcription FactorsMolecular Endocrinology
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The induction of cytochrome P450 3A5 (CYP3A5) in the human liver and intestine is mediated by the xenobiotic sensors pregnane X receptor (PXR) and co…

2004

Induction of cytochrome P450 3A (CYP3A) by xenobiotics may lead to clinically relevant drug interactions. In contrast with other CYP3A family members, studies on the inducibility of CYP3A5 indicate conflicting results. We report the induction of CYP3A5 mRNA in 13 of 16 hepatocyte preparations exposed to rifampin. Furthermore, induction of CYP3A5 mRNA was observed in intestinal biopsies in three of eight probands following exposure to the antibiotic. The highest absolute levels of CYP3A5 transcripts were found following rifampin treatment in hepatocytes and intestines from carriers of CYP3A5*1 alleles. Elucidation of the mechanism involved in CYP3A5 induction revealed that constitutively act…

Receptors SteroidTime FactorsCYP3ABiopsyAmino Acid MotifsReceptors Cytoplasmic and NuclearPharmacology030226 pharmacology & pharmacyBiochemistryTransactivation0302 clinical medicineCytochrome P-450 Enzyme SystemGenes ReporterCytochrome P-450 CYP3AIntestinal MucosaReceptorPromoter Regions GeneticGenes Dominant0303 health sciencesPregnane X receptorPregnane X Receptor3. Good healthmedicine.anatomical_structureLiverHepatocyteRifampinPlasmidsProtein BindingTranscriptional ActivationHeterozygoteGenotypeBiologyTransfectionXenobiotics03 medical and health sciencesmedicineHumansRNA MessengerMolecular BiologyAllelesConstitutive Androstane Receptor030304 developmental biologyMessenger RNACYP3A4Cell BiologyMolecular biologyProtein Structure TertiaryHepatocytesRNADrug metabolismTranscription FactorsThe Journal of biological chemistry
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Recommendation of RILEM TC237-SIB on fragmentation test for recycled asphalt

2019

This recommendation describes how to characterize the reclaimed asphalt through the fragmentation test. The guidelines given hereafter are based on the results of a round robin test organized by the RILEM Technical Committee 237-SIB ‘‘Testing and characterization of sustainable innovative bituminous materials and systems’’ and provide information on the testing procedure, data analysis and indications for the preparation of a test report.

Reclaimed asphalt (RA)Round robin test (RRT)Computer science0211 other engineering and technologies02 engineering and technologyBuilding and ConstructionBituminous materialsFragmentation testCivil engineeringTest (assessment)Market fragmentationTest reportMechanics of MaterialsAsphalt021105 building & constructionFragmentation test; Reclaimed asphalt (RA); Round robin test (RRT)General Materials ScienceTechnical committeeRound robin testCivil and Structural Engineering
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Protection from lethal septic peritonitis by neutralizing the biological function of interleukin 27

2006

The immune response to bacterial infections must be tightly controlled to guarantee pathogen elimination while preventing tissue damage by uncontrolled inflammation. Here, we demonstrate a key role of interleukin (IL)-27 in regulating this critical balance. IL-27 was rapidly induced during murine experimental peritonitis induced by cecal ligation and puncture (CLP). Furthermore, mice deficient for the EBI3 subunit of IL-27 were resistant to CLP-induced septic peritonitis as compared with wild-type controls, and this effect could be suppressed by injection of recombinant single-chain IL-27. EBI3−/− mice displayed significantly enhanced neutrophil migration and oxidative burst capacity during…

Recombinant Fusion ProteinsImmunologyDown-RegulationPeritonitisInflammationPeritonitisBiologySepsisMiceImmune systemSepsismedicineAnimalsImmunology and AllergyInterleukin 27Innate immune systemBacteriaInterleukinsBrief Definitive ReportInterleukinReceptors Interleukinmedicine.diseaseImmunity InnateUp-RegulationRespiratory burstMice Inbred C57BLProtein SubunitsSolubilityImmunologyBrief Definitive Reportsmedicine.symptomGranulocytesJournal of Experimental Medicine
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Specific Binding of Baculoviruses Displaying gp64 Fusion Proteins to Mammalian Cells

2001

Viral vectors displaying specific ligand binding moieties have raised an increasing interest in the area of targeted gene therapy. In this report, we describe baculovirus vectors displaying either a functional single chain antibody fragment (scFv) specific for the carcinoembryonic antigen (CEA) or the synthetic IgG binding domains (ZZ) derived from protein A of Staphylococcus aureus. In addition, the vectors were engineered to incorporate a reporter gene encoding the enhanced green fluorescent protein (EGFP) under the transcriptional regulation of the cytomegalovirus (CMV) IE promoter. Display of the targeting moieties on the viral surface was achieved through fusion to the N-terminus of gp…

Recombinant Fusion ProteinsvirusesGenetic VectorsGreen Fluorescent ProteinsImmunoglobulin Variable RegionBiophysicsSpodopteraTransfectionBiochemistryCell LineGreen fluorescent proteinViral vector03 medical and health sciencesGenes ReporterTransduction GeneticCricetinaeTumor Cells CulturedAnimalsStaphylococcal Protein AMolecular Biology030304 developmental biology0303 health sciencesReporter genebiology030302 biochemistry & molecular biologyAntibodies MonoclonalGenetic TherapyCell BiologyTransfectionFusion proteinMolecular biologyCarcinoembryonic Antigen3. Good healthLuminescent ProteinsMicroscopy FluorescenceIgG bindingbiology.proteinAntibodyProtein ABaculoviridaeViral Fusion ProteinsBiochemical and Biophysical Research Communications
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The Tobacco mosaic virus movement protein associates with but does not integrate into biological membranes

2014

Plant positive-strand RNA viruses require association with plant cell endomembranes for viral translation and replication, as well as for intra- and intercellular movement of the viral progeny. The membrane association and RNA binding of the Tobacco mosaic virus (TMV) movement protein (MP) are vital for orchestrating the macromolecular network required for virus movement. A previously proposed topological model suggests that TMV MP is an integral membrane protein with two putative -helical transmembrane (TM) segments. Here we tested this model using an experimental system that measured the efficiency with which natural polypeptide segments were inserted into the ER membrane under conditions…

Recombinant Fusion ProteinsvirusesMolecular Sequence DataImmunologyGene ExpressionMicrobiologiaBiologyEndoplasmic ReticulumMicrobiologyCell membraneGenes ReporterPlant CellsVirologymedicineTobacco mosaic virusAmino Acid SequenceMovement proteinIntegral membrane proteinStructure and AssemblyCell MembraneViral translationfungifood and beveragesBiological membraneVirologyTransmembrane proteinTransport proteinCell biologyVirusPlant Viral Movement ProteinsTobacco Mosaic VirusProtein Transportmedicine.anatomical_structureInsect ScienceHydrophobic and Hydrophilic InteractionsProtein Binding
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Combined surgical and prosthetic approach for rehabilitation of frontonasal defect using custom made titanium implant: a case report

2011

Cranial vault deformities as a sequelae to trauma may be as high as 70%. The basic indications for cranioplasty are improvement of disfigured aesthetics and to provide protection to the intra-cranial contents. The possibility of producing custom-made implants opened a new era in the reconstructive surgery of the craniofacial deformities. The outcome of craniofacial bone reconstruction is thought to be dependent on surgical skills, quality of adjacent soft tissues, size and location of the bone defect and choice of repair method. The use of autologous bone for craniofacial reconstruction may be restricted due to limited amounts of donor bone. Combined surgical and prosthetic rehabilitation o…

Reconstructive surgerymedicine.medical_specialtyTitanium implantRehabilitationbusiness.industrymedicine.medical_treatmentSoft tissueDentistryOdontología:CIENCIAS MÉDICAS [UNESCO]Ciencias de la saludCranioplastySurgeryClinical reportUNESCO::CIENCIAS MÉDICASCranial vaultmedicineCraniofacialbusinessGeneral DentistryJournal of Clinical and Experimental Dentistry
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Vascular atherosclerotic disease: Behaviour of the red cell phospholipids and their relationships with the erythrocyte membrane fluidity. Preliminary…

2016

Red CellPhysiologyPhospholipidAtherosclerotic diseaseHematologyBiologychemistry.chemical_compoundRed blood cellErythrocyte membranemedicine.anatomical_structureBiochemistrychemistryPreliminary reportPhysiology (medical)medicineCardiology and Cardiovascular Medicine
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Electrochemical characterization of mangrove sediments: A proposal of new proxies for organic matter oxidation

2019

Abstract The voltammetric response of microparticulate deposits resulting from solvent evaporation of ethanolic extracts of mangrove sediments in contact with aqueous acetate buffer is hereby described. Dated sediment cores sampled from the Peruvian mangrove system (“Manglares de Tumbes” National Sanctuary) presented voltammetric responses dominated by oxidative signals of organic components that exhibit significant variations depending on the depth. Voltammetric data allowed for the definition of electrochemical indexes representative of the electrochemically oxidable organic matter fraction. These electrochemical indexes were fEAOM (for the total amount of organic matter being electrochem…

Redox state010501 environmental sciences010502 geochemistry & geophysicsElectrochemistryMangrove sediments01 natural sciencesevaporationPeruElectrochemistryChemical analysisInfrared spectroscopyorganic matterchemistry.chemical_classificationAqueous solutionChemistryBiogeochemistryPollutionOrganic matter oxidationEnvironmental chemistryOrganic matter degradationsMangroveoxidationInfrared spectroscopyredox conditionssolventSedimentsVoltammetric responseGeochemistry and PetrologyOrganic compoundsOrganic matter fractionEnvironmental ChemistryOrganic matter14. Life underwaterSpectroscopyOxidation proxies0105 earth and related environmental sciencesparticulate mattermangrove//purl.org/pe-repo/ocde/ford#1.05.00 [https]SedimentBiological materials15. Life on landcoastal sedimentElectrochemical characterizationssediment coreSolvent evaporationVoltammetryethanolacetateaqueous solutionReactive oxygen species
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