Search results for "Base Sequence"

showing 10 items of 1146 documents

Targeted next-generation sequencing of deafness genes in hearing-impaired individuals uncovers informative mutations

2014

Purpose: Targeted next-generation sequencing provides a remarkable opportunity to identify variants in known disease genes, particularly in extremely heterogeneous disorders such as nonsyndromic hearing loss. The present study attempts to shed light on the complexity of hearing impairment. Methods: Using one of two next-generation sequencing panels containing either 80 or 129 deafness genes, we screened 30 individuals with nonsyndromic hearing loss (from 23 unrelated families) and analyzed 9 normal-hearing controls. Results: Overall, we found an average of 3.7 variants (in 80 genes) with deleterious prediction outcome, including a number of novel variants, in individuals with nonsyndromic h…

MaleProbandUsher syndromeGene DosageDeafnessBioinformaticsmedicine.disease_causesensorineural hearing lossConnexinsCohort Studiestargeted next-generation sequencingOriginal Research Articlemutational loadChildGenetics (clinical)Oligonucleotide Array Sequence AnalysisGeneticsMutationmedicine.diagnostic_testHomozygoteHigh-Throughput Nucleotide SequencingPedigreeConnexin 26Treatment OutcomeChild PreschoolFemalemedicine.symptomAdultAdolescentSequence analysisHearing lossdeafness gene panelMolecular Sequence DataBiologynonsyndromic hearing lossDNA sequencingYoung AdultAudiometryGenetic variationotorhinolaryngologic diseasesmedicineHumansGenetic Predisposition to DiseaseFamily HealthBase SequenceGenetic VariationInfantDNASequence Analysis DNAmedicine.diseaseMutationAudiometryGene DeletionGenetics in Medicine
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Down-regulation of Glutathione and Bcl-2 Synthesis in Mouse B16 Melanoma Cells Avoids Their Survival during Interaction with the Vascular Endothelium

2003

B16 melanoma (B16M) cells with high GSH content show high metastatic activity. However, the molecular mechanisms linking GSH to metastatic cell survival are unclear. The possible relationship between GSH and the ability of Bcl-2 to prevent cell death was studied in B16M cells with high (F10) and low (F1) metastatic potential. Analysis of a Bcl-2 family of genes revealed that B16M-F10 cells, as compared with B16M-F1 cells, overexpressed preferentially Bcl-2 (approximately 5.7-fold). Hepatic sinusoidal endothelium-induced B16M-F10 cytotoxicity in vitro increased from approximately 19% (controls) to approximately 97% in GSH-depleted B16M-F10 cells treated with an antisense Bcl-2 oligodeoxynucl…

MaleProgrammed cell deathPore complexCell SurvivalMelanoma ExperimentalDown-RegulationOxidative phosphorylationBiologyBiochemistryOligodeoxyribonucleotides AntisenseMicechemistry.chemical_compoundDownregulation and upregulationCell Line TumorAnimalsButhionine SulfoximineMolecular BiologyBase SequenceTransition (genetics)Cell BiologyGlutathioneGlutathioneMolecular biologyGenes bcl-2Cell biologyMice Inbred C57BLOxidative StressCytosolchemistryEndothelium VascularEffluxCell DivisionJournal of Biological Chemistry
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Epigenetic Control of the foxp3 Locus in Regulatory T Cells

2007

Compelling evidence suggests that the transcription factor Foxp3 acts as a master switch governing the development and function of CD4+ regulatory T cells (Tregs). However, whether transcriptional control of Foxp3 expression itself contributes to the development of a stable Treg lineage has thus far not been investigated. We here identified an evolutionarily conserved region within the foxp3 locus upstream of exon-1 possessing transcriptional activity. Bisulphite sequencing and chromatin immunoprecipitation revealed complete demethylation of CpG motifs as well as histone modifications within the conserved region in ex vivo isolated Foxp3+CD25+CD4+ Tregs, but not in naïve CD25−CD4+ T cells. …

MaleQH301-705.5Bisulfite sequencingImmunologyMolecular Sequence Datachemical and pharmacologic phenomenaCell SeparationThymus GlandBiologyT-Lymphocytes RegulatoryGeneral Biochemistry Genetics and Molecular BiologyEpigenesis GeneticMiceTranscriptional regulationAnimalsEpigeneticsBiology (General)Regulation of gene expressionMice Inbred BALB CGeneral Immunology and MicrobiologyBase SequenceGeneral NeuroscienceInterleukin-2 Receptor alpha SubunitFOXP3Homo (human)hemic and immune systemsForkhead Transcription FactorsDNA MethylationFlow CytometryMolecular biologyMus (mouse)Cell biologyIn VitroDNA demethylationGene Expression RegulationDNA methylationCpG IslandsGeneral Agricultural and Biological SciencesChromatin immunoprecipitationResearch ArticlePLoS Biology
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Pregnane X receptor and yin yang 1 contribute to the differential tissue expression and induction of CYP3A5 and CYP3A4.

2012

The hepato-intestinal induction of the detoxifying enzymes CYP3A4 and CYP3A5 by the xenosensing pregnane X receptor (PXR) constitutes a key adaptive response to oral drugs and dietary xenobiotics. In contrast to CYP3A4, CYP3A5 is additionally expressed in several, mostly steroidogenic organs, which creates potential for induction-driven disturbances of the steroid homeostasis. Using cell lines and mice transgenic for a CYP3A5 promoter we demonstrate that the CYP3A5 expression in these organs is non-inducible and independent from PXR. Instead, it is enabled by the loss of a suppressing yin yang 1 (YY1)-binding site from the CYP3A5 promoter which occurred in haplorrhine primates. This YY1 sit…

MaleReceptors SteroidDrugs and DevicesMolecular Sequence Datalcsh:MedicineSequence HomologyMice TransgenicBiologyModels BiologicalMolecular GeneticsMiceDogsGene expressionMolecular Cell BiologyGeneticsAnimalsCytochrome P-450 CYP3AHumansTissue DistributionEvolutionary SystematicsPharmacokineticsEnzyme inducerBinding sitelcsh:ScienceBiologyCYP3A5 GeneCells CulturedPhylogenyYY1 Transcription FactorPregnane X receptorEvolutionary BiologyMultidisciplinaryCYP3A4Base SequenceYY1lcsh:RPregnane X ReceptorComputational BiologyPromoterMolecular biologyOrganismal EvolutionMice Inbred C57BLNephrologyEnzyme Inductionbiology.proteinMedicinelcsh:QFemaleResearch ArticlePloS one
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The Drosophila ACP65A cuticle gene: deletion scanning analysis of cis-regulatory sequences and regulation by DHR38.

2005

The regulatory sequences of the Drosophila ACP65A cuticle gene were analyzed in vivo in transgenic flies, using both fusion genes constructs and transposase-mediated deletions within a P element containing ACP65A regulatory sequences fused to the lacZ gene (deletion scanning). The sequences located between −594 and +161 are sufficient to confer both temporal and spatial expression specificities, indicating the presence of tissue-specific enhancers and response elements to hormone-induced factors. In addition, timing of expression and tissue-specificity appear to be controlled by distinct cis-regulatory elements, which suggests the existence of independent hormonal and tissue-specific signal…

MaleReceptors SteroidTranscription GeneticTransgenelac operonReceptors Cytoplasmic and NuclearBiologyFusion geneP elementAnimals Genetically ModifiedEndocrinologyGeneticsNuclear Receptor Subfamily 4 Group A Member 1AnimalsDrosophila ProteinsEnhancerGeneCrosses GeneticSequence DeletionGeneticsBase SequenceActivator (genetics)fungiPupaCell BiologyDNA-Binding ProteinsGene Expression RegulationRegulatory sequenceInsect ProteinsDrosophilaFemaleTranscription FactorsGenesis (New York, N.Y. : 2000)
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Convergent sets of data from in vivo and in vitro methods point to an active role of Hsp60 in chronic obstructive pulmonary disease pathogenesis.

2011

BackgroundIt is increasingly clear that some heat shock proteins (Hsps) play a role in inflammation. Here, we report results showing participation of Hsp60 in the pathogenesis of chronic obstructive pulmonary diseases (COPD), as indicated by data from both in vivo and in vitro analyses.Methods and resultsBronchial biopsies from patients with stable COPD, smoker controls with normal lung function, and non-smoker controls were studied. We quantified by immunohistochemistry levels of Hsp10, Hsp27, Hsp40, Hsp60, Hsp70, Hsp90, and HSF-1, along with levels of inflammatory markers. Hsp10, Hsp40, and Hsp60 were increased during progression of disease. We found also a positive correlation between th…

MaleSTRESSPulmonologyChronic Obstructive Pulmonary DiseasesNeutrophilsBiopsyGene ExpressionCD8-Positive T-Lymphocytesmedicine.disease_causeBiochemistryEpitheliumPulmonary function testingPathogenesisACTIVATIONPulmonary Disease Chronic ObstructiveMolecular Cell BiologyLungCOPDMultidisciplinaryReverse Transcriptase Polymerase Chain ReactionCOPD Hsp60QRCOPD heat shock proteins inflammationMiddle AgedImmunohistochemistrymedicine.anatomical_structureEXPERIMENTAL AUTOIMMUNE ENCEPHALOMYELITISMedicineFemalemedicine.symptomInflammation MediatorsSPINAL-CORDResearch ArticleEXPRESSIONanimal structuresCOPD; heat shock proteins; inflammationScienceImmunologyMolecular Sequence DataInflammationBronchichemical and pharmacologic phenomenaHEAT-SHOCK-PROTEIN EXPERIMENTAL AUTOIMMUNE ENCEPHALOMYELITIS ACUTE LUNG INJURY SPINAL-CORD CELL-DEATH KAPPA-B HEAT-SHOCK-PROTEIN-60 STRESS EXPRESSION ACTIVATIONKAPPA-BBiologyHEAT-SHOCK-PROTEINMicrobiologycomplex mixturesCell LineACUTE LUNG INJURYMolecular GeneticsIn vivoStress PhysiologicalHeat shock proteinmedicineGeneticsHumansCOPDRNA MessengerBiologyAgedLungMucous MembraneBase SequenceSettore BIO/16 - Anatomia UmanaMacrophagesfungiImmunityTranscription Factor RelAProteinsComputational BiologyChaperonin 60medicine.diseaseChaperone Proteinsrespiratory tract diseasesGene Expression RegulationCELL-DEATHHEAT-SHOCK-PROTEIN-60inflammationImmunologyheat shock proteinsClinical ImmunologyOxidative stressBiomarkers
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Expression of MMP-2 and MMP-9 in odontogenic myxoma in a child: report of a clinical case

2011

Odontogenic myxoma (OM) is a benign, locally invasive, non-metastasizing neoplasm of the jaw bones. Despite the benign nature of these lesions, there is a high rate of recurrence and the current recommended therapy, depending on the size and behaviour of the lesion, can vary from curettage with peripheral ostectomy, segmental resection up to radical resections for more aggressive lesions. OM is a rare tumour which occurs predominantly in the third decade of life and it is rare in children. Matrix metalloproteinases (MMPs) are a family of extracellular endopeptidases responsible for the degradation and remodelling of extracellular matrix, they are known to be involved in the progression and …

MaleSettore BIO/17 - IstologiaPathologymedicine.medical_specialtymedicine.medical_treatmentOdontogenic TumorsBiologyInferior alveolar nerveMatrix metalloproteinaseOdontogenic myxomaLesionExtracellular matrixSettore MED/28 - Malattie OdontostomatologichemedicineHumansChildGeneral DentistryDNA PrimersBase SequenceReverse Transcriptase Polymerase Chain Reactionmedicine.diseaseCurettageMatrix Metalloproteinase 9ImmunohistochemistryMatrix Metalloproteinase 2medicine.symptomSegmental resectionMyxomaOdontogenic myxoma Child MMP-2 MMP-9 it
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Identification of a novel mutation in the alpha-galactosidase A gene in patients with Fabry disease.

2012

Abstract Objectives Mutation analysis of the alpha-galactosidase A (GLA) gene is a valuable tool for the diagnosis of affected families. In our work, we analyze about one thousand samples per year from patients suspected of having Fabry disease (FD). Design and methods We carried out high resolution melting analysis (HRM) and DNA sequencing of all the exons of the GLA gene. We also assayed the alpha-galactosidase A activity in patients' blood. Results In some members of one family, we identified a new mutation in the GLA gene, c.614delC. This is a deletion of a single nucleotide, a cytosine, in exon 4 of the gene which causes a frameshift mutation. Conclusions Patients with the c.614delC mu…

MaleSettore MED/09 - Medicina InternaClinical BiochemistryDNA Mutational AnalysisHigh Resolution MeltFrameshift mutationExonmedicineHumansFrameshift MutationGeneSequence DeletionGeneticsFamily HealthAlpha-galactosidasebiologyBase Sequencealpha-galactosidase A geneGeneral MedicineExonsmedicine.diseaseMolecular biologyFabry diseasealpha-GalactosidaseMutation (genetic algorithm)Mutation testingbiology.proteinFabry DiseaseFemalemutationClinical biochemistry
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An impaired peroxisomal targeting sequence leading to an unusual bicompartmental distribution of cytosolic epoxide hydrolase

1991

AbstractTo gain an understanding of the mechanism by which the subcellular distribution of cytosolic epoxide hydrolase (cEH) is directed, we have analyzed the carboxy terminal region of rat liver cEH by means of cDNA cloning to define the structure of its possible peroxisomal targeting sequence (PTS). Purified cEH was subjected to peptide analysis following endoproteinase Glu-C digestion and HPLC-separation of the fragments. The obtained sequence information was used to perform PCR experiments resulting in the isolation of a 680 bp cDNA clone encoding the carboxy terminus of cEH. The deduced amino acid sequence displays a terminal tripeptide Ser-Lys-Ile which is highly homologous to the PTS…

MaleSignal peptidePTSanimal structures1303 BiochemistryMolecular Sequence DataBiophysics10050 Institute of Pharmacology and Toxicology610 Medicine & healthTripeptideProtein Sorting SignalsBiologyMicrobodiesBiochemistryAmino acid sequence1307 Cell BiologyCytosol1315 Structural Biology1311 GeneticsStructural BiologyComplementary DNAGenetics1312 Molecular BiologyAnimalsCloning MolecularEpoxide hydrolaseMolecular BiologyPeptide sequenceEpoxide Hydrolaseschemistry.chemical_classificationBase SequencecDNA sequenceDNACell BiologyPeroxisomeMolecular biologyRatsIsoenzymesCytosolPCREnzymeLiverchemistryBiochemistrycEH570 Life sciences; biologyPeptide analysis1304 Biophysics
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Repetitive DNAs in the slug Milax nigricans: association of ribosomal (18S–28S and 5S rDNA) and (TTAGGG)n telomeric sequences) in the slug M. nigrica…

2003

Spermatocyte chromosomes of the slug Milax nigricans (Mollusca: Gastropoda: Pulmonata) were studied using silver staining (Ag-NOR) and fluorescent in situ hybridization (FISH) with four repetitive DNA probes [18S rDNA, 5S rDNA, (TTAGGG)n and (GATA)n]. Silver impregnation was inadequate to localize the chromosome sites of nucleolus organizer regions (NORs) since no silver dots occurred on the chromosomes at spermatogonial metaphase and a diffuse silver stainability could be observed on the bivalents at metaphase-I. Unlike silver staining, single-colour rDNA FISH consistently mapped major ribosomal sites (18S-28S rDNA) on two small-sized chromosomes in spermatogonial cells and on the correspo…

MaleSilverNucleolusGeneral Physics and AstronomyBiologyDNA RibosomalBivalent (genetics)Silver stainSpermatocytesStructural BiologyRNA Ribosomal 28SNucleolus Organizer RegionRNA Ribosomal 18SAnimalsGeneral Materials ScienceRepeated sequenceMetaphaseIn Situ Hybridization FluorescenceRepetitive Sequences Nucleic AcidGeneticsBase SequenceStaining and LabelingRNA Ribosomal 5SChromosomeDNACell BiologyTelomereRibosomal RNAMolecular biologyMolluscaNucleolus organizer regionMicron
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