Search results for "TRANSCRIPTION FACTORS"

showing 10 items of 848 documents

A recurrent TP63 mutation causing EEC3 and Rapp–Hodgkin syndromes

2016

The ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome 3 (EEC3; OMIM #604292), the Rapp-Hodgkin syndrome (RHS), and various other syndromes are caused by mutations in the TP63 gene, which encodes a p53-like transcription factor. Here, we report on a woman aged 37 years and her daughter aged 3 years with the previously reported c.1028G>A (p.Arg343Gln) mutation in exon 8 of TP63. The mother lacked ectrodactyly, indicating a diagnosis of RHS, whereas the girl presented with all three major features (ectrodactyly, ectodermal dysplasia, clefting) and different minor features (including small and brittle nails, and recurrent conjunctivitis believed to be because of stenotic and blo…

Adult0301 basic medicineHeterozygoteEctodermal dysplasiamedicine.medical_specialtyEctrodactylyFoot Deformities CongenitalCleft Lipmedia_common.quotation_subjectmedicine.disease_causePathology and Forensic MedicineFingers030207 dermatology & venereal diseases03 medical and health sciencesExon0302 clinical medicineEctodermal DysplasiaTP63medicineHumansAlleleAllelesGenetics (clinical)media_commonDaughterMutationbusiness.industryTumor Suppressor ProteinsFaciesExonsGeneral Medicinemedicine.diseaseDermatologyPenetrancePedigreeCleft PalatePhenotype030104 developmental biologyAmino Acid SubstitutionChild PreschoolMutationPediatrics Perinatology and Child HealthFemaleAnatomybusinessHand Deformities CongenitalTranscription FactorsClinical Dysmorphology
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The role of nesfatin and selected molecular factors in various types of endometrial cancer

2019

Objectives: Endometrial cancers (ECs) are the most common gynaecological cancers in well developed countries. Diabetes and metabolic syndrome are among the biggest risk factors. Nesfatin-1, the adipokine derivative of NUCB2 (nucleobindin derivative 2) is linked to the clinical course of EC. Molecular factors, including mutations in MLH1 and MHS2 genes, c-MET and ARID1A are also related to prognosis in endometrial cancer. Material and methods: Using sections of paraffin-embedded preparations and immunohistochemistry, the expression of NESF1, MLH1, MSH2,c-MET and ARID1A were examined. Results: In this study on protein expression, EC tissues manifested (although insignificantly) an elevated ex…

AdultC-MetARID1AAdipokineMLH1chemistry.chemical_compoundNESF-1Biomarkers TumormedicineHumansNucleobindinsc-METAgedRetrospective StudiesAged 80 and overbusiness.industryEndometrial cancerMLH1Obstetrics and GynecologyMiddle AgedPrognosismedicine.diseaseARID1AImmunohistochemistryMSH2Endometrial NeoplasmsDNA-Binding ProteinschemistryMSH2endometrial cancerCancer researchImmunohistochemistryFemaleMetabolic syndromebusinessTranscription FactorsGinekologia Polska
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The transcription factor ZEB1 (deltaEF1) promotes tumour cell dedifferentiation by repressing master regulators of epithelial polarity.

2007

Epithelial to mesenchymal transition (EMT) is implicated in the progression of primary tumours towards metastasis and is likely caused by a pathological activation of transcription factors regulating EMT in embryonic development. To analyse EMT-causing pathways in tumouri-genesis, we identified transcriptional targets of the E-cadherin repressor ZEB1 in invasive human cancer cells. We show that ZEB1 repressed multiple key determinants of epithelial differentiation and cell–cell adhesion, including the cell polarity genes Crumbs3, HUGL2 and Pals1-associated tight junction protein. ZEB1 associated with their endogenous promoters in vivo, and strongly repressed promotor activities in reporter …

AdultCancer ResearchChromatin ImmunoprecipitationCellular differentiationImmunoblottingDown-RegulationBreast NeoplasmsBiologymedicine.disease_causeEpitheliumArticleCell polarityGeneticsmedicineTumor Cells CulturedHumansNeoplasm InvasivenessEpithelial–mesenchymal transitionCell adhesionPromoter Regions GeneticMolecular BiologyTranscription factorEpithelial polarityAgedOligonucleotide Array Sequence AnalysisHomeodomain ProteinsMembrane GlycoproteinsReverse Transcriptase Polymerase Chain ReactionGene Expression ProfilingCell PolarityMembrane ProteinsZinc Finger E-box-Binding Homeobox 1Cell DifferentiationMiddle AgedCadherinsCytoskeletal ProteinsMicroscopy FluorescenceCancer cellColonic NeoplasmsCancer researchDisease ProgressionSnail Family Transcription FactorsCarcinogenesisNucleoside-Phosphate KinaseTranscription FactorsOncogene
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Lack of correlation between expression of HIF-1alpha protein and oxygenation status in identical tissue areas of squamous cell carcinomas of the uter…

2004

Abstract Hypoxia inducible factor-1α (HIF-1α) has been proposed as a candidate endogenous marker of tumor hypoxia and as a molecular mediator of hypoxia-driven malignant progression and acquired treatment resistance. In this study, HIF-1α expression in 68 biopsies of oxygenation measurement tracks from squamous cell carcinomas of the uterine cervix of 38 patients was assessed. Expression of HIF-1α was commonly found to increase as a function of distance from microvessels, at the center of tumor cell aggregations, and in the vicinity of necrotic areas. However, there was no correlation of HIF-1α expression with median oxygen tension (oxygen partial pressure; pO2) and hypoxic fractions (hypox…

AdultCancer ResearchPathologymedicine.medical_specialtyPartial PressureCellUterine Cervical NeoplasmsEndogenyBiologyGene expressionmedicineHumansAgedNeoplasm StagingCell NucleusTumor hypoxiaOxygenationHypoxia (medical)Middle AgedHypoxia-Inducible Factor 1 alpha SubunitCell HypoxiaOxygen tensionOxygenmedicine.anatomical_structureOncologyEpidermoid carcinomaCarcinoma Squamous CellFemalemedicine.symptomTranscription FactorsCancer research
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Quantitative methylation analysis of developmentally important genes in human pregnancy losses after ART and spontaneous conception.

2009

To study possible effects of assisted reproductive technologies (ART) on epigenetic reprogramming, we have analyzed the DNA methylation levels of differentially methylated regions (DMRs) of seven imprinted genes (H19, MEG3, LIT1, MEST, NESP55, PEG3 and SNRPN) as well as the promoter regions of the pluripotency gene NANOG and the tumor suppressor gene APC in chorionic villus samples (CVS) of 42 spontaneous miscarriages and stillbirths after ART and 29 abortions/stillbirths after spontaneous conception. We did not find an increased rate of faulty methylation patterns after ART, but significant and trend differences (ROC curve analysis, Wilcoxon test) in the methylation levels of LIT1 (P = 0.0…

AdultEmbryologyGenes APCReproductive Techniques AssistedKruppel-Like Transcription FactorsGestational AgeReproductive technologyBiologyRisk AssessmentYoung AdultPregnancyRisk FactorsGermanyGeneticsHumansGenetic Predisposition to DiseaseEpigeneticsIsraelMolecular BiologyGeneticsRegulation of gene expressionMosaicismObstetrics and GynecologyGene Expression Regulation DevelopmentalCell BiologyMethylationDNA MethylationMiddle AgedStillbirthAbortion SpontaneousDifferentially methylated regionsPhenotypeReproductive MedicineDNA methylationLinear ModelsFemaleGenomic imprintingReprogrammingDevelopmental BiologyMaternal AgeMolecular human reproduction
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The extent of HLA-DR expression on HLA-DR+Tregs allows the identification of patients with clinically relevant borderline rejection

2013

Regulatory T cells (Tregs) were shown to be involved into the pathogenesis of acute rejection after transplantation. The suppressive activity of the total regulatory T cell pool depends on its percentage of highly suppressive HLA-DR(+) -Treg cells. Therefore, both the suppressive activity of the total Treg pool and the extent of HLA-DR expression of HLA-DR(+) -Tregs (MFI HLA-DR) were estimated in non transplanted volunteers, patients with end-stage renal failure (ESRF), healthy renal transplant patients with suspicion on rejection, due to sole histological Bord-R or sole acute renal failure (ARF), and patients with clinically relevant borderline rejection (Bord-R and ARF). Compared to patie…

AdultGraft RejectionMaleRegulatory T cellRisk AssessmentSensitivity and SpecificityT-Lymphocytes RegulatoryFlow cytometryCohort StudiesPathogenesisYoung AdultPredictive Value of TestsReference ValuesBiopsymedicineHLA-DRHumansSurvival rateAgedSubclinical infectionTransplantationmedicine.diagnostic_testbusiness.industryBiopsy NeedleForkhead Transcription FactorsHLA-DR AntigensMiddle AgedFlow CytometryImmunohistochemistryKidney TransplantationSurvival RateTransplantationTreatment Outcomemedicine.anatomical_structureROC CurveCase-Control StudiesImmunologyLinear ModelsKidney Failure ChronicFemalebusinessBiomarkersTransplant International
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DR(high+)CD45RA(-)-Tregs potentially affect the suppressive activity of the total Treg pool in renal transplant patients.

2011

Recent studies show that regulatory T cells (Tregs) play an essential role in tolerance induction after organ transplantation. In order to examine whether there are differences in the composition of the total CD4(+)CD127(low+/-)FoxP3(+)- Treg cell pool between stable transplant patients and patients with biopsy proven rejection (BPR), we compared the percentages and the functional activity of the different Treg cell subsets (DR(high+)CD45RA(-)-Tregs, DR(low+)CD45RA(-)-Tregs, DR(-)CD45RA(-)-Tregs, DR(-)CD45RA(+)-Tregs). All parameters were determined during the three different periods of time after transplantation (0-30 days, 31-1,000 days, >1,000 days). Among 156 transplant patients, 37 pat…

AdultGraft Rejectionmedicine.medical_specialtymedicine.drug_classClinical Research DesignImmune Cellslcsh:Medicinechemical and pharmacologic phenomenaMonoclonal antibodyT-Lymphocytes RegulatoryOrgan transplantationInterleukin-7 Receptor alpha SubunitYoung AdultT-Lymphocyte SubsetsBiopsymedicineHumanslcsh:ScienceKidney transplantationAgedKidneyMultidisciplinarymedicine.diagnostic_testbusiness.industrylcsh:RInterleukin-2 Receptor alpha Subunithemic and immune systemsForkhead Transcription FactorsHLA-DR AntigensMiddle AgedImmunologic Subspecialtiesmedicine.diseaseKidney TransplantationTransplant rejectionTransplantationTolerance inductionmedicine.anatomical_structureNephrologyImmunologyLeukocyte Common AntigensMedicinelcsh:QClinical ImmunologySurgerybusinessResearch ArticlePloS one
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Cardiomyopathy due to PRDM16 mutation: First description of a fetal presentation, with possible modifier genes

2020

PRDM16 (positive regulatory domain 16) is localized in the critical region for cardiomyopathy in patients with deletions of chromosome 1p36, as defined by Gajecka et al., American Journal of Medical Genetics, 2010, 152A, 3074-3083, and encodes a zinc finger transcription factor. We present the first fetal case of left ventricular non-compaction (LVNC) with a PRDM16 variant. The third-trimester obstetric ultrasound revealed a hydropic fetus with hydramnios and expanded hypokinetic heart. After termination of pregnancy, foetopathology showed a eutrophic fetus with isolated cardiomegaly. Endocardial fibroelastosis was associated with non-compaction of the myocardium of the left ventricle. Exom…

AdultHeart Defects CongenitalMalemedicine.medical_specialtyCardiomyopathyBiologyLabor PresentationGenetic HeterogeneityPregnancyExome SequencingGeneticsmedicineHumansMissense mutationGenetic Predisposition to DiseaseGenetics (clinical)Exome sequencingGeneticsFetusGenes ModifierGenetic heterogeneityInfant NewbornEndocardial fibroelastosisMiddle AgedFetal Presentationmedicine.diseasePedigreeDNA-Binding ProteinsMutationMedical geneticsFemaleCardiomyopathiesTranscription FactorsAmerican Journal of Medical Genetics Part C: Seminars in Medical Genetics
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The autoantigen La/SS-B: Analysis of the expression of alternatively spliced La mRNA isoforms

1996

The gene for the nuclear autoantigen La/SS-B encodes two La mRNA isoforms. In order to study the function and expression of both La mRNA forms, an in situ hybridization procedure was developed allowing the selective identification of either exon 1 or exon 1'. For this purpose, digoxigenin-labeled exon-specific sense and anti-sense probes were prepared by in vitro transcription from plasmids that contained the respective exon sequence. Detection of the probes was carried out by using rhodamine-conjugated anti-digoxigenin antibody and confocal laser scanning microscopy. Both La mRNAs were found in the cytoplasm of endothelial cells but not in smooth muscle cells. In addition to the in situ te…

AdultHistologyMolecular Sequence DataGene ExpressionIn situ hybridizationBiologyAutoantigensPolymerase Chain ReactionPathology and Forensic MedicineExonExon trappingIsomerismGene expressionHumansSaphenous VeinEndotheliumRNA MessengerMammary ArteriesGeneIn Situ HybridizationMessenger RNABase SequenceExonsCell BiologyMolecular biologyAlternative SplicingLiverRibonucleoproteinsCytoplasmPrimer (molecular biology)DNA ProbesTranscription Factors
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Lack of Hypoxic Response in Uterine Leiomyomas despite Severe Tissue Hypoxia

2008

Abstract Hypoxia is now established as a key factor influencing the pathophysiology of malignant growth. Among other effects, hypoxia modulates the expression of a multitude of genes through the induction of hypoxia-inducible transcription factors. This differential gene expression favors angiogenesis, cell survival, an invasive/metastatic phenotype, and resistance to anticancer therapies. Because benign tumors do not exhibit these traits, one might expect these entities to be neither hypoxic nor to induce the genetic hypoxia response program. To test this hypothesis, an investigation of the oxygenation status of 17 leiomyomas and 1 leiomyosarcoma of the uterus using polarographic needle el…

AdultLeiomyosarcomaLeiomyosarcomaCancer ResearchPathologymedicine.medical_specialtyAngiogenesisUterusUterine Cervical NeoplasmsApoptosisBiologyImmunoenzyme TechniquesAntigens NeoplasmBiopsyBasic Helix-Loop-Helix Transcription FactorsIn Situ Nick-End LabelingmedicineHumansProspective StudiesCarbonic Anhydrase IXHypoxiaCarbonic AnhydrasesCell ProliferationGlucose Transporter Type 1Uterine leiomyomaLeiomyomamedicine.diagnostic_testMiddle AgedHypoxia (medical)Hypoxia-Inducible Factor 1 alpha Subunitmedicine.diseasePhenotypeOxygenmedicine.anatomical_structureLeiomyomaOncologyMyometriumFemalemedicine.symptomCancer Research
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