Search results for "Repressor"

showing 10 items of 212 documents

Genetic and phenotypic dissection of 1q43q44 microdeletion syndrome and neurodevelopmental phenotypes associated with mutations in ZBTB18 and HNRNPU

2017

Subtelomeric 1q43q44 microdeletions cause a syndrome associating intellectual disability, microcephaly, seizures and anomalies of the corpus callosum. Despite several previous studies assessing genotype-phenotype correlations, the contribution of genes located in this region to the specific features of this syndrome remains uncertain. Among those, three genes, AKT3, HNRNPU and ZBTB18 are highly expressed in the brain and point mutations in these genes have been recently identified in children with neurodevelopmental phenotypes. In this study, we report the clinical and molecular data from 17 patients with 1q43q44 microdeletions, four with ZBTB18 mutations and seven with HNRNPU mutations, an…

[SDV.GEN]Life Sciences [q-bio]/GeneticsRepressor Proteins/geneticsddc:618Neurodevelopmental Disorders/geneticsHeterogeneous-Nuclear Ribonucleoproteins/geneticsHeterogeneous-Nuclear RibonucleoproteinsChromosomesRepressor ProteinsPhenotypeChromosomes Human Pair 1Neurodevelopmental DisordersMutationGeneticsPair 1HumansGenetics(clinical)Chromosome Deletion[ SDV.GEN ] Life Sciences [q-bio]/GeneticsOriginal InvestigationHuman
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Glucose uptake in germinating Aspergillus nidulans conidia: involvement of the creA and sorA genes

2003

d-Glucose uptake in germinating wild-typeAspergillus nidulansconidia is an energy-requiring process mediated by at least two transport systems of differing affinities for glucose: a low-affinity system (Km∼1·4 mM) and a high-affinity system (Km∼16 μM). The low-affinity system is inducible by glucose; the high-affinity system is subject to glucose repression effected by the carbon catabolite repressor CreA and is absent insorA3mutant conidia, which exhibit resistance tol-sorbose toxicity. An intermediate-affinity system (Km∼400 μM) is present insorA3conidia germinating in derepressing conditions.creAderepressed mutants show enhanced sensitivity tol-sorbose. The high-affinity uptake system ap…

biologyGlucose uptakeGenes FungalMutantFungal geneticsCatabolite repressionBiological Transport ActiveRepressorCarbohydrate metabolismbiology.organism_classificationMicrobiologyAspergillus nidulansConidiumFungal ProteinsRepressor ProteinsKineticsGlucoseBiochemistryDrug Resistance FungalAspergillus nidulansMutationSorboseMicrobiology
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Engineering the smallest transcription factor: accelerated evolution of a 63-amino acid peptide dual activator-repressor

2019

Transcription factors control gene expression in all life. This raises the question of what is the smallest protein that can support such activity. In nature, Cro from bacteriophage λ is the smallest known repressor (66 amino acids; a.a.) but activators are typically much larger (e.g. λ cI, 237 a.a.). Indeed, previous efforts to engineer a minimal activator from Cro resulted in no activityin vivo. In this study, we show that directed evolution results in a new Cro activator-repressor that functions as efficiently as λ cI,in vivo. To achieve this, we develop Phagemid-Assisted Continuous Evolution: PACEmid. We find that a peptide as small as 63-a.a. functions efficiently as an activator and/o…

chemistry.chemical_classification0303 health sciencesActivator (genetics)RepressorPeptideDirected evolutionAmino acidCell biology03 medical and health sciences0302 clinical medicinechemistryGene expressionGeneTranscription factor030217 neurology & neurosurgery030304 developmental biology
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SAP30L interacts with members of the Sin3A corepressor complex and targets Sin3A to the nucleolus.

2006

Histone acetylation plays a key role in the regulation of gene expression. The chromatin structure and accessibility of genes to transcription factors is regulated by enzymes that acetylate and deacetylate histones. The Sin3A corepressor complex recruits histone deacetylases and in many cases represses transcription. Here, we report that SAP30L, a close homolog of Sin3-associated protein 30 (SAP30), interacts with several components of the Sin3A corepressor complex. We show that it binds to the PAH3/HID (Paired Amphipathic Helix 3/Histone deacetylase Interacting Domain) region of mouse Sin3A with residues 120–140 in the C-terminal part of the protein. We provide evidence that SAP30L induces…

entsyymitvuorovaikutustumajyvänenBiologySAP30Protein Sorting SignalsHistone DeacetylasesArticleCell Line03 medical and health sciencesMice0302 clinical medicineHistone H1Histone H2AGeneticsHistone codeAnimalsHumansGene Silencingnucleolus030304 developmental biologyNuclear receptor co-repressor 2Histone deacetylationGenetics0303 health sciencesgeenitbiokemiaNuclear ProteinsCell biologyRepressor ProteinsProtein TransportSin3 Histone Deacetylase and Corepressor Complextranskriptio (biologia)030220 oncology & carcinogenesisSin-associated proteinsHistone deacetylase complexhistonideasetylaatioHistone deacetylaseproteiinitCorepressorSin-assosioituvat proteiinitCell NucleolusNucleic acids research
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The closure of Pak1-dependent macropinosomes requires the phosphorylation of CtBP1/BARS

2007

Membrane fission is an essential process in membrane trafficking and other cellular functions. While many fissioning and trafficking steps are mediated by the large GTPase dynamin, some fission events are dynamin independent and involve C-terminal-binding protein-1/brefeldinA-ADP ribosylated substrate (CtBP1/BARS). To gain an insight into the molecular mechanisms of CtBP1/BARS in fission, we have studied the role of this protein in macropinocytosis, a dynamin-independent endocytic pathway that can be synchronously activated by growth factors. Here, we show that upon activation of the epidermal growth factor receptor, CtBP1/BARS is (a) translocated to the macropinocytic cup and its surroundi…

genetic structuresEndocytic cycleGTPaseBiologyTRANSCRIPTIONAL COREPRESSOREPIDERMAL GROWTH-FACTORArticleGeneral Biochemistry Genetics and Molecular BiologySYNAPTIC VESICLE ENDOCYTOSISMembrane fissionCell Line TumorMacropinocytic cupHumansPhosphorylationMacropinosomeMolecular BiologyDynaminEpidermal Growth FactorGeneral Immunology and MicrobiologyMEMBRANE FISSIONGeneral NeuroscienceActinsEnterovirus B HumanProtein Structure TertiaryTransport proteinCell biologyDNA-Binding ProteinsAlcohol OxidoreductasesProtein Transportp21-Activated KinasesPLASMA-MEMBRANEPinocytosisPhosphorylationCell Surface ExtensionsIntegrin alpha2beta1The EMBO Journal
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The ecdysone-induced DHR4 orphan nuclear receptor coordinates growth and maturation in Drosophila

2005

0092-8674 (Print) Journal Article Research Support, Non-U.S. Gov't; A critical determinant of insect body size is the time at which the larva stops feeding and initiates wandering in preparation for metamorphosis. No genes have been identified that regulate growth by contributing to this key developmental decision to terminate feeding. We show here that mutations in the DHR4 orphan nuclear receptor result in larvae that precociously leave the food to form premature prepupae, resulting in abbreviated larval development that translates directly into smaller and lighter animals. In addition, we show that DHR4 plays a central role in the genetic cascades triggered by the steroid hormone ecdyson…

medicine.medical_specialtyEcdysonemedicine.medical_treatmentmedia_common.quotation_subjectRepressorReceptors Cytoplasmic and NuclearBiologymedicine.disease_causeGeneral Biochemistry Genetics and Molecular Biologychemistry.chemical_compoundInternal medicineReceptorsmedicineDrosophila ProteinsAnimalsMetamorphosisDrosophila/genetics/*growth & developmentPupa/physiologyRegulator genemedia_commonLarvaMutationMetamorphosisBiochemistry Genetics and Molecular Biology(all)Biological/physiologyfungiMetamorphosis BiologicalPupaGene Expression Regulation DevelopmentalDrosophila Proteins/genetics/*metabolismDevelopmental/physiologyCytoplasmic and Nuclear/genetics/*metabolismNeurosecretory SystemsCell biologyEcdysone/*metabolismSteroid hormoneEndocrinologyNuclear receptorchemistryGene Expression RegulationLarvaLarva/growth & developmentMutationNeurosecretory Systems/metabolismDrosophilaEcdysone
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Increased risk for cervical disease progression of French women infected with the human papillomavirus type 16 E6-350G variant.

2006

Abstract To test the significance of human papillomavirus (HPV) type 16 and HPV16 E6 variants as risk factors for viral persistence and progression to high-grade lesion, we did a nested case-control study within a cohort study of >15,000 Caucasian French women. Three groups infected with high-risk HPV were compared: (a) women with cleared infection (controls, n = 201), (b) women with persistent infection (cases, n = 87), and (c) women who progressed into high-grade lesion (cases, n = 58). Women with persistent HPV infection and those that progressed into high-grade lesions were likelier to harbor HPV16 than other high-risk HPV types [odds ratio (OR), 2.4; 95% confidence interval (95%…

medicine.medical_specialtyEpidemiologyUterine Cervical NeoplasmsCervical intraepithelial neoplasiaLesionCohort StudiesRisk FactorsInternal medicineEpidemiologymedicineOdds RatioHumansRisk factorbusiness.industryPapillomavirus InfectionsHPV infectionvirus diseasesOdds ratioOncogene Proteins Viralmedicine.diseaseUterine Cervical DysplasiaRepressor ProteinsOncologyCase-Control StudiesImmunologyDisease ProgressionFemaleViral diseaseFrancemedicine.symptombusinessCohort studyCancer epidemiology, biomarkersprevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology
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The estrogen receptor α:insulin receptor substrate 1 complex in breast cancer: structure–function relationships

2007

Background: Insulin receptor substrate 1 (IRS-1) is a signaling molecule that exerts a key role in mediating cross talk between estrogen receptor a (ERa) and insulin-like growth factor 1 (IGF-1) in breast cancer cells. Previously, we demonstrated that a fraction of IRS-1 binds ERa, translocates to the nucleus, and modulates ERa-dependent transcription at estrogen response elements (ERE). Here, we studied structure-function relationships of the ER-a:IRS-1 complex under IGF-1 and/or estradiol (E 2 ) stimulation. Materials and methods: ERa and IRS-1 deletion mutants were used to analyze structural and functional ERα/IRS-1 interactions. IRS-1 binding to ERE and IRS-1 role in ERa-dependent ERE t…

medicine.medical_specialtyInsulin Receptor Substrate ProteinsActive Transport Cell NucleusEstrogen receptorRepressorBreast NeoplasmsBiologyStructure-Activity Relationshipestrogen receptor alpha (ERa) Insulin receptor substrate 1 (IRS-1) breast cancerCell Line TumorInternal medicineCoactivatormedicineHumansInsulin-Like Growth Factor IReceptors InterferonEstradiolEstrogen Receptor alphaHematologyDNA-binding domainPhosphoproteinsPeptide FragmentsReceptor InsulinProtein Structure TertiaryCell biologyIRS1Repressor ProteinsPleckstrin homology domainEndocrinologyOncologyInsulin Receptor Substrate ProteinsFemaleChromatin immunoprecipitationProtein BindingAnnals of Oncology
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Estradiol, acting through estrogen receptor alpha, restores dimethylarginine dimethylaminohydrolase activity and nitric oxide production in oxLDL-tre…

2011

Asymmetric dimethylarginine (ADMA) is an endogenous inhibitor of nitric oxide (NO) synthase. ADMA accumulation, mainly due to a decreased dimethylarginine dimethylaminohydrolase (DDAH) activity, has been related to the development of cardiovascular diseases. We investigate whether estradiol prevents the changes induced by oxidized low density lipoprotein (oxLDL) on the DDAH/ADMA/NO pathway in human umbilical artery endothelial cells (HUAEC). HUAEC were exposed to estradiol, native LDL (nLDL), oxLDL and their combinations for 24 h. In some experiments, cells were also exposed to the unspecific estrogen receptor (ER) antagonist ICI 182780, the specific ERα antagonist MPP or specific agonists …

medicine.medical_specialtyProtein-Arginine N-MethyltransferasesEndotheliumNitric Oxide Synthase Type IIImedicine.drug_classBlotting WesternArginineNitric OxideBiochemistryUmbilical ArteriesNitric oxideAmidohydrolasesReceptors G-Protein-Coupledchemistry.chemical_compoundEndocrinologyEnosInternal medicinemedicineEstrogen Receptor betaHumansEstrogens Non-SteroidalMolecular BiologyCells CulturedbiologyEstradiolArtèriesProtein StabilityEstrogen AntagonistsEstrogen Receptor alphaEndoteli vascularbiology.organism_classificationNitric oxide synthaseIsoenzymesLipoproteins LDLRepressor Proteinsmedicine.anatomical_structureEndocrinologychemistryReceptors EstrogenEstrogenbiology.proteinlipids (amino acids peptides and proteins)Endothelium VascularAsymmetric dimethylarginineEstrogen receptor alphaGPER
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A MiR-142-3p/EGR2 Feedback Circuitry In Human CSF-1 Driven Differentiation of Monocytes Into Macrophages

2011

Abstract Abstract 2366 Colony-stimulating factor-1 (CSF-1 or M-CSF) triggers the differentiation of human peripheral blood monocytes into macrophages through and integrated cytokine/transcription factors circuitry. Using microarray profiling to explore the role of microRNAs (miRNAs) in this molecular circuitry, we identified the down-regulation of miR-142-3p in human macrophages obtained from CSF-1-treated monocytes. We show that miR-142-3p is a repressor of the transcription factor EGR2 (Early Growth Response 2) through direct 3'UTR interactions. Interestingly, EGR2 binds the promoter of the pre-miR-142-3p gene to negatively regulate its expression, identifying a self-regulatory feedback l…

medicine.medical_treatmentImmunologyRepressorChronic myelomonocytic leukemiaCell BiologyHematologyBiologyColony-stimulating factormedicine.diseaseBiochemistryCell biologyCytokinemicroRNAmedicineGeneTranscription factorProto-oncogene tyrosine-protein kinase SrcBlood
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