Search results for "nuclei"

showing 10 items of 1273 documents

A novel DFNB1 deletion allele supports the existence of a distant cis-regulatory region that controls GJB2 and GJB6 expression

2010

Contains fulltext : 87760_1.pdf (author's version ) (Open Access) Contains fulltext : 87760_2.pdf (Publisher’s version ) (Closed access) Eleven affected members of a large German-American family segregating recessively inherited, congenital, non-syndromic sensorineural hearing loss (SNHL) were found to be homozygous for the common 35delG mutation of GJB2, the gene encoding the gap junction protein Connexin 26. Surprisingly, four additional family members with bilateral profound SNHL carried only a single 35delG mutation. Previously, we demonstrated reduced expression of both GJB2 and GJB6 mRNA from the allele carried in trans with that bearing the 35delG mutation in these four persons. Usin…

MaleGenetics and epigenetic pathways of disease [NCMLS 6][SDV]Life Sciences [q-bio]PenetranceMESH: Base SequenceRegulatory Sequences Nucleic Acidsensorineural hearing lossConnexinsMESH: GenotypeMESH: Hearing Loss Sensorineural/diagnosisMESH: PenetranceGenotypeCopy-number variationGenetics (clinical)Sequence DeletionGeneticsComparative Genomic Hybridization0303 health sciencesMESH: Genetic TestingMESH: Gene Expression Regulation*030305 genetics & heredityPenetranceGJB2PedigreeConnexin 26MESH: Sequence Deletion*MESH: Hearing Loss Sensorineural/geneticsFemaleChromosome DeletionFunctional Neurogenomics [DCN 2]GJB6GenotypeMESH: PedigreeMESH: Chromosome DeletionHearing Loss SensorineuralMolecular Sequence Dataconnexin 26connexin 30DFNB1gene expression regulationGJB2GJB6sensorineural hearing losssequence deletionBiologyMESH: Connexin 30MESH: Connexins/genetics*MESH: Sequence Homology Nucleic AcidArticleGenomic disorders and inherited multi-system disorders [IGMD 3]03 medical and health sciencesMonoallelic MutationGJB6MESH: Connexin 26Sequence Homology Nucleic AcidConnexin 30otorhinolaryngologic diseasesGeneticsHumansGenetic TestingAlleleGeneMESH: Regulatory Sequences Nucleic Acid/genetics*AllelesDFNB1030304 developmental biologyFamily HealthMESH: HumansMESH: Molecular Sequence DataBase SequenceChromosomes Human Pair 13MESH: AllelesBreakpointMESH: MaleMESH: Comparative Genomic HybridizationGene Expression RegulationMESH: Family Healthbiology.proteinHuman medicineMESH: Chromosomes Human Pair 13/geneticsMESH: FemaleClinical Genetics
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Inhibition of clastogenicity of benzo[a]pyrene and of its trans-7,8-dihydrodiol in mice in vivo by fruits, vegetables, and flavonoids.

2003

In the in vivo mouse bone marrow micronucleus assay, homogenates of spinach, artichoke, peaches, and blue grapes as well as commercial concentrates of these vegetables and fruits reduced induction of micronuclei by benzo[a]pyrene (BaP) by 43-50%. Concentrates of strawberries (31% reduction) and of cauliflower (20% reduction) were less potent. Inhibition of genotoxicity by spinach and peaches was not caused by any delay in maturation of micronucleated erythrocytes as shown by experiments with sampling times of 24, 48, and 72 h after dosing of BaP. Pre-treatment of the mice with spinach 48, 24, and 12h before application of BaP resulted in a 44% reduction of micronuclei while peaches generate…

MaleHealth Toxicology and MutagenesisFlavonoidAdministration OralBone Marrow CellsMice Inbred Strainsmedicine.disease_causecomplex mixturesDihydroxydihydrobenzopyreneschemistry.chemical_compoundClastogenMiceVegetablesGeneticsmedicineBenzo(a)pyreneCytochrome P-450 CYP1A1AnimalsFood scienceMicronuclei Chromosome-Defectivechemistry.chemical_classificationMicronucleus TestsbiologyDose-Response Relationship DrugPlant Extractsfood and beveragesAntimutagenic Agentsbiology.organism_classificationDose–response relationshipBenzo(a)pyrenechemistryBiochemistryLiverFruitMicronucleus testCytochrome P-450 CYP2B1SpinachDrug Therapy CombinationQuercetinQuercetinGenotoxicityInjections IntraperitonealMutagensMutation research
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A Single Copy of the Recently Identified Dual Oxidase Maturation Factor (DUOXA) 1 Gene Produces Only Mild Transient Hypothyroidism in a Patient with …

2011

Dual oxidases (DUOX1 and DUOX2) play a crucial role in the generation of hydrogen peroxide required in the oxidation of iodide and the synthesis of thyroid hormone. Heterodimerization with specific maturation factors (DUOXA1 and DUOXA2) is essential for the maturation and function of the DUOX enzyme complexes. Biallelic loss-of-function mutations of DUOX2 result in congenital hypothyroidism (CH), whereas a single reported case of homozygous DUOXA2 mutation (Y246X) has been associated with mild CH.We now report an infant with transient CH due to a complex genetic alteration of the DUOX/DUOXA system.Our patient was born to euthyroid nonconsanguineous parents and presented with an elevated TSH…

MaleHeterozygoteendocrine system diseasesEndocrinology Diabetes and MetabolismBlotting WesternGenetic VectorsClinical BiochemistryGene DosageMutation MissenseThyrotropinBiologyTransfectionmedicine.disease_causePolymorphism Single NucleotideBiochemistryGene dosageEndocrinologyHypothyroidismPolymorphism (computer science)medicineHumansMissense mutationAlleleGeneAllelesCells CulturedOligonucleotide Array Sequence AnalysisGeneticsMutationfungiBiochemistry (medical)Infant NewbornMembrane ProteinsNADPH OxidasesNucleic Acid Hybridizationfood and beveragesHeterozygote advantageJCEM Online: Brief ReportsDNADual OxidasesMolecular biologyMembrane proteinGene DeletionThe Journal of Clinical Endocrinology & Metabolism
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Efferent connections of the "olfactostriatum": a specialized vomeronasal structure within the basal ganglia of snakes.

2005

Abstract The olfactostriatum is a portion of the basal ganglia of snakes that receives substantial vomeronasal afferents through projections from the nucleus sphericus. In a preceding article, the olfactostriatum of garter snakes (Thamnophis sirtalis) was characterized on the basis of chemoarchitecture (distribution of serotonin, neuropeptide Y and tyrosine hydroxylase) and pattern of afferent connections [Martinez-Marcos, A., Ubeda-Banon, I., Lanuza, E., Halpern, M., 2005. Chemoarchitecture and afferent connections of the “olfactostriatum”: a specialized vomeronasal structure within the basal ganglia of snakes. J. Chem. Neuroanat. 29, 49–69]. In the present study, its efferent connections …

MaleHypoglossal nucleusHypothalamus PosteriorBiotinBiologyNucleus accumbensAmygdalaEfferent PathwaysBasal GangliaNucleus AccumbensVentral pallidumCellular and Molecular NeuroscienceBasal gangliamedicineAnimalsRhodaminesColubridaeDextransAnatomyOlfactory PathwaysAmygdalaVentral tegmental areaSmellStria terminalismedicine.anatomical_structureFemaleFluoresceinVomeronasal OrganRaphe nucleiNeuroscienceJournal of chemical neuroanatomy
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A Polymorphism in the Crhr1 Gene Determines Stress Vulnerability in Male Mice

2014

Chronic stress is a risk factor for psychiatric disorders but does not necessarily lead to uniform long-term effects on mental health, suggesting modulating factors such as genetic predispositions. Here we address the question whether natural genetic variations in the mouse CRH receptor 1 (Crhr1) locus modulate the effects of adolescent chronic social stress (ACSS) on long-term stress hormone dysregulation in outbred CD1 mice, which allows a better understanding of the currently reported genes × environment interactions of early trauma and CRHR1 in humans. We identified 2 main haplotype variants in the mouse Crhr1 locus that modulate the long-term effects of ACSS on basal hypothalamic-pitui…

MaleHypothalamo-Hypophyseal SystemGenotypeGene ExpressionPituitary-Adrenal SystemLocus (genetics)Single-nucleotide polymorphismRegulatory Sequences Nucleic AcidBiologyBinding CompetitivePolymorphism Single NucleotideReceptors Corticotropin-Releasing HormoneMiceEndocrinologyGene FrequencyGenetic predispositionAnimalsHumansGenetic Predisposition to DiseaseChronic stressCRHR1 GeneGeneIn Situ HybridizationSocial stressGeneticsBehavior AnimalTriazinesHaplotypeHaplotypesPituitary GlandPyrazolesFemaleGene-Environment InteractionCorticosteroneStress PsychologicalSignal TransductionEndocrinology
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Gitelman-Like Syndrome Caused by Pathogenic Variants in mtDNA

2022

Contains fulltext : 248375.pdf (Publisher’s version ) (Closed access) BACKGROUND: Gitelman syndrome is the most frequent hereditary salt-losing tubulopathy characterized by hypokalemic alkalosis and hypomagnesemia. Gitelman syndrome is caused by biallelic pathogenic variants in SLC12A3, encoding the Na(+)-Cl(-) cotransporter (NCC) expressed in the distal convoluted tubule. Pathogenic variants of CLCNKB, HNF1B, FXYD2, or KCNJ10 may result in the same renal phenotype of Gitelman syndrome, as they can lead to reduced NCC activity. For approximately 10 percent of patients with a Gitelman syndrome phenotype, the genotype is unknown. METHODS: We identified mitochondrial DNA (mtDNA) variants in th…

MaleKidneyDISEASEion transportGenotypeSolute Carrier Family 12 Member 3Gitelman-s syndromeCHANNEL GENEChildRNA Transfer IlePHOSPHORYLATIONNCCbiologygenetic renal diseaseblood pressureMetabolic Disorders Radboud Institute for Molecular Life Sciences [Radboudumc 6]General MedicineMiddle Agedchronic kidney failureTUBULENa transportPedigreemitochondriaBARTTER-SYNDROMEPhenotypemedicine.anatomical_structureMitochondrial respiratory chainMAGNESIUMNephrologyChild Preschoolepithelial sodium transportFemaleGitelman SyndromeAdultMitochondrial DNAAdolescentGenotypehuman geneticsKCNJ10DNA MitochondrialModels BiologicalPolymorphism Single NucleotideRNA Transfer PheYoung AdultTubulopathymedicineHumansDistal convoluted tubuleHYPOMAGNESEMIAAgedCLCNKBNeurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7]MITOCHONDRIAL-DNA MUTATIONBase SequenceInfantGitelman syndromemedicine.diseaseMolecular biologySODIUM-CHLORIDE COTRANSPORTERHEK293 CellsRenal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11]Basic ResearchMutationbiology.proteinNucleic Acid Conformationchronic kidney disease
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Gene toxicity studies on titanium dioxide and zinc oxide nanomaterials used for UV-protection in cosmetic formulations

2010

Titanium dioxide and zinc oxide nanomaterials, used as UV protecting agents in sunscreens, were investigated for their potential genotoxicity in in vitro and in vivo test systems. Since standard OECD test methods are designed for soluble materials and genotoxicity testing for nanomaterials is still under revision, a battery of standard tests was used, covering different endpoints. Additionally, a procedure to disperse the nanomaterials in the test media and careful characterization of the dispersed test item was added to the testing methods. No genotoxicity was observed in vitro (Ames' Salmonella gene mutation test and V79 micronucleus chromosome mutation test) or in vivo (mouse bone marrow…

MaleMaterials scienceBiomedical EngineeringBone Marrow CellsNanotechnologyCosmeticsGene mutationToxicologymedicine.disease_causeCell LineNanomaterialsMicechemistry.chemical_compoundSalmonellaIn vivoCricetinaeAdministration InhalationMacrophages AlveolarmedicineAnimalsRats WistarMicronuclei Chromosome-DefectiveTitaniumChromatographyMutagenicity TestsBody WeightIn vitroNanostructuresRatschemistryData Interpretation StatisticalMicronucleus testTitanium dioxideZinc OxideMicronucleusSunscreening AgentsGenotoxicityNanotoxicology
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Autoimmune skin inflammation is dependent on plasmacytoid dendritic cell activation by nucleic acids via TLR7 and TLR9

2010

Lupus-prone mice develop a chronic inflammatory response to cutaneous injury that depends on the production of type I interferon, TLR7, and TLR9.

MaleMice 129 StrainImmunologyGene ExpressionInflammationchemical and pharmacologic phenomenaMice Inbred StrainsReceptor Interferon alpha-betaBiologySkin DiseasesArticleProinflammatory cytokinePathogenesisTLR9MiceAutoimmune skin inflammationimmune system diseasesNucleic AcidsmedicineImmunology and AllergyAnimalsLupus Erythematosus SystemicReceptorskin and connective tissue diseasesTLR7SkinAutoimmune skin inflammation; TLR7; TLR9; plasmacytoid dendritic cells.Mice KnockoutPlasmacytoid dendritic cell activationLupus erythematosusReverse Transcriptase Polymerase Chain ReactionTLR9virus diseaseshemic and immune systemsTLR7DNADendritic Cellsmedicine.diseaseFlow CytometryMice Inbred C57BLplasmacytoid dendritic cells.Toll-Like Receptor 7Toll-Like Receptor 9ImmunologyMyeloid Differentiation Factor 88CytokinesFemalemedicine.symptomThe Journal of Experimental Medicine
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Evolution of sex chromosomes: dosage compensation of the Lcp1-4 gene cluster on the evolving neo-X chromosome in Drosophila miranda.

2007

In Drosophila miranda the small multigene family of the larval cuticle protein (Lcp1-4) genes resides on the evolving neo-X and neo-Y sex chromosome pair while in the sibling species Drosophila pseudoobscura and Drosophila persimilis the gene cluster is inherited autosomally. The neo-Y chromosomal Lcp1, Lcp2 and Lcp4 genes are, as previously shown by us, not expressed and only Lcp3 is expressed at a strongly reduced level. As a first step in understanding the evolutionary mechanism(s) transforming an autosome into a dosage compensated X we analysed the expression behaviour and promoter structure of the Lcp1-4 genes on the neo-X. The normalized relative expression levels reveal that all four…

MaleMolecular Sequence DataGenes InsectDrosophila pseudoobscuraGenes Y-LinkedGenes X-LinkedDosage Compensation GeneticSequence Homology Nucleic AcidGene clusterGeneticsAnimalsDrosophila ProteinsPromoter Regions GeneticMolecular BiologyGeneX chromosomeDrosophila persimilisGeneticsDosage compensationAutosomebiologyBase Sequencefungibiology.organism_classificationEvolutionary biologyInsect ScienceMultigene FamilyDrosophilaFemaleDrosophila ProteinInsect molecular biology
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Transposition of minisatellite-like DNA in Chironomus midges.

1994

Cla elements are a family of tandem repetitive DNA sequences present in the genome of several Chironomus species. Interspersed clusters of Cla elements are widely distributed all over the chromosomes in C. thummi thummi, while they seem to be limited to the centromeric regions in the closely related subspecies C. t. piger. Here we present molecular evidence that this differential distribution is due to a transposition of Cla elements during evolution of the C. t. thummi genome. We have cloned a "filled" integration site (containing a Cla element cluster) from C. t. thummi and the corresponding "empty" genomic site from C. t. piger and other related species. The comparison shows that tandem…

MaleMolecular Sequence DataMolecular evidenceGenes InsectSubspeciesDNA SatelliteGenomeChironomidaeTransposition (music)chemistry.chemical_compoundSpecies SpecificitySequence Homology Nucleic AcidGeneticsAnimalsCloning MolecularRepeated sequenceMolecular BiologyIn Situ HybridizationRepetitive Sequences Nucleic AcidGeneticsintegumentary systembiologyBase Sequencefood and beveragesGeneral Medicinebiology.organism_classificationMinisatellitechemistryDNA Transposable Elementslipids (amino acids peptides and proteins)ChironomusFemaleDNABiotechnologyGenome
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