Search results for "Glycosyl"

showing 10 items of 317 documents

High Rate of Recurrent De Novo Mutations in Developmental and Epileptic Encephalopathies

2017

Item does not contain fulltext Developmental and epileptic encephalopathy (DEE) is a group of conditions characterized by the co-occurrence of epilepsy and intellectual disability (ID), typically with developmental plateauing or regression associated with frequent epileptiform activity. The cause of DEE remains unknown in the majority of cases. We performed whole-genome sequencing (WGS) in 197 individuals with unexplained DEE and pharmaco-resistant seizures and in their unaffected parents. We focused our attention on de novo mutations (DNMs) and identified candidate genes containing such variants. We sought to identify additional subjects with DNMs in these genes by performing targeted sequ…

Male0301 basic medicineCandidate genemedicine.medical_specialtymedical geneticsglycosylationNonsense mutationGenome-wide association studyGene mutationBiologySensory disorders Donders Center for Medical Neuroscience [Radboudumc 12]Articlesevere intellectual disability03 medical and health sciencesEpilepsy0302 clinical medicinechildrenRecurrenceSeizuresGenetic linkageIntellectual Disability[ SDV.MHEP ] Life Sciences [q-bio]/Human health and pathologyJournal ArticleGeneticsmedicineHumansChilddisordersGenetics (clinical)Genetic associationGeneticsBrain DiseasesdiseaseEpilepsycis-prenyltransferaseGenome Humanstructural basismedicine.diseasediphosphate synthase030104 developmental biologyChild PreschoolMutationMedical geneticsFemalenogo-b receptor030217 neurology & neurosurgery[SDV.MHEP]Life Sciences [q-bio]/Human health and pathologyGenome-Wide Association StudyMeta-Analysis
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The possible association of clusterin fucosylation changes with male fertility disorders

2021

AbstractIn the seminal plasma (n = 118) and serum (n = 90) clusterin (CLU) the fucosylation and the expression of selected fucosyltransferases (FUTs) were analyzed. Samples from infertile men were divided into groups based on the results of the standard semen analysis: normozoospermic (N), teratozoospermic (T), asthenoteratozoospermic (AT) and oligoasthenoteratozoospermic (OAT). The CLU fucosylation was analyzed using lectin-ELISAs with biotinylated lectins specific to α1,3-, α1,2-linked antennary fucose, and α1,6-linked core fucose (LTA, UEA, and LCA, respectively). The concentrations of FUT3 and FUT4, reflecting the expression of Le oligosaccharide structures, were measured using ELISA te…

Male0301 basic medicineGlycosylationMolecular biologyScienceDiseasesBiologySemen analysisArticleFucoseMale infertilityAndrologyFucosyltransferases03 medical and health scienceschemistry.chemical_compound0302 clinical medicineBlood serumPolysaccharidesmedicineHumansInfertility MaleFucosylation030219 obstetrics & reproductive medicineMultidisciplinaryClusterinmedicine.diagnostic_testQRmedicine.diseaseSpermClusterin030104 developmental biologychemistrybiology.proteinMedicineBiomarkersScientific Reports
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Diagnostic strategy in segmentation defect of the vertebrae: a retrospective study of 73 patients

2018

BackgroundSegmentation defects of the vertebrae (SDV) are non-specific features found in various syndromes. The molecular bases of SDV are not fully elucidated due to the wide range of phenotypes and classification issues. The genes involved are in the Notch signalling pathway, which is a key system in somitogenesis. Here we report on mutations identified in a diagnosis cohort of SDV. We focused on spondylocostal dysostosis (SCD) and the phenotype of these patients in order to establish a diagnostic strategy when confronted with SDV.Patients and methodsWe used DNA samples from a cohort of 73 patients and performed targeted sequencing of the five known SCD-causing genes (DLL3,MESP2,LFNG,HES7…

Male0301 basic medicineOncologymedicine.medical_specialtyCandidate geneAdolescent030105 genetics & heredityspondylocostal dysostosisdiagnostic strategysegmentation defect of the vertebraewhole exome sequencingLFNG03 medical and health sciencesgene panelInternal medicineExome SequencingBasic Helix-Loop-Helix Transcription FactorsGeneticsmedicineHumansFLNBChildGenetics (clinical)Exome sequencingBone Diseases Developmentalbusiness.industryIntracellular Signaling Peptides and ProteinsGlycosyltransferasesInfantMembrane ProteinsRetrospective cohort studymedicine.diseasePhenotypeSpineSpondylocostal dysostosisPedigreePhenotype[SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human geneticsChild PreschoolMutationCohortFemaleT-Box Domain Proteinsbusiness
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Stable Oxidative Cytosine Modifications Accumulate in Cardiac Mesenchymal Cells From Type2 Diabetes Patients

2018

Rationale: Human cardiac mesenchymal cells (CMSCs) are a therapeutically relevant primary cell population. Diabetes mellitus compromises CMSC function as consequence of metabolic alterations and incorporation of stable epigenetic changes. Objective: To investigate the role of α-ketoglutarate (αKG) in the epimetabolic control of DNA demethylation in CMSCs. Methods and Results: Quantitative global analysis, methylated and hydroxymethylated DNA sequencing, and gene-specific GC methylation detection revealed an accumulation of 5-methylcytosine, 5-hydroxymethylcytosine, and 5-formylcytosine in the genomic DNA of human CMSCs isolated from diabetic donors. Whole heart genomic DNA analysis reveale…

Male0301 basic medicinePhysiologyPopulationheartBiologyMixed Function OxygenasesCytosineMice03 medical and health sciencesProto-Oncogene ProteinsfibroblastsHuman Umbilical Vein Endothelial CellsAnimalsHumansMyocytes CardiacEpigeneticsEnzyme InhibitorseducationCells CulturedEpigenomicsDemethylationeducation.field_of_studyDNA methylationDNA methylation; epigenomics; fibroblasts; heart; hyperglycemia; metabolism; physiology; cardiology and cardiovascular medicineMesenchymal Stem CellsSettore MED/13 - ENDOCRINOLOGIABase excision repairMolecular biologyThymine DNA GlycosylaseMice Inbred C57BLHEK293 Cells030104 developmental biologyDNA demethylationDiabetes Mellitus Type 2epigenomicsDNA methylationKetoglutaric AcidshyperglycemiaThymine-DNA glycosylaseCardiology and Cardiovascular MedicineOxidation-ReductionmetabolismCirculation Research
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Constitutive expression and inducibility of O6-methylguanine-DNA methyltransferase and N-methylpurine-DNA glycosylase in rat liver cells exhibiting d…

1995

AbstractWe have analyzed the expression of the DNA repair genes O6-methylguanine-DNA methyltransferase (MGMT) and N-methylpurine-DNA glycosylase (MPG) at RNA and protein activity level in primary rat hepatocytes in vitro and various rat hepatoma cell lines exhibiting different status of differentiation. The basal level of MGMT mRNA and activity correlated well with the degree of differentiation, as measured by tyrosine aminotransferase (TAT) mRNA expression. Induction of MGMT mRNA and protein activity by X-ray and Nmethyl-N′-nitro-N-nitrosoguanidine (MNNG) treatment was most pronounced in the well-differentiated hepatocytes and in various differentiated hepatoma cell lines (up to 6-fold). T…

MaleAlkylating AgentsMethyltransferaseDNA repairDNA repairBiology(Rat)DNA methyltransferaseCell LineDNA GlycosylasesRats Sprague-DawleyO(6)-Methylguanine-DNA MethyltransferaseTyrosine aminotransferaseGene expressionAnimalsHepatocyteRNA MessengerN-Glycosyl HydrolasesMolecular BiologyneoplasmsMessenger RNACell DifferentiationMethyltransferasesMolecular biologydigestive system diseasesRatsPerfusionLiverCell cultureDNA glycosylaseEnzyme InductionMolecular MedicineGene expressionMGMTMPGBiochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
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Odorant metabolism catalyzed by olfactory mucosal enzymes influences peripheral olfactory responses in rats.

2013

International audience; A large set of xenobiotic-metabolizing enzymes (XMEs), such as the cytochrome P450 monooxygenases (CYPs), esterases and transferases, are highly expressed in mammalian olfactory mucosa (OM). These enzymes are known to catalyze the biotransformation of exogenous compounds to facilitate elimination. However, the functions of these enzymes in the olfactory epithelium are not clearly understood. In addition to protecting against inhaled toxic compounds, these enzymes could also metabolize odorant molecules, and thus modify their stimulating properties or inactivate them. In the present study, we investigated the in vitro biotransformation of odorant molecules in the rat …

MaleAnatomy and Physiology[ SDV.AEN ] Life Sciences [q-bio]/Food and NutritionSensory PhysiologyEnzyme Metabolismlcsh:MedicineQuinolonesBiochemistryCarboxylesterasechemistry.chemical_compoundPentanols0302 clinical medicineCoumarinsEnzyme Inhibitorslcsh:Sciencechemistry.chemical_classification0303 health sciencesMultidisciplinaryEnzyme ClassesEsterasesSensory SystemsEnzymes3. Good healthElectrophysiologyProtein Transportmedicine.anatomical_structureBiochemistryMedicineSensory PerceptionMetabolic PathwaysResearch ArticleIsoamyl acetateBiologyNeurological SystemXenobiotics03 medical and health sciencesOlfactory mucosaOlfactory MucosaTransferasesmedicineAnimalsRats WistarBiology030304 developmental biologyOlfactory Systemlcsh:RGlycosyltransferasesCytochrome P450MonooxygenaseOlfactory PerceptionRatsMetabolismEnzymechemistryOdorantsBiocatalysisbiology.proteinlcsh:Q[SDV.AEN]Life Sciences [q-bio]/Food and NutritionOlfactory epithelium030217 neurology & neurosurgeryDrug metabolismNeuroscience
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Liraglutide improves carotid intima-media thickness in patients with type 2 diabetes and non-alcoholic fatty liver disease: An 8-month prospective pi…

2015

Objective: To explore the effects of the glucagon-like peptide-1 receptor analogue liraglutide on subclinical atherosclerosis in diabetic subjects with non-alcoholic fatty liver disease (NAFLD).Research design and methods: In this 8-month prospective study, 29 subjects with type 2 diabetes (T2DM) and NAFLD (16 men and 13 women, mean age: 61 ± 10 years) were matched for age and gender with 29 subjects with T2DM without NAFLD (16 men and 13 women, mean age: 61 ± 8 years). Liraglutide 0.6 mg/day for 2 weeks, followed by 1.2 mg/day, was given in addition to metformin.Main outcome measures: Anthropometric variables, glucometabolic parameters and carotid intima-media thickness (IMT) using B-mode …

MaleClinical BiochemistryPilot ProjectsType 2 diabetesCarotid Intima-Media ThicknessGastroenterologychemistry.chemical_compoundRisk FactorsDrug DiscoveryProspective StudiesProspective cohort studyliraglutideMedicine (all)Fatty liverLipidMiddle AgedLipidsHemoglobin AAtherosclerosiFemalemedicine.drugHumanmedicine.medical_specialtycarotid intima-media thickneInternal medicineDiabetes mellitusmedicineHumansHypoglycemic AgentsPilot ProjectAgedGlycated HemoglobinHemoglobin A GlycosylatedPharmacologytype 2 diabeteHypoglycemic AgentLiraglutidebusiness.industryRisk FactorDrug Discovery3003 Pharmaceutical Sciencenutritional and metabolic diseasesnon-alcoholic fatty liver diseaseAtherosclerosismedicine.diseaseProspective StudieEndocrinologychemistryIntima-media thicknessDiabetes Mellitus Type 2Glycated hemoglobinbusiness
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DNA repair activity of 8-oxoguanine DNA glycosylase 1 (OGG1) in human lymphocytes is not dependent on genetic polymorphism Ser326/Cys326.

2001

8-oxoguanine DNA glycosylase 1 (OGG1) is a DNA repair enzyme that excises 7,8-dihydro-8-oxoguanine (8oxoG) from DNA. Since 8oxoG is a highly mispairing lesion, decreased OGG1 expression level could lead to a higher background mutation frequency and could possibly increase the cancer risk of an individual under oxidative stress. In order to analyse the natural variation of OGG1, we measured the DNA repair activity in human lymphocytes of healthy individuals by means of an 8oxoG-containing oligonucleotide assay. The data obtained revealed a two fold interindividual variation of OGG1 activity in lymphocytes. There was no difference in OGG1 activity due to gender and smoking behaviour. Transcri…

MaleDNA RepairDNA damageDNA repairBiologyIn Vitro TechniquesToxicologyDNA-formamidopyrimidine glycosylasechemistry.chemical_compoundGene FrequencyMUTYHGeneticsHumansAmino Acid SequenceLymphocytesMolecular BiologyGeneN-Glycosyl HydrolasesAllelesDNA PrimersPolymorphism GeneticBase SequenceOligonucleotideReverse Transcriptase Polymerase Chain ReactionMolecular biologyIsoenzymeschemistryDNA-Formamidopyrimidine GlycosylaseDNA glycosylaseDNADNA DamageMutation research
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A discoidal lipoprotein from the coelomic fluid of the polychaete Nereis virens.

2005

A discoidal lipoprotein was isolated from the coelomic fluid of the polychaete, Nereis virens, by density gradient centrifugation. The lipoprotein was present in both sexes and moved as a uniform band in an agarose gel. The average diameter of the lipoprotein particles determined by electron microscopy was 42 nm with a thickness of 10 nm. SDS electrophoresis showed two apoprotein subunits with molecular masses of 247 and 85 kDa, respectively. In lectin blots, both apoproteins were reactive with Concanavalin A indicating the presence of N-glycans. The small subunit was also reactive with peanut lectin, indicating additional O-glycosylation. The total lipid content was 48% and consisted mainl…

MaleEmbryo NonmammalianGlycosylationPhysiologyLipoproteinsBiologyIn Vitro TechniquesBiochemistrychemistry.chemical_compoundPolysaccharidesAnimalsParticle SizeMolecular BiologyDifferential centrifugationMolecular massLectinPolychaetaLipidsThin-layer chromatographyBody FluidsMolecular WeightElectrophoresisProtein SubunitschemistryBiochemistryConcanavalin Abiology.proteinAgaroselipids (amino acids peptides and proteins)FemaleLipoproteinComparative biochemistry and physiology. Part B, Biochemistrymolecular biology
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Follistatin as potential therapeutic target in prostate cancer

2012

Follistatin is a single-chain glycosylated protein whose primary function consists in binding and neutralizing some members of the transforming growth factor-β superfamily such as activin and bone morphogenic proteins. Emerging evidence indicates that this molecule may also play a role in the malignant progression of several human tumors including prostate cancer. In particular, recent findings suggest that, in this tumor, follistatin may also contribute to the formation of bone metastasis through multiple mechanisms, some of which are not related to its specific activin or bone morphogenic proteins' inhibitory activity. This review provides insight into the most recent advances in understa…

MaleFollistatinendocrine systemCancer Researchmedicine.medical_specialtyanimal structuresGlycosylated proteinProstate cancerInternal medicinemedicineAnimalsHumansPharmacology (medical)Molecular Targeted TherapyActivin Bone metastasis.Cancer . Follistatin Prostate cancerbiologybusiness.industryProstatic NeoplasmsCancerBone metastasisSUPERFAMILYmedicine.diseaseEndocrinologyOncologyembryonic structuresDisease Progressionbiology.proteinCancer researchbusinesshormones hormone substitutes and hormone antagonistsFunction (biology)FollistatinTransforming growth factor
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