Search results for "binding"

showing 10 items of 3896 documents

A two base pair deletion in the PQBP1 gene is associated with microphthalmia, microcephaly, and mental retardation.

2007

X-linked mental retardation has been traditionally divided into syndromic (S-XLMR) and non-syndromic forms (NS-XLMR), although the borderlines between these phenotypes begin to vanish and mutations in a single gene, for example PQBP1, can cause S-XLMR as well as NS-XLMR. Here, we report two maternal cousins with an apparently X-linked phenotype of mental retardation (MR), microphthalmia, choroid coloboma, microcephaly, renal hypoplasia, and spastic paraplegia. By multipoint linkage analysis with markers spanning the entire X-chromosome we mapped the disease locus to a 28-Mb interval between Xp11.4 and Xq12, including the BCOR gene. A missense mutation in BCOR was described in a family with …

AdultMaleMicrocephalycongenital hereditary and neonatal diseases and abnormalitiesGermline mosaicismLocus (genetics)BiologyMicrophthalmiaFrameshift mutationGenetic linkageGenes X-LinkedIntellectual DisabilityGeneticsmedicineMissense mutationHumansMicrophthalmosAbnormalities MultipleFrameshift MutationGenetics (clinical)GeneticsChromosomes Human XNuclear ProteinsGenetic Diseases X-LinkedSyndromemedicine.diseasePedigreeLenz microphthalmia syndromeDNA-Binding ProteinsChild PreschoolMicrocephalyFemaleCarrier ProteinsGene DeletionEuropean journal of human genetics : EJHG
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Epigenetic modifications precede molecular alterations and drive human hepatocarcinogenesis

2021

Development of primary liver cancer is a multistage process. Detailed understanding of sequential epigenetic alterations is largely missing. Here, we performed Infinium Human Methylation 450k BeadChips and RNA-Seq analyses for genome-wide methylome and transcriptome profiling of cirrhotic liver (n = 7), low- (n = 4) and high-grade (n = 9) dysplastic lesions, and early (n = 5) and progressed (n = 3) hepatocellular carcinomas (HCC) synchronously detected in 8 patients with HCC with chronic hepatitis B infection. Integrative analyses of epigenetically driven molecular changes were identified and validated in 2 independent cohorts comprising 887 HCCs. Mitochondrial DNA sequencing was further em…

AdultMaleMitochondrial DNACarcinoma HepatocellularCirrhosisMolecular biologyCarcinogenesisBiologyEpigenesis GeneticHepatitis B ChronicmedicineHumansEpigeneticsAgedHepatologyGene Expression ProfilingLiver NeoplasmsDNA NeoplasmGeneral MedicineMethylationDNA MethylationMiddle AgedHCCSmedicine.diseaseGene Expression Regulation NeoplasticOncologyApoptosisDNA methylationCancer researchEpigeneticsCalmodulin-Binding ProteinsFemaleLiver cancerLiver cancerResearch ArticleJCI Insight
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Cloning of the human NCNF gene.

1998

We have cloned from a cDNA library of human testis tissue the human homologue to the mouse nuclear orphan receptor NCNF (neuronal cell nuclear factor). The open reading frame encodes a protein of 480 amino acids, the sequence of which (EMBL accession no. X99975) is 98.3% identical to the mouse homologue. Northern blot analysis of adult human tissues revealed a broad pattern of tissue expression. Similar to NCNF expression in mouse testis, two transcript forms of the single copy gene are expressed in human tissues. The two transcript forms which differ only in their 3'UTR, result in human from differential polyadenylation, in mouse from alternative splicing. Based on the high level of sequen…

AdultMaleMolecular Sequence DataReceptors Cytoplasmic and NuclearBiologyBiochemistryMiceNuclear Receptor Subfamily 6 Group A Member 1Sequence Homology Nucleic AcidTestisAnimalsHumansNorthern blotAmino Acid SequenceCloning MolecularMolecular BiologyPeptide sequenceGeneCloningOrphan receptorRegulation of gene expressionBase SequencecDNA libraryAlternative splicingCell BiologyDNAMolecular biologyDNA-Binding ProteinsRepressor ProteinsAlternative SplicingGene Expression RegulationOrgan SpecificityJournal of receptor and signal transduction research
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Differential expression of specific microRNA and their targets in acute myeloid leukemia

2010

Acute myeloid leukemia (AML) the most common acute leukemia in adults is characterized by various cytogenetic and molecular abnormalities. However, the genetic etiology of the disease is not yet fully understood. MicroRNAs (miRNA) are small noncoding RNAs which regulate the expression of target mRNAs both at transcriptional and translational level. In recent years, miRNAs have been identified as a novel mechanism in gene regulation, which show variable expression during myeloid differentiation. We studied miRNA expression of leukemic blasts of 29 cases of newly diagnosed and genetically defined AML using quantitative reverse transcription polymerase chain reaction (RT-PCR) for 365 human miR…

AdultMaleNPM1Down-RegulationBiologySettore MED/15 - Malattie Del SangueYoung Adulthemic and lymphatic diseasesmicroRNAmedicineGene silencingHumansLeukemia microarray data microRNAGranulocyte Precursor CellsAgedCell ProliferationGeneticsRegulation of gene expressionAged 80 and overAcute leukemiaReverse Transcriptase Polymerase Chain ReactionGene Expression ProfilingCore Binding FactorsMyeloid leukemiaNuclear ProteinsCell DifferentiationHematologyMiddle Agedmedicine.diseaseUp-RegulationGene expression profilingGene Expression Regulation NeoplasticLeukemiaLeukemia Myeloid AcuteMicroRNAsfms-Like Tyrosine Kinase 3Case-Control StudiesMutationFemaleSettore SECS-S/01 - StatisticaNucleophosmin
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Ribonucleotide Reductase Messenger RNA Expression and Survival in Gemcitabine/Cisplatin-Treated Advanced Non-Small Cell Lung Cancer Patients

2004

Abstract Purpose: No chemotherapy regimen, including the widely used combination of gemcitabine/cisplatin, confers significantly improved survival over any other in metastatic non-small cell lung cancer (NSCLC); however, the selection of patients according to key genetic characteristics can help to tailor chemotherapy. Ribonucleotide reductase subunit M1 (RRM1) is involved in DNA synthesis and repair and in gemcitabine metabolism, and the excision repair cross-complementing group 1 (ERCC1) gene has been related to cisplatin activity. Experimental Design: Patients were part of a large randomized trial carried out from September 1998 to July 2000, comparing gemcitabine/cisplatin versus gemcit…

AdultMaleOncologyAntimetabolites AntineoplasticCancer Researchmedicine.medical_specialtyPathologyLung NeoplasmsTime FactorsDNA RepairRibonucleoside Diphosphate Reductasemedicine.medical_treatmentAntineoplastic AgentsBiologyVinorelbineDeoxycytidineCarcinoma Non-Small-Cell LungInternal medicineRibonucleotide ReductasesmedicineHumansRNA MessengerLung cancerAgedCisplatinChemotherapyPredictive markerTumor Suppressor ProteinsDNAMiddle AgedEndonucleasesPrognosismedicine.diseaseGemcitabineChemotherapy regimenGemcitabineDNA-Binding ProteinsTreatment OutcomeOncologyFemaleCisplatinERCC1medicine.drugClinical Cancer Research
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Bcl-6 mutation status provides clinically valuable information in early-stage B-cell chronic lymphocytic leukemia

2004

In B-cell chronic lymphocytic leukemia (B-CLL), somatic mutation of IgVH genes defines a subgroup with favorable prognosis, whereas the absence of IgVH mutations is correlated with a worse outcome. Mutations of the BCL-6 gene are also observed in a subset of B-CLL, but the clinical significance of this molecular alteration remains uncertain. We examined the distribution of IgVH and BCL-6 gene mutations in 95 well-characterized patients with Binet stage A B-CLL, and correlated them with clinical, laboratory, cytogenetic findings and disease progression. Mutations of the BCL-6 gene were observed only in cases harboring mutated IgVH. Unexpectedly, coexistence of IgVH and BCL-6 mutations was co…

AdultMaleOncologyCancer Researchmedicine.medical_specialtyChronic lymphocytic leukemiaImmunoglobulin Variable RegionLocus (genetics)BiologyGene mutationDisease-Free SurvivalGermline mutationProto-Oncogene ProteinsInternal medicinemedicineHumansB-cell chronic lymphocytic leukemiaClinical significanceProspective StudiesGeneAgedAged 80 and overHematologyChromosomes Human Pair 11HematologyMiddle AgedPrognosismedicine.diseaseLeukemia Lymphocytic Chronic B-CellDNA-Binding ProteinsOncologyMutationImmunologyProto-Oncogene Proteins c-bcl-6FemaleChromosome DeletionChromosomes Human Pair 17Follow-Up StudiesTranscription FactorsLeukemia
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Influence of ABCB1 polymorphisms upon the effectiveness of standard treatment for acute myeloid leukemia: A systematic review and meta-analysis of ob…

2015

The ABCB1 gene encodes for P-glycoprotein (P-gp), an efflux pump for a variety of xenobiotics. The role of ABCB1 polymorphisms in acute myeloid leukemia (AML) outcomes of standard chemotherapy (cytarabine plus anthracyclines) remains controversial. A systematic search was made of studies evaluating the association between ABCB1 polymorphisms 1236C>T, 2677G>T/A and 3435C>T and effectiveness variables. We found seven cohort studies (1241 patients) showing a significantly higher overall survival (OS) among carriers of the variant allele of 1236C>T at year 4 (odds ratio (OR): 1.47, 95% confidence interval (CI): 1.07-2.01), 2677G>T/A at years 4-5 (OR: 1.37, 95% CI: 1.01-1.86) and 3435C>T at year…

AdultMaleOncologymedicine.medical_specialtyATP Binding Cassette Transporter Subfamily BAdolescentSubgroup analysisCohort StudiesYoung AdultInternal medicineGeneticsmedicineHumansAnthracyclinesChildAgedAged 80 and overPharmacologyGeneticsPolymorphism Geneticbusiness.industryStandard treatmentCytarabineInfantMyeloid leukemiaOdds ratioMiddle AgedConfidence intervalLeukemia Myeloid AcuteObservational Studies as TopicTreatment OutcomeChild PreschoolMeta-analysisCytarabineMolecular MedicineFemalebusinessmedicine.drugCohort studyThe Pharmacogenomics Journal
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Lack of efficacy of mitoxantrone in primary progressive Multiple Sclerosis irrespective of pharmacogenetic factors: A multi-center, retrospective ana…

2014

Abstract Background Mitoxantrone is used on an off-label basis in primary progressive MS (PPMS). ABC -transporter-genotypes are associated with therapeutic response in relapsing/secondary progressive MS (RP/SPMS). Objective To evaluate potential pharmacogenetic response markers for mitoxantrone in PPMS. Methods 41 mitoxantrone-treated PPMS-patients, 155 mitoxantrone-treated RP/SPMS-patients and 43 PPMS-controls were retrospectively assessed for clinical therapy-response and in correlation with four single-nucleotide-polymorphisms in ABCB1 - and ABCG2 -genes. Results 53.7% PPMS-patients were mitoxantrone-responders, in comparison to 78.1% of RP/SPMS-patients (p = 0.039). There was no associa…

AdultMaleOncologymedicine.medical_specialtyTreatment responseATP Binding Cassette Transporter Subfamily Bmedicine.medical_treatmentImmunologyPrimary Progressive Multiple SclerosisPharmacologyInternal medicineGenotypeLack of efficacymedicineRetrospective analysisATP Binding Cassette Transporter Subfamily G Member 2HumansImmunology and AllergyRetrospective StudiesAnalgesicsMitoxantronebusiness.industryImmunosuppressionMiddle AgedMultiple Sclerosis Chronic ProgressiveNeoplasm Proteins3. Good healthNeurologyPharmacogeneticsATP-Binding Cassette TransportersFemaleNeurology (clinical)MitoxantronebusinessPharmacogeneticsmedicine.drugJournal of Neuroimmunology
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Basal opioid receptor binding is associated with differences in sensory perception in healthy human subjects: a [18F]diprenorphine PET study.

2009

The endogenous opioid system is involved in many body functions including pain processing and analgesia. To determine the role of basal opioid receptor availability in the brain in pain perception, twenty-three healthy subjects underwent positron emission tomography (PET) utilizing the subtype-nonselective opioid antagonist [(18)F]diprenorphine, quantitative sensory testing (QST) and the cold pressor test. Binding potentials (BPs) were calculated using a non-invasive reference tissue model and statistical parametric mapping was applied for t-statistical analysis on a voxelwise basis. We found that cold pain-sensitive subjects present a significantly lower BP in regions including the bilater…

AdultMalePain Thresholdmedicine.drug_classCognitive NeuroscienceSensationDiprenorphinePainInsular cortexYoung AdultOpioid receptorOpioid Receptor BindingPhysical StimulationmedicinePressureHumansEndogenous opioidBrain ChemistryBrainSomatosensory CortexMiddle AgedCold TemperatureNeurologyOpioidData Interpretation StatisticalPositron-Emission TomographySensory ThresholdsReceptors OpioidOrbitofrontal cortexPerceptionRadiopharmaceuticalsPsychologyDiprenorphineNeuroscienceOpioid antagonistmedicine.drugNeuroImage
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Central opioidergic neurotransmission in complex regional pain syndrome

2010

Objective: Complex regional pain syndrome (CRPS) is a chronic pain condition characterized by sensory, motor, and autonomic symptoms. It develops after limb trauma and may be associated with relevant psychiatric comorbidity. As there is evidence for central pathophysiology which might be related to an altered opioidergic neurotransmission, we investigated the cerebral opioid receptor status under resting conditions in this patient population.Methods: In this case-control study, 10 patients with CRPS and 10 age- and gender-matched healthy subjects underwent a PET scan using the subtype-nonselective opioidergic radioligand [18F]fluoroethyl-diprenorphine. As a surrogate for regional cerebral o…

AdultMalePain Thresholdmedicine.drug_classPainAnxietyNeuropsychological TestsSynaptic TransmissionAmygdalaOpioid receptorOpioid Receptor BindingImage Processing Computer-AssistedmedicineHumansRadionuclide ImagingPain MeasurementNeuronsTemporal cortexOpioidergicBrain MappingChronic painBrainMiddle Agedmedicine.diseaseAffectComplex regional pain syndromemedicine.anatomical_structureCase-Control StudiesAnesthesiaReceptors OpioidAnxietyFemaleNeurology (clinical)medicine.symptomPsychologyComplex Regional Pain SyndromesNeurology
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