Search results for " and neonatal diseases and abnormalities"

showing 10 items of 344 documents

Effects of chemically modified tetracyclines (CMTs) in sensitive, multidrug resistant and apoptosis resistant leukaemia cell lines

2001

Recently discovered chemically modified tetracyclines (CMTs) have shown in vitro and in vivo anti-proliferative and anti-tumour activities. Here, we evaluated in vitro the anti-proliferative and apoptotic activity of six different dedimethylamino chemically modified tetracyclines (CMT-1, CMT-3, CMT-5, CMT-6, CMT-7 and CMT-8) in sensitive and multidrug resistant myeloid leukaemia cells (HL60 and HL60R) in vitro. Three of these compounds (CMT-5, CMT-6, CMT-7) showed low cytotoxic activity both in sensitive and in resistant cells, CMT-3 was endowed with a high anti-proliferative activity only in sensitive cells and was moderately effective as apoptosis inducing agent, with an activity similar …

Pharmacologycongenital hereditary and neonatal diseases and abnormalitiesProgrammed cell deathbiologyBiological activitynervous system diseasesMultiple drug resistanceBiochemistryCell cultureApoptosisCancer researchbiology.proteinCytotoxic T cellCaspaseAntibacterial agentBritish Journal of Pharmacology
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Brugada phenocopy in diabetic ketoacidosis, the importance of the diagnostic approach

2020

Abstract Brugada phenocopies (BrP) are clinical entities that present with identical ECG patterns to those of true Brugada Syndrome (BrS) but are elicited by various other clinical circumstances. Our manuscript shows an interesting case of a type-1 Class A BrP in a young patient with diabetic ketoacidosis and hyperkalemia.

Phenocopycongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyHyperkalemiaDiabetic ketoacidosisbusiness.industry030204 cardiovascular system & hematologymedicine.disease03 medical and health sciences0302 clinical medicineBrugada phenocopy Brugada syndrome Diabetic ketoacidosis Differential diagnosis Hyperkalemia Treatment030225 pediatricsInternal medicinePediatrics Perinatology and Child HealthCardiologyMedicinecardiovascular diseasesmedicine.symptomCardiology and Cardiovascular MedicinebusinessBrugada syndrome
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Forward-central two-particle correlations in p–Pb collisions at sNN=5.02 TeV

2016

Two-particle angular correlations between trigger particles in the forward pseudorapidity range (2.5 < |η| < 4.0) and associated particles in the cen

Physicscongenital hereditary and neonatal diseases and abnormalitiesNuclear and High Energy PhysicsParticle physicsTime projection chamber010308 nuclear & particles physicsAtlas detector01 natural sciencesCharged particleNuclear physicsMuon spectrometerPseudorapidity0103 physical sciencesTransverse momentumRapidityMultiplicity (chemistry)010306 general physicsPhysics Letters B
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Multiple Pluripotent Stem Cell Markers in Human Anaplastic Thyroid Cancer: The Putative Upstream Role of SOX2

2013

Anaplastic thyroid carcinoma (ATC) is a rare and aggressive endocrine tumor with highly undifferentiated morphology. It has been suggested that cancer stem cells (CSCs) might play a central role in ATC. The objectives of this study were (i) to characterize CSCs from ex vivo ATC specimens by investigating the expression of several pluripotent stem cell markers, and (ii) to evaluate in vitro drug resistance modifications after specific CSC transcription factor switch-off.In ex vivo experiments, eight formalin-fixed, paraffin-embedded ATC specimens were analyzed by reverse-transcription and real-time quantitative PCR and immunohistochemistry. In in vitro experiments using ATC SW1736 cells, the…

Pluripotent Stem Cellscongenital hereditary and neonatal diseases and abnormalitiesEndocrinology Diabetes and MetabolismDown-RegulationBiologyThyroid Carcinoma AnaplasticAnaplastic thyroid cancer cancer stem cell SOX-2Settore MED/13 - EndocrinologiaThyroid carcinomaKruppel-Like Factor 4EndocrinologySOX2Cancer stem cellCell Line TumormedicineATP Binding Cassette Transporter Subfamily G Member 2HumansNeoplasmThyroid NeoplasmsAnaplastic thyroid cancerInduced pluripotent stem cellHomeodomain ProteinsSOXB1 Transcription FactorsNanog Homeobox ProteinNanog Homeobox ProteinThyroid Cancer and Nodulesmedicine.diseaseNeoplasm ProteinsUp-RegulationDoxorubicinDrug Resistance NeoplasmCell cultureNeoplastic Stem CellsCancer researchATP-Binding Cassette TransportersCisplatinOctamer Transcription Factor-3Thyroid
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Microplastics in the global aquatic environment: Analysis, effects, remediation and policy solutions

2019

This opinion paper reports on Microplastics (MPs) pollution in the aquatic environment. MPs are a global problem being detected everywhere: marine environment, wastewaters, surface waters, soils, sediments, food and air. MPs can also absorb organic contaminants, and can be ingested by organisms and introduced into the food web. MPs can be a vector as well of pathogens whereas airborne fibrous MPs may enter our respiratory system with risk to the environment and humans. Main issues and gaps related to MPs on-going and future research are highlighted: chemical analysis, fate in wastewater and drinking water treatment plants, environmental and human health effects as well as remediation strate…

Pollutioncongenital hereditary and neonatal diseases and abnormalitiesMicroplasticsEnvironmental remediationMicroplasticsmedia_common.quotation_subjectRemediation and policy02 engineering and technology010501 environmental sciences01 natural sciencesEnvironmental protectionChemical Engineering (miscellaneous)Chemical analysisskin and connective tissue diseasesWater treatment plantsWaste Management and Disposal0105 earth and related environmental sciencesmedia_commonProcess Chemistry and Technologyfunginutritional and metabolic diseasesContamination021001 nanoscience & nanotechnologyPollutionFood webHuman health effectsWastewaterRisk to the environmentAquatic environmentEnvironmental scienceWater treatment0210 nano-technologyJournal of Environmental Chemical Engineering
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Glutamine Codon Usage and polyQ Evolution in Primates Depend on the Q Stretch Length

2018

Abstract Amino acid usage in a proteome depends mostly on its taxonomy, as it does the codon usage in transcriptomes. Here, we explore the level of variation in the codon usage of a specific amino acid, glutamine, in relation to the number of consecutive glutamine residues. We show that CAG triplets are consistently more abundant in short glutamine homorepeats (polyQ, four to eight residues) than in shorter glutamine stretches (one to three residues), leading to the evolutionary growth of the repeat region in a CAG-dependent manner. The length of orthologous polyQ regions is mostly stable in primates, particularly the short ones. Interestingly, given a short polyQ the CAG usage is higher in…

Primatescongenital hereditary and neonatal diseases and abnormalitiescodon usageProteomeGlutaminehomorepeatEvolution MolecularAnimalsHumansglutamine stretchCodonPeptidespolyQ-associated diseasesResearch ArticleGenome Biology and Evolution
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Pharmacological Induction of Redundant Genes for a Therapy of X-ALD

2003

X-linked adrenoleukodystrophy (X-ALD) is a recessive neurologic disease with an incidence among males of 1/17 000. Since the identification of the X-ALD gene (ABCD1) ten years ago (Mosser et al 1993), no satisfactory therapy has been available. A close homologue (ABCD2) was then cloned and presented as a putative modifier gene that could account for some of the extreme phenotypic variability of X-ALD (Lombard-Platet et al 1996). The inducibility of Abcd2 by the hypolipidemic drug fenofibrate in the liver of rodents (Albet et al 1997), correlated to a partial normalisation of the biochemical phenotype of X-ALD (Netik et al 1999), opened up the way of a pharmacological therapy of X-ALD. The b…

Pristanic acidcongenital hereditary and neonatal diseases and abnormalitiesFenofibrateendocrine system diseasesPharmacological therapybiologyPharmacologymedicine.diseasePhenylbutyrateBiochemical phenotypechemistry.chemical_compoundchemistrymedicineABCD2biology.proteinAdrenoleukodystrophyGenemedicine.drug
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The impact of gender and age at onset on the familial aggregation of schizophrenia.

1993

Some recent family studies have shown that the familial risk for schizophrenia is higher in female than in male schizophrenics. It is debated whether the risks for the other disorders, such as schizotypal personality disorder or affective disorders in families of schizophrenics are similarly influenced by the proband's gender. Also, the reason for the effect of proband's gender on the recurrence risk for schizophrenia has not been clarified. This family study (159 probands, 589 first degree relatives) confirms that schizophrenia, but also schizophrenia spectrum disorders were more frequent in families of female compared with male schizophrenics. Neither age at onset in probands nor the inte…

ProbandAdultMalecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyPsychosisComorbidityPersonality DisordersSex Factorsmental disordersmedicineHumansPharmacology (medical)Spectrum disorderFamilyRisk factorFirst-degree relativesPsychiatryBiological PsychiatryAgedPsychiatric Status Rating ScalesMood DisordersAge FactorsFamily aggregationGeneral MedicineMiddle Agedmedicine.diseaseSchizotypal personality disorderPsychiatry and Mental healthSchizophreniaSchizophreniaFemalePsychologyEuropean archives of psychiatry and clinical neuroscience
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BBS1 Mutations in a Wide Spectrum of Phenotypes Ranging From Nonsyndromic Retinitis Pigmentosa to Bardet-Biedl Syndrome

2012

OBJECTIVE: To investigate the involvement of the Bardet-Biedl syndrome (BBS) gene BBS1 p.M390R variant in nonsyndromic autosomal recessive retinitis pigmentosa (RP). METHODS: Homozygosity mapping of a patient with isolated RP was followed by BBS1 sequence analysis. We performed restriction fragment length polymorphism analysis of the p.M390R allele in 2007 patients with isolated RP or autosomal recessive RP and in 1824 ethnically matched controls. Patients with 2 BBS1 variants underwent extensive clinical and ophthalmologic assessment. RESULTS: In an RP proband who did not fulfill the clinical criteria for BBS, we identified a large homozygous region encompassing the BBS1 gene, which carrie…

ProbandMaleBBS1Genetics and epigenetic pathways of disease [NCMLS 6]DNA Mutational AnalysisEvaluation of complex medical interventions Genomic disorders and inherited multi-system disorders [NCEBP 2]GenotypeEthnicityPrevalenceIsraelGeneticseducation.field_of_studyRetinitis Pigmentosa/diagnosisMiddle AgedDisease gene identificationPedigreeEuropePhenotypeFemaleMicrotubule-Associated ProteinsRetinitis PigmentosaAdultcongenital hereditary and neonatal diseases and abnormalitiesCanadaPopulationCanada/epidemiologyMicroscopy AcousticMutation MissenseEthnic GroupsDNA/geneticsBiologyEurope/epidemiologyGenomic disorders and inherited multi-system disorders [IGMD 3]Bardet-Biedl Syndrome/diagnosisBardet–Biedl syndromeRetinitis pigmentosamedicineElectroretinographyHumansAlleleeducationBardet-Biedl SyndromeIsrael/epidemiologyAllelesDNAMicrotubule-Associated Proteins/geneticsmedicine.diseaseOphthalmoscopyOphthalmologyGenetics and epigenetic pathways of disease Genomic disorders and inherited multi-system disorders [NCMLS 6]
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Contribution of molecular analyses in diagnosing Marfan syndrome and type I fibrillinopathies: an international study of 1009 probands.

2008

International audience; BACKGROUND: The diagnosis of Marfan syndrome (MFS) is usually initially based on clinical criteria according to the number of major and minor systems affected following international nosology. The number of FBN1 mutation carriers, at risk of aortic complications who would not be properly diagnosed based only on clinical grounds, is of growing importance owing to the increased availability of molecular screening. The aim of the study was to identify patients who should be considered for FBN1 mutation screening. METHODS: Our international series included 1009 probands with a known FBN1 mutation. Patients were classified as either fulfilling or not fulfilling "clinical"…

ProbandNosologyMarfan syndromeMalePediatricsSystemic diseaseMESH : International CooperationFibrillin-1International CooperationMESH : Aged[SDV.GEN] Life Sciences [q-bio]/GeneticsMarfan SyndromeMESH : ChildMESH: ChildEpidemiologyMESH : FemaleEctopia lentisChildGenetics (clinical)AortaAortic dissectionMESH: Aged0303 health sciences030305 genetics & heredityMicrofilament ProteinsMESH: AortaMESH : AdultConnective tissue disease3. Good healthFemaleMESH : Mutationmusculoskeletal diseasesAdultmedicine.medical_specialtycongenital hereditary and neonatal diseases and abnormalitiesMESH: MutationMESH : Microfilament ProteinsAdolescentMESH : MaleFibrillinsMESH: Marfan Syndrome03 medical and health sciencesMESH: Microfilament ProteinsMESH : AdolescentGeneticsmedicineHumans030304 developmental biologyAgedMESH: Adolescent[SDV.GEN]Life Sciences [q-bio]/GeneticsMESH : Marfan SyndromeMESH: Humansbusiness.industryMESH : HumansMESH : AortaMESH: Adultmedicine.diseaseMESH: MaleMESH: International CooperationMutation[ SDV.GEN ] Life Sciences [q-bio]/GeneticsbusinessMESH: FemaleJournal of medical genetics
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