Search results for "DEFICIENCY"

showing 10 items of 1071 documents

The role of post-transcriptional modulators of metalloproteins in response to metal deficiencies

2021

Copper and iron proteins play a wide range of functions in living organisms. Metal assembly into metalloproteins is a complex process, where mismetalation is detrimental and energy-consuming to cells. Under metal deficiency, metal distribution is expected to reach a metalation ranking, prioritizing essential versus dispensable metalloproteins, while avoiding interferences with other metals and protecting metal-sensitive processes. In this review, we propose that posttranscriptional Modulators of Metalloprotein messenger RNA (ModMeR) are good candidates in metal prioritization under metal-limited conditions. ModMeR target high quota or redundant metalloproteins and, by adjusting their synthe…

Arabidopsis thalianaPhysiologyMetalationIronArabidopsischemistry.chemical_elementSaccharomyces cerevisiaePlant ScienceMetalMetalloproteinCth2MetalloproteinsMetalloproteinMetalationAnimalsArabidopsis thalianaIron deficiency (plant disorder)Mammalschemistry.chemical_classificationbiologyIron deficiencyIron DeficienciesCopper deficiencybiology.organism_classificationCopperCell biologyCu-miRNAsMetal flowchemistryMetalsvisual_artvisual_art.visual_art_mediumIRPPosttranscriptional regulationCopperFunction (biology)Journal of Experimental Botany
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Comparison of global responses to mild deficiency and excess copper levels in Arabidopsis seedlings

2013

[EN] Copper is an essential micronutrient in higher plants, but it is toxic in excess. The fine adjustments required to fit copper nutritional demands for optimal growth are illustrated by the diverse, severe symptoms resulting from copper deficiency and excess. Here, a differential transcriptomic analysis was done between Arabidopsis thaliana plants suffering from mild copper deficiency and those with a slight copper excess. The effects on the genes encoding cuproproteins or copper homeostasis factors were included in a CuAt database, which was organised to collect additional information and connections to other databases. The categories overrepresented under copper deficiency and copper e…

ArabidopsisBiophysicsFunctional homologchemistry.chemical_elementCircadian clockTransporterBiochemistryBiomaterialsTranscriptomeSuperoxide dismutaseStomatal closureGene Expression Regulation PlantIron homeostasisArabidopsisThalianamedicineHomeostasisArabidopsis thalianaGeneOligonucleotide Array Sequence AnalysisGeneticsDose-Response Relationship DrugbiologyArabidopsis ProteinsReverse Transcriptase Polymerase Chain ReactionSuperoxide DismutaseProteinMetals and AlloysBindingMicronutrientbiology.organism_classificationmedicine.diseaseCopperDNA-Binding ProteinschemistryBiochemistrySeedlingsChemistry (miscellaneous)biology.proteinFeedback loopTranscription factorTranscriptomeCopper deficiencyCopperTranscription FactorsMetallomics
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Safety, tolerability, pharmacokinetics, pharmacodynamics, and exploratory efficacy of the novel enzyme replacement therapy avalglucosidase alfa (neoG…

2019

This multicenter/multinational, open-label, ascending-dose study (NCT01898364) evaluated safety, tolerability, pharmacokinetics, pharmacodynamics, and exploratory efficacy of repeat-dose avalglucosidase alfa (neoGAA), a second-generation, recombinant acid α-glucosidase replacement therapy, in late-onset Pompe disease (LOPD). Patients ≥18 years, alglucosidase alfa naïve (Naïve) or previously receiving alglucosidase alfa for ≥9 months (Switch), with baseline FVC ≥50% predicted and independently ambulatory, received every-other-week avalglucosidase alfa 5, 10, or 20 mg/kg over 24 weeks. 9/10 Naïve and 12/14 Switch patients completed the study. Avalglucosidase alfa was well-tolerated; no deaths…

Avalglucosidase alfa (neoGAA)0301 basic medicineMaleGLUCOSE TETRASACCHARIDELysosomal acid alpha-glucosidase (GAA) deficiencyCHILDRENPulmonary function testingMOTOR FUNCTION0302 clinical medicineMedicineGenetics (clinical)Late-onset Pompe disease (LOPD)Glycogen Storage Disease Type IIAlglucosidase alfaMOUSE MODELEnzyme replacement therapyMiddle AgedTreatment OutcomeNeurologyTolerabilityEnzyme replacement therapySKELETAL-MUSCLEFemaleLife Sciences & BiomedicineMUSCLE TRAINING RMTGlycogen6-MINUTE WALKmedicine.drugAdultmedicine.medical_specialtyClinical NeurologyGLYCOGEN03 medical and health sciencesFEV1/FVC ratioPharmacokineticsInternal medicineHumansEnzyme Replacement TherapyAdverse effectAlglucosidase alfaScience & Technologybusiness.industryNeurosciencesalpha-GlucosidasesADULTSGlycogen storage disease type IISEVERITY030104 developmental biologyPharmacodynamicsPediatrics Perinatology and Child HealthNeurosciences & NeurologyNeurology (clinical)Glucan 14-alpha-Glucosidasebusiness030217 neurology & neurosurgeryNeuromuscular Disorders
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Performance of QuantiFERON-TB Gold Plus for detection of latent tuberculosis infection in pregnant women living in a tuberculosis- and HIV-endemic se…

2017

We evaluated the performance of QuantiFERON-TB Gold Plus (QFT-Plus), which includes two Mycobacterium tuberculosis antigen formulations (TB1 and TB2), for detection of latent tuberculosis infection during pregnancy. Eight-hundred-twenty-nine Ethiopian pregnant women (5.9% HIV-positive) were tested with QFT-Plus, with bacteriological sputum analysis performed for women with clinically suspected tuberculosis and HIV-positive women irrespective of clinical presentation. QFT-Plus read-out was categorized according to the conventional cut-off (0.35 IU/ml) for both antigen formulations. In addition, we analysed the distribution of QFT-Plus results within a borderline zone (0.20–0.70 IU/ml), and i…

Bacterial DiseasesRNA virusesPhysiologyMaternal Healthlcsh:MedicineHIV InfectionsPathology and Laboratory MedicineGastroenterologyWhite Blood Cells0302 clinical medicineImmunodeficiency VirusesPregnancyAnimal CellsMedicine and Health SciencesCell Cycle and Cell Division030212 general & internal medicinePregnancy Complications InfectiousYoung adultlcsh:ScienceMultidisciplinarybiologyLatent tuberculosisT CellsObstetrics and GynecologyGestational ageActinobacteriaInfectious DiseasesMedical MicrobiologyCell ProcessesViral PathogensVirusesFemalePathogensCellular Typesmedicine.symptomResearch ArticleAdultmedicine.medical_specialtyTuberculosisImmune CellsImmunologyViral diseasesMicrobiologyMycobacterium tuberculosisInterferon-gammaYoung Adult03 medical and health sciencesAntigenLatent TuberculosisInternal medicineRetrovirusesmedicineHumansTuberculosisMicrobial PathogensSecretionPregnancyBlood CellsBacteriaTuberculin Testbusiness.industryLentiviruslcsh:ROrganismsHIVBiology and Life SciencesMycobacterium tuberculosisCell BiologyTropical Diseasesbiology.organism_classificationmedicine.disease030228 respiratory systemWomen's HealthSputumlcsh:QEthiopiaMitogensPhysiological ProcessesbusinessInterferon-gamma Release TestsPLOS ONE
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Test-retest reliability of a new self reported comprehensive questionnaire measuring frequencies of different modes of adolescents commuting to schoo…

2009

Published version of an article from the journal: International Journal of Behavioral Nutrition and Physical Activity Article available from: http://www.ijbnpa.org/content/6/1/68 Background: Studies assessing active commuting to school usually use simple questionnaires, and often is mode of commuting reported with a single questionnaire item only. The purpose of the present study is to report the test-retest reliability of a newly developed comprehensive questionnaire on active commuting to school and work among 6thgrade school children and their parents in Norway. Methods: A total of 106 pupils and 77 parents completed a questionnaire two times, 14 days apart. The questionnaire consisted o…

Bicycle commutingNutrition and DieteticsVDP::Medical disciplines: 700::Health sciences: 800::Preventive medicine: 804lcsh:Public aspects of medicinePoison controlBehavioural sciencesMedicine (miscellaneous)Physical Therapy Sports Therapy and RehabilitationSample (statistics)lcsh:RA1-1270Occupational safety and healthTest (assessment)Travel behaviorlcsh:Nutritional diseases. Deficiency diseasesInjury preventionShort PaperVDP::Medical disciplines: 700::Sports medicine: 850Psychologylcsh:RC620-627DemographyThe international journal of behavioral nutrition and physical activity
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Scientific HIV research in Africa and the Middle East: a socio-economic demographic analysis

2021

Background: In Africa, HIV/AIDS research is concentrated in certain countries, particularly South Africa. This distribution may not accurately reflect the disease prevalence or the true research efforts of countries.Objectives: To identify HIV/AIDS research productivity of countries in Africa and the Middle East, in absolute terms and adjusted for people living with HIV, population size and economic development.Methods: We identified all the articles and reviews on HIV and AIDS in the Web of Science Core Collection in which African or Middle Eastern countries had participated. After determining the number of documents produced by each country, we adjusted the findings for the number of peop…

Biomedical ResearchPrevalenceDistribution (economics)HIV InfectionsEfficiencyGross domestic productMiddle East03 medical and health sciences0302 clinical medicineAcquired immunodeficiency syndrome (AIDS)Virologyparasitic diseasesPrevalencemedicineHumans030212 general & internal medicineSocioeconomicsDeveloping CountriesProductivity030505 public healthMiddle Eastbusiness.industryPublic Health Environmental and Occupational HealthGeneral Medicinemedicine.diseaseDemographic analysisInfectious DiseasesGeographyGross national incomeSocioeconomic FactorsAfrica0305 other medical sciencebusinessAfrican Journal of AIDS Research
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Recessive mutations in EPG5 cause Vici syndrome, a multisystem disorder with defective autophagy

2013

Vici syndrome is a recessively inherited multisystem disorder characterized by callosal agenesis, cataracts, cardiomyopathy, combined immunodeficiency and hypopigmentation. To investigate the molecular basis of Vici syndrome, we carried out exome and Sanger sequence analysis in a cohort of 18 affected individuals. We identified recessive mutations in EPG5 (previously KIAA1632), indicating a causative role in Vici syndrome. EPG5 is the human homolog of the metazoan-specific autophagy gene epg-5, encoding a key autophagy regulator (ectopic P-granules autophagy protein 5) implicated in the formation of autolysosomes. Further studies showed a severe block in autophagosomal clearance in muscle a…

BiopsyVesicular Transport ProteinsAutophagy-Related ProteinsGenes RecessiveConsanguinityBiologymedicine.disease_causeArticleCataract03 medical and health sciencesConsanguinity0302 clinical medicineCataractsAntigens NeoplasmGeneticsmedicineAutophagyHumansVici syndromeExomeFamilyMuscle SkeletalExomeImmunodeficiency030304 developmental biologyGenetics0303 health sciencesMutationAutophagyIntracellular Signaling Peptides and ProteinsLysosome-Associated Membrane GlycoproteinsProteinsmedicine.diseaseMutationAutophagy Protein 5Agenesis of Corpus CallosumLysosomes030217 neurology & neurosurgeryNature genetics
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Elimination of Vitamin D Signaling Causes Increased Mortality in a Model of Overactivation of the Insulin Receptor: Role of Lipid Metabolism

2022

Vitamin D (VD) deficiency has been associated with cancer and diabetes. Insulin signaling through the insulin receptor (IR) stimulates cellular responses by activating the PI3K/AKT pathway. PTEN is a tumor suppressor and a negative regulator of the pathway. Its absence enhances insulin signaling leading to hypoglycemia, a dangerous complication found after insulin overdose. We analyzed the effect of VD signaling in a model of overactivation of the IR. We generated inducible double KO (DKO) mice for the VD receptor (VDR) and PTEN. DKO mice showed severe hypoglycemia, lower total cholesterol and increased mortality. No macroscopic tumors were detected. Analysis of the glucose metabolism did n…

BioquímicaBiologiaNutrition and DieteticsdiabetesVitaminshypoglycemia; diabetes; insulin overdose; fatty acids; lipolysisLipid MetabolismVitamin D Deficiencyfatty acidsHypoglycemiaReceptor InsulinMicePhosphatidylinositol 3-Kinasesinsulin overdosehypoglycemialipolysisAnimalsHumansInsulinInsulin ResistanceVitamin DFood ScienceNutrients; Volume 14; Issue 7; Pages: 1516
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Comunicazione orale

2011

Bleeding PhenotypeFactor VII deficiency
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LO SCORBUTO, SEGNALE DI ALLARME DI UN DISTURBO DELLO SPETTRO AUTISTICO

2022

The authors describe the case of a 3-year-old girl with bleeding gums and leg pain finally diagnosed with vitamin C deficiency (scurvy) and autistic spectrum disorder (ASD). The increased risk of developing scurvy due to a restricted diet in children affected by ASD is stressed.

Bleeding gums Vitamin C deficiency Autistic spectrum disorder
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