Search results for "P50"

showing 10 items of 56 documents

Characterization of the Clinical and Immunologic Phenotype and Management of 157 Individuals with 56 Distinct Heterozygous NFKB1 Mutations

2020

Contains fulltext : 229571.pdf (Publisher’s version ) (Closed access) BACKGROUND: An increasing number of NFKB1 variants are being identified in patients with heterogeneous immunologic phenotypes. OBJECTIVE: To characterize the clinical and cellular phenotype as well as the management of patients with heterozygous NFKB1 mutations. METHODS: In a worldwide collaborative effort, we evaluated 231 individuals harboring 105 distinct heterozygous NFKB1 variants. To provide evidence for pathogenicity, each variant was assessed in silico; in addition, 32 variants were assessed by functional in vitro testing of nuclear factor of kappa light polypeptide gene enhancer in B cells (NF-κB) signaling. RESU…

0301 basic medicineMaleNF-KAPPA-BMedizinlnfectious Diseases and Global Health Radboud Institute for Molecular Life Sciences [Radboudumc 4]Fluorescent Antibody TechniqueAutoimmunityDiseaseNUCLEAR-FACTORKaplan-Meier Estimatemedicine.disease_causeHypogammaglobulinemia0302 clinical medicineNFKB1 variants and mutations; autosomal dominant inheritance; common variable immunodeficiency; reduced penetrance; variable expressivityHDE PEDImmunology and Allergyvariants and mutationsNF-κB1-related phenotypeImmunodeficiencyIMMUNODEFICIENCY*NF-?B1-related phenotypeNFKB1 variants and mutations1184 Genetics developmental biology physiologycommon variable immunodeficiencyDisease ManagementMiddle AgedNF-kappa B1-related phenotypereduced penetrancePrognosisPenetranceImmunohistochemistryMagnetic Resonance Imaging3. Good healthPhenotypeNFKB1 variant*NFKB1 variant*common variable immunodeficiencyFemaleHaploinsufficiency*reduced penetranceNFKB1 mutationAdultHeterozygote*NFKB1 mutationImmunologyHAPLOINSUFFICIENCYArticle03 medical and health sciencesvariable expressivityautosomal dominantmedicineHumansGenetic Predisposition to DiseaseGenetic Association StudiesAgedbusiness.industryCommon variable immunodeficiencyNF-kappa B p50 SubunitNF-KAPPA-B1Immune dysregulationmedicine.diseaseautosomal dominant inheritance030104 developmental biologyBiological Variation PopulationImmunologyCELLSMutation*autosomal dominantPrimary immunodeficiency3111 BiomedicinebusinessTomography X-Ray ComputedBiomarkers030215 immunology
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Use of autochthonous yeasts and bacteria in order to control Brettanomyces bruxellensis in wine

2017

Biocontrol strategies for the limitation of undesired microbial developments in foods and beverages represent a keystone toward the goal of more sustainable food systems. Brettanomyces bruxellensis is a wine spoilage microorganism that produces several compounds that are detrimental for the organoleptic quality of the wine, including some classes of volatile phenols. To control the proliferation of this yeast, sulfur dioxide is commonly employed, but the efficiency of this compound depends on the B. bruxellensis strain; and it is subject to wine composition and may induce the entrance in a viable, but nonculturable state of yeasts. Moreover, it can also elicit allergic reactions in humans. …

0301 basic medicineMicroorganism030106 microbiologyFood spoilageVolatile phenolsBrettanomyces bruxellensisWineSaccharomyces cerevisiaePlant ScienceBiochemistry Genetics and Molecular Biology (miscellaneous)Aliments Microbiologia03 medical and health sciencesMalolactic fermentationFood scienceNon- SaccharomycesOenologyOenococcus oeniWinelcsh:TP500-660non-Saccharomycesbiology<i>Brettanomyces bruxellensis</i>; volatile phenols; biocontrol; <i>Saccharomyces cerevisiae</i>; non-<i>Saccharomyces</i>; <i>Oenococcus oeni</i>; wineBiocontrolfood and beverageslcsh:Fermentation industries. Beverages. Alcoholbiology.organism_classificationYeastBrettanomyces bruxellensisViniculturaBiocontrol; Brettanomyces bruxellensis; Non- Saccharomyces; Oenococcus oeni; Saccharomyces cerevisiae; Volatile phenols; WineOenococcus oeniSettore AGR/16 - Microbiologia AgrariaFood Science
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Performances of Different Metabolic Lactobacillus Groups During the Fermentation of Pizza Doughs Processed from Semolina

2018

The main hypothesis of this work is that facultative and obligate heterofermentative Lactobacillus species can differently impact the final characteristics of pizza. The objective was to evaluate separately the behaviors of the obligate heterofermentative species (OHS), such as Lactobacillus sanfranciscensis, Lactobacillus brevis, and Lactobacillus rossiae, and the facultative heterofermentative species (FHS), including Lactobacillus plantarum, and Lactobacillus curvatus, in the sourdoughs to be used for pizza production. The production of the experimental pizzas was carried out with semolina (Triticum turgidum L. ssp. durum). The acidification process—which was followed by pH, total titrat…

0301 basic medicineSeasoningsourdough fermentationfacultative heterofermentative species030106 microbiologyLactobacillus sanfranciscensisTitratable acidPlant ScienceBiochemistry Genetics and Molecular Biology (miscellaneous)03 medical and health scienceschemistry.chemical_compoundLactobacilluspizzavolatile organic compoundsFood scienceobligate heterofermentative specieobligate heterofermentative specieslcsh:TP500-660biologyfacultative heterofermentative speciefood and beveragesSettore AGR/15 - Scienze E Tecnologie Alimentaribiology.organism_classificationlcsh:Fermentation industries. Beverages. AlcoholLactic acidlactic acid bacteriachemistryFermentationshape descriptorshape descriptorsBacteriaLactobacillus plantarumFood ScienceSettore AGR/16 - Microbiologia Agraria
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Characterization of sulfhydryl oxidase from Aspergillus tubingensis

2017

Background Despite of the presence of sulfhydryl oxidases (SOXs) in the secretomes of industrially relevant organisms and their many potential applications, only few of these enzymes have been biochemically characterized. In addition, basic functions of most of the SOX enzymes reported so far are not fully understood. In particular, the physiological role of secreted fungal SOXs is unclear. Results The recently identified SOX from Aspergillus tubingensis (AtSOX) was produced, purified and characterized in the present work. AtSOX had a pH optimum of 6.5, and showed a good pH stability retaining more than 80% of the initial activity in a pH range 4-8.5 within 20 h. More than 70% of the initia…

0301 basic medicineentsyymitBOVINE-MILKThioredoxin reductaselcsh:Animal biochemistryBiochemistrySubstrate Specificitychemistry.chemical_compoundNonribosomal peptide synthesisEnzyme Stabilitylcsh:QD415-436DisulfidesDISULFIDE BONDSPeptide Synthaseschemistry.chemical_classificationbiologyGliotoxinChemistrynonribosomal peptide synthesisHydrogen-Ion ConcentrationGlutathioneFAMILYSOXSglutathione oxidationhomesienetAspergillusBiochemistrySENSITIVITYsecreted sulfhydryl oxidaseOxidoreductasesResearch ArticleDithiol oxidaseCofactorlcsh:Biochemistry03 medical and health sciencesNonribosomal peptideNATURAL-PRODUCTSoksidoreduktaasitBIOSYNTHESISlcsh:QP501-801Molecular Biologysecondary metabolismPURIFICATIONIDENTIFICATION030102 biochemistry & molecular biologyCXXC-MOTIFGlutathioneNIGERluonnonaineet030104 developmental biologyEnzymedithiol oxidasebiology.protein1182 Biochemistry cell and molecular biologyAspergillus tubingensisSecreted sulfhydryl oxidaseSecondary metabolismGlutathione oxidationCysteineBMC Biochemistry
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Corporate Governance Theories: From Micro Theories to National Systems Theories

2004

The objective of this article is to conduct a survey of the different corporate governance theories. In the first part, we present the micro theories by opposing the disciplinary view to the knowledge-based view. The second part deals with the macro or national systems theories. We separate the theories based on appropriation of the organizational rent from those attributing a dominant role to production. This survey highlights that the financial view of corporate governance is a very particular case and presents many limits.

050208 financebusiness.industryCorporate governance05 social sciencesjel:G30AccountingAppropriationjel:P50Systems theoryPolitical science0502 economics and businessnational systems of governance;micro theories of corporate governance;macro theories of corporate governance;disciplinary view;knowledge-based view;law and finance view;political theory;varieties of capitalism.Production (economics)Political philosophy050207 economicsMacroPositive economicsbusinessDiscipline
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Magic informationally complete POVMs with permutations

2017

Eigenstates of permutation gates are either stabilizer states (for gates in the Pauli group) or magic states, thus allowing universal quantum computation [M. Planat and Rukhsan-Ul-Haq, Preprint 1701.06443]. We show in this paper that a subset of such magic states, when acting on the generalized Pauli group, define (asymmetric) informationally complete POVMs. Such IC-POVMs, investigated in dimensions $2$ to $12$, exhibit simple finite geometries in their projector products and, for dimensions $4$ and $8$ and $9$, relate to two-qubit, three-qubit and two-qutrit contextuality.

1003permutation groups159informationally complete povmsFOS: Physical sciences01 natural sciences157[SPI.MAT]Engineering Sciences [physics]/Materialslaw.inventionCombinatorics81P50 81P68 81P13 81P45 20B05Permutationlaw0103 physical sciences1009[SPI.NANO]Engineering Sciences [physics]/Micro and nanotechnologies/Microelectronics010306 general physicslcsh:ScienceEigenvalues and eigenvectorsQuantum computer[SPI.ACOU]Engineering Sciences [physics]/Acoustics [physics.class-ph]PhysicsQuantum Physics120Multidisciplinary010308 nuclear & particles physicsPhysicsMagic (programming)Q Science (General)16. Peace & justiceKochen–Specker theoremProjectorfinite geometryPauli groupquantum contextualitylcsh:QPreprintmagic statesQuantum Physics (quant-ph)Research Article
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Elevated levels of Bcl-3 inhibits Treg development and function resulting in spontaneous colitis

2017

Bcl-3 is an atypical NF-κB family member that regulates NF-κB-dependent gene expression in effector T cells, but a cell-intrinsic function in regulatory T (Treg) cells and colitis is not clear. Here we show that Bcl-3 expression levels in colonic T cells correlate with disease manifestation in patients with inflammatory bowel disease. Mice with T-cell-specific overexpression of Bcl-3 develop severe colitis that can be attributed to defective Treg cell development and function, leading to the infiltration of immune cells such as pro-inflammatory γδT cells, but not αβ T cells. In Treg cells, Bcl-3 associates directly with NF-κB p50 to inhibit DNA binding of p50/p50 and p50/p65 NF-κB dimers, t…

AdultMale0301 basic medicineP50ScienceGeneral Physics and AstronomyBiologyT-Lymphocytes RegulatoryInflammatory bowel diseaseArticleGeneral Biochemistry Genetics and Molecular BiologyYoung Adult03 medical and health sciences0302 clinical medicineImmune systemB-Cell Lymphoma 3 ProteinProto-Oncogene ProteinsGene expressionmedicineAnimalsHumansColitisMice KnockoutRegulation of gene expressionMultidisciplinaryEffectorHEK 293 cellsQNF-kappa BTranscription Factor RelANF-kappa B p50 SubunitGeneral ChemistryMiddle AgedColitismedicine.diseaseMice Inbred C57BLHEK293 Cells030104 developmental biologyGene Expression Regulation030220 oncology & carcinogenesisImmunologyFemaleProtein BindingTranscription FactorsNature Communications
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Transcriptional and post-transcriptional regulation of iNOS expression in human chondrocytes

2009

Chondrocytes are important for the development and maintenance of articular cartilage. However, both in osteoarthritis (OA) and rheumatoid arthritis (RA) chondrocytes are involved in the process of cartilage degradation and synthesize important immunomodulatory mediators, including nitric oxide (NO) generated by the inducible NO synthase (iNOS). To uncover the role of iNOS in the pathomechanisms of OA and RA, we analyzed the regulation of iNOS expression using immortalized human chondrocytes as a reproducible model. In C-28/I2 chondrocytes, iNOS expression was associated with the expression of the chondrocyte phenotype. Peak induction by a cytokine cocktail occurred between 6 and 8h and dec…

Cartilage Articularmedicine.medical_specialtyAnti-Inflammatory AgentsNitric Oxide Synthase Type IIBiologyBiochemistryp38 Mitogen-Activated Protein KinasesChondrocyteArticleGene Expression Regulation EnzymologicGlucocorticoid receptorChondrocytesReceptors GlucocorticoidInternal medicineGene expressionmedicineHumansRNA MessengerRNA Processing Post-TranscriptionalPost-transcriptional regulationCell Line TransformedPharmacologyRegulation of gene expressionNF-kappa B p50 SubunitRNA-Binding ProteinsInterferon-Stimulated Gene Factor 3Janus Kinase 2Cell biologyNitric oxide synthaseEndocrinologymedicine.anatomical_structureCell cultureEnzyme Inductionbiology.proteinTrans-ActivatorsCytokinesZearalenoneSignal transduction
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Combined approaches to identify genomic regions involved in phenotypic differentiation between low divergent breeds: Application in Sardinian sheep p…

2019

Selective breeding has led to modifications in the genome of many livestock breeds. In this study, we identified the genomic regions that may explain some of the phenotypic differences between two closely related breeds from Sardinia. A total of 44 animals, 20 Sardinian Ancestral Black (SAB) and 24 Sardinian White (SW), were genotyped using the Illumina Ovine 50K array. A total of 68, 38 and 15 significant markers were identified using the case–control genome-wide association study (GWAS), the Bayesian population differentiation analysis (FST) and the Rsb metric, respectively. Comparisons among the approaches revealed a total of 22 overlapping markers between GWAS and FST and one marker bet…

CoatGenotypePopulationGenome-wide association studygenome-wide methodsBiologySelective breedingGenomePolymorphism Single NucleotideSettore AGR/17 - Zootecnica Generale E Miglioramento GeneticoFood Animalsgenome-wide methods; genomic regions; Ovine BeadChip50K; Sardinian sheep breedsSardinian sheep breedsAnimalsGenetic variabilitygenomic regionseducationGeneOvine BeadChip50Keducation.field_of_studySheepHomozygoteMolecular Sequence AnnotationGeneral MedicineGenomicsgenome-wide methodgenomic regionWhite (mutation)PhenotypeEvolutionary biologyAnimal Science and ZoologyJournal of animal breeding and genetics = Zeitschrift fur Tierzuchtung und ZuchtungsbiologieREFERENCES
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Evaluation of the Fermentation Dynamics of Commercial Baker’s Yeast in Presence of Pistachio Powder to Produce Lysine-Enriched Breads

2019

The present work was carried out to evaluate the microbiological, physicochemical, and sensory characteristics of fortified pistachio breads. Pistachio powder (5% w/w) was added to flour or semolina and fermented by a commercial baker&#8217;s yeast (Saccharomyces cerevisiae). Pistachio powder did not influence the biological leavening of the doughs. The kinetics of pH and total titratable acidity (TTA) during dough fermentation showed that the leavening process occurred similarly for all trials. The concentration of yeasts increased during fermentation and reached levels of 108 CFU/g after 2 h. Pistachio powder decreased the height and softness of the final breads and increased cell density…

Dough fermentationlcsh:TP500-660lysinebiologyChemistrybiological leaveningdigestive oral and skin physiologyLysineSaccharomyces cerevisiaefood and beveragesTitratable acidPlant Sciencelcsh:Fermentation industries. Beverages. Alcoholbiology.organism_classificationBiochemistry Genetics and Molecular Biology (miscellaneous)Yeastbread fortificationcommercial baker’s yeastCell densityFermentationFood sciencepistachio powderSettore AGR/16 - Microbiologia AgrariaFood ScienceLeavening agentFermentation
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