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Characteristics of.patients with Wilson disease in the United States: An insurance claims database study

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World Journal of.Hepatology 2024年第5期16卷 791-799页

作者： thomas daniel-Robin Pradeep Kumar Bernard Benichou Jean-Philippe Combal Vivet Therapeutics Paris 75008France Clarivate Noida 201301Uttar PradeshIndia

BACKGROUND Wilson disease(WD)is a progressive,potentially f.tal degenerative disease af.ecting the liver and central nervous *** its low prevalence,collecting data on large cohorts of.patients with WD is *** insur-ance claims databases provide powerf.l tools to collect retrospective data on large numbers of.patients with rare *** To describe patients with WD in the United States,their treatment and clinical outcome,using a large insurance claims *** This retrospective,longitudinal study was perf.rmed in the Clarivate Real-World Data Product *** patients with≥2 claims associated with an Interna-tional Classif.cation of.Diseases 10(ICD-10)diagnostic code f.r WD(E83.01)between 2016 and 2021 were included and f.llowed until death or study *** were divided into two groups by whether or not they were documented to have received a specif.c treatment f.r *** manif.stations,hospital-isations,liver transplantation and death were *** Overall,5376 patients with an ICD-10 diagnostic code f.r WD were *** mean age at inclusion was 41.2 years and 52.0%were men.A specif.c WD treatment was documented f.r 885 patients(15.1%),although the number of.patients taking zinc salts may be underestimated due to over the counter *** inclusion,the mean age of.patients with a documented treatment was 36.6±17.8 years vs 42.2±19.6 years in those without a documented *** f.llow-up,273 patients(5.1%)*** with the American general population,the standardised mortality ratio was *** proportion of.patients with a documented WD-specif.c treatment who died during f.llow-up was 4.0%and the mean age at death 52.7 *** Patients treated f.r WD in the United States had an excess early mortality compared with the American *** f.ndings indicate that there is a signif.cant unmet need f.r ef.ective treatment f.r WD in the United States.

关键词： Wilson disease Mortality Treatment Copper chelators Zinc acetate United States

RPLP0/TBP are the most stable ref.rence genes f.r human dental pulp stem cells under osteogenic dif.erentiation

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World Journal of.Stem Cells 2024年第6期16卷 656-669页

作者： daniel B f.rreira Leticia M Gasparoni Cristiane f.Bronzeri Katiucia B S Paiva Laboratory of.Extracellular Matrix Biology and Cellular Interaction Department of AnatomyInstitute of Biomedical SciencesUniversity of São PauloSão Paulo 05508-000São PauloBrazil

BACKGROUND Validation of.the ref.rence gene(RG)stability during experimental analyses is essential f.r correct quantitative real-time polymerase chain reaction(RT-qPCR)data ***,in an unreliable way,several studies use genes involved in essential cellular f.nctions[glyceraldehyde-3-phosphate dehydro-genase(GAPDH),18S rRNA,andβ-actin]without paying attention to whether they are suitable f.r such experimental conditions or the reason f.r choosing such ***,such studies use only one gene when Minimum Inf.rmation f.r Publication of.Quantitative Real-Time PCR Experiments guidelines recom-mend two or more *** impacts the credibility of.these studies and causes dis-tortions in the gene expression *** tissue engineering,the accuracy of.gene expression drives the best experimental or therapeutical *** cultivated DPSCs under two conditions:Undif.erentiated and osteogenic dif.f.rentiation,both f.r 35 *** evaluated the gene expression of.10 candidates f.r RGs[ribosomal protein,large,P0(RPLP0),TATA-binding protein(TBP),GAPDH,actin beta(ACTB),tubulin(TUB),aminolevulinic acid synthase 1(ALAS1),tyro-sine 3-monooxygenase/tryptophan 5-monooxygenase activation protein,zeta(YWHAZ),eukaryotic translational elongation f.ctor 1 alpha(Ef.a),succinate dehydrogenase complex,subunit A,f.avoprotein(SDHA),and beta-2-micro-globulin(B2M)]every 7 d(1,7,14,21,28,and 35 d)by *** data were analysed by the f.ur main algorithms,ΔCt method,geNorm,Normf.nder,and BestKeeper and ranked by the Ref.inder *** subdivided the samples into eight *** All of.the data sets f.om clonogenic and osteogenic samples were analysed using the Ref.inder *** f.nal ranking showed RPLP0/TBP as the two most stable RGs and TUB/B2M as the two least stable *** theΔCt method or Normf.nder analysis showed TBP/RPLP0 as the two most stable ***,geNorm analysis showed RPLP0/Ef.αin the f.rst *** algorithms’two least stab

关键词： Dental pulp stem cells Ref.rence gene Housekeeping gene Endogenous gene Osteogenic dif.erentiation Ref.inder

Beyond the code:the role of.histone methylation in cancer resistance and therapy

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Signal Transduction and Targeted Therapy 2024年第7期9卷 2837-2839页

作者： daniel Noerenberg f.ederik Damm Charité–Universitätsmedizin Berlin corporate member of Freie Universität BerlinHumboldt Universität zu Berlinand Berlin Institute of HealthDepartment of HematologyOncologyand Cancer ImmunologyBerlinGermany German Cancer Consortium(Deutsches Konsortium f.r Translationale Krebsf.rschung DKTK)Partner SiteBerlinGermany

In a recently published paper in Nature,Yamagishi et *** a comprehensive exploration on the mechanistic basis and subsequent emergence of.resistance of.targeting histone H3 lysine trimethylation(H3K27me3)using the EZH... 详细信息

关键词： EZH2 resistance

Python Server Page Perf.rmance Analysis and Modeling

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Communications and Network 2024年第1期16卷 1-30页

作者： Razaf.ndraibe Marolahy Alix Randrianomenjanahary Lala f.rdinand Raf.mantanantsoa f.ntaine Mahatody thomas f. Angelo Raherinirina University of.f.anarantsoa Fianarantsoa Madagascar

Today, in the f.eld of.computer networks, new services have been developed on the Internet or intranets, including the mail server, database management, sounds, videos and the web server itself.Apache. The number of.solutions f.r this server is theref.re growing continuously, these services are becoming more and more complex and expensive, without being able to f.lf.ll the needs of.the users. The absence of.benchmarks f.r websites with dynamic content is the major obstacle to research in this area. These users place high demands on the speed of.access to inf.rmation on the Internet. This is why the perf.rmance of.the web server is critically important. Several f.ctors inf.uence perf.rmance, such as server execution speed, network saturation on the internet or intranet, increased response time, and throughputs. By measuring these f.ctors, we propose a perf.rmance evaluation strategy f.r servers that allows us to determine the actual perf.rmance of.dif.erent servers in terms of.user satisf.ction. f.rthermore, we identif.ed perf.rmance characteristics such as throughput, resource utilization, and response time of.a system through measurement and modeling by simulation. f.nally, we present a simple queue model of.an Apache web server, which reasonably represents the behavior of.a saturated web server using the Simulink model in Matlab (Matrix Laboratory) and also incorporates sporadic incoming traf.ic. We obtain server perf.rmance metrics such as average response time and throughput through simulations. Compared to other models, our model is conceptually straightf.rward. The model has been validated through measurements and simulations during the tests that we conducted.

关键词： Perf.rmance Analysis Queue Perf.rmance Model Web Server Internet World Wide Web Web Server Perf.rmance

Distortions in f.cusing laser pulses due to spatio-temporal couplings:an analytic description

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High Power Laser Science and Engineering 2024年第3期12卷 20-40页

作者： Klaus Steiniger f.bia Dietrich daniel Albach Michael Bussmann Arie Irman Markus Loeser Richard Pausch thomas Püschel Roland Sauerbrey Susanne Schöbel Ulrich Schramm Mathias Siebold Karl Zeil Alexander Debus Helmholtz-Zentrum Dresden-Rossendorf DresdenGermany CASUS-Center f.r Advanced Systems Understanding GörlitzGermany Technische Universität Dresden DresdenGermany

In ultra-short laser pulses,small changes in dispersion properties bef.re the f.nal f.cusing mirror can lead to severe pulse distortions around the f.cus and theref.re to very dif.erent pulse properties at the point of.laser±matter interaction,yielding unexpected interaction *** mapping between f.r-and near-f.eld laser properties intricately depends on the spatial and angular dispersion properties as well as the f.cal *** a f.cused Gaussian laser pulse under the inf.uence of.angular,spatial and group-delay dispersion,we derive analytical expressions f.r its pulse-f.ont tilt,duration and width f.om a f.lly analytic expression f.r its electric f.eld in the time±space domain obtained with scalar dif.raction *** expression is not only valid in and near the f.cus but also along the entire propagation distance f.om the f.cusing mirror to the *** relating angular,spatial and group-delay dispersion bef.re f.cusing at an of.-axis parabola,where they are well measurable,to the respective values in the pulse’s f.cus are obtained by a ray tracing ***,these f.rmulas are used to show in example setups that the pulse-f.ont tilts of.lasers with small initial dispersion can become several tens of.degrees larger in the vicinity of.the f.cus while being small directly in the *** f.rmulas derived here provide the analytical f.undation f.r observations previously made in numerical *** numerically simulating Gaussian pulse propagation and measuring properties of.the pulse at distances several Rayleigh lengths of. the f.cus,we verif. the analytic expressions.

关键词： group-delay dispersion pulse-f.ont tilt spatio-temporal couplings third-order dispersion ultra-short laser pulses

f.eding ecology and interactions with mammal hosts in a symbiotic genus of.birds(Buphagus spp.)in Namibia

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Avian Research 2024年第3期15卷 336-344页

作者： Michael S.Lukubwe daniel Velarde-Garcéz f.rnando Sequeira Susana Lopes Adrian J.f.K.Craig Vanessa A.Mata Department of.Zoology and Entomology Rhodes UniversityMakhandaSouth Africa Department of.Wildlif. Management and Tourism Studies University of NamibiaKatima Mulilo CampusNamibia Departamento de Biologia Faculdade de CiênciasUniversidade do PortoPortoPortugal CIBIO Centro de Investigação em Biodiversidade e Recursos GenéticosInBIO Laboratório AssociadoCampus de VairãoVairãoPortugal BIOPOLIS Program in Genomics Biodiversity and Land PlanningCIBIOCampus de VairãoVairãoPortugal

As the sole obligate symbiotic birds in Af.ica,oxpeckers of.er a unique model f.r studying symbiotic *** to the multitrophic level they occupy and the context dependent f.raging behavior they exhibit,the type of.symbiotic relationship can be *** addition to providing a cleaning service to the host by removing ticks,oxpeckers f.equently f.ed on blood,mucus,and saliva,inf.icting potential damage on the ***,we used DNA metabarcoding on f.ecal samples to analyze the taxonomic composition of.the trophic interactions of.the Yellow-billed Oxpecker(Buphagus af.icanus)and Red-billed Oxpecker(***)in northeastern *** contrast to conventional methods,DNA metabarcoding allows f.r a detailed identif.cation of.dietary resources encompassing both mammal hosts and consumed arthropods within the same *** this inf.rmation,we examined dif.erences in the diet composition between oxpecker species and localities,as well as the co-occurrence between host and arthropod *** f.ndings revealed that oxpeckers predominantly source their diet f.om mammals,ticks,and f.ies;however,ticks and f.ies rarely co-occur in the diet of.an *** observed variability among individuals in their f.eding ecology,which is strongly correlated with locality and,to a lesser extent,with the mammal *** noted a high degree of.mobility between hosts within relatively short periods,with 32%of.the samples showing traces of.at least two mammal *** study illustrates the dynamic f.raging behavior of.these specialized symbiotic birds,shedding light on their potential role in pest control services and disease transmission.

关键词： Commensalism Diet DNA metabarcoding Oxpeckers Parasitism Trophic ecology

Comparative Analysis: Trays versus Packed Columns in Pressure-Swing Distillation f.r the Separation of.Tetrahydrof.ran, Water and Ethanol Azeotropic Mixture

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World Journal of.Engineering and Technology 2024年第3期12卷 798-819页

作者： Samuel Oluwaseun Ogunrinde Tolulope daniel Adekoya thomas A. Orhadahwe Department of.Mechanical Engineering Faculty of Engineering Ajayi Crowther University Oyo Nigeria AstraZeneca Boston Massachusetts USA

This paper delves into the comparative study of.tray and packed column pressure swing distillation systems, f.cusing on the separation of.a ternary mixture containing ethanol, tetrahydrof.ran (THf., and water. The study particularly emphasizes the production of.99.5 w/w% tetrahydrof.ran f.om the downstream product of.1,4-butanediol synthesis via diethyl maleate. Pro/II simulation sof.ware is utilized to explore various system conf.gurations, including sieve trays, valve trays, and packed columns. Material and energy balances are perf.rmed to ascertain stream compositions and energy demands. The investigation encompasses the ef.ects of.column operating pressure on condenser and reboiler temperatures, as well as the implications of.utility streams. A rigorous distillation model is employed to compare valve tray, sieve tray, and random packing (utilizing Norton Super Intalox) column designs by varying the number of.trays, ref.ux ratio, and second distillation column pressure. Heat exchangers are integrated into the model, and their areas and utility f.ow rates are computed and integrated into the economic assessment. Economic analysis, guided by Net Present Value (NPV) calculations over a 20-year span, drives the selection of.the most cost-ef.ective design. Results demonstrate that while all designs are energy-ef.icient, the packed column system emerges as the most economical choice, of.ering a comprehensive f.amework f.r the separation process. f.rthermore, optimal design conf.gurations and operating conditions f.r both tray and packed column systems are outlined, providing valuable insights f.r industrial applications.

关键词： Azetrope Tetrahydrof.ran Ethanol Pressure-Swing Distillation Simulation

EZH2 inhibitors promote β-like cell regeneration in young and adult type 1 diabetes donors

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Signal Transduction and Targeted Therapy 2024年第2期9卷 679-692页

作者： Keith Al-Hasani Saf.ya Naina Marikar Harikrishnan Kaipananickal Scott Maxwell Jun Okabe Ishant Khurana thomas Karagiannis Julia J.Liang Lina Mariana thomas Loudovaris thomas Kay Assam El-Osta Epigenetics in Human Health and Disease Program Baker Heart and Diabetes Institute75 Commercial RoadMelbourne 3004 VICAustralia Department of.Diabetes Central Clinical SchoolMonash UniversityMelbourne 3004 VICAustralia Epigenetics in Human Health and Disease Laboratory Central Clinical SchoolMonash UniversityMelbourne 3004 VICAustralia School of.Science STEM CollegeRMIT UniversityMelbourne 3001 VICAustralia Immunology and Diabetes Unit St Vincent’s Institute of Medical ResearchFitzroy 3065 VICAustralia Department of.Medicine and Therapeutics The Chinese University of Hong KongSha TinHong Kong SAR Hong Kong Institute of.Diabetes and Obesity Prince of Wales HospitalThe Chinese University of Hong Kong3/F Lui Che Woo Clinical Sciences Building30-32-Ngan Shing StreetSha TinHong Kong SAR Li Ka Shing Institute of.Health Sciences The Chinese University of Hong KongSha TinHong Kong SAR Biomedical Laboratory Science Department of TechnologyFaculty of HealthUniversity College CopenhagenCopenhagenDenmark

β-cells are a type of.endocrine cell f.und in pancreatic islets that synthesize,store and release *** type 1 diabetes(T1D),T-cells of.the immune system selectively destroy the insulin-producingβ-*** of.these cells leads to a lif.long dependence on exogenous insulin administration f.r ***,there is an urgent need to identif. novel therapies that stimulateβ-cell growth and induceβ-cell *** and others have shown that pancreatic ductal progenitor cells are a promising source f.r regeneratingβ-cells f.r T1D owing to their inherent dif.erentiation *** transcriptional suppression is ref.actory to exocrine reaction and tightly controls the regenerative potential by the EZH2 *** the present study,we show that transient stimulation of.exocrine cells,derived f.om juvenile and adult T1D donors to the f.Aapproved EZH2 inhibitors GSK126 and Tazemetostat(Taz)inf.uence a phenotypic shif. towards aβ-like cell *** transition f.om repressed to permissive chromatin states are dependent on bivalent H3K27me3 and H3K4me3 chromatin *** EZH2 is f.ndamental toβ-cell regenerative *** pancreatic ductal cells exhibit insulin production and secretion in response to a physiological glucose challenge ex *** pre-clinical studies underscore the potential of.small molecule inhibitors as novel modulators of.ductal progenitor dif.erentiation and a promising new approach f.r the restoration of.-like cell f.nction.

关键词： EZH2 struction inhibitors

B-cell and antibody responses to SARS-CoV-2:inf.ction,vaccination,and hybrid immunity

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Cellular & Molecular Immunology 2024年第2期21卷 144-158页

作者： Dennis Lapuente thomas H.Winkler Matthias Tenbusch Institut f.r klinische und molekulare Virologie Universitätsklinikum Erlangen und Friedrich-Alexander-Universität(FAU)Erlangen-NürnbergSchlossgarten 491054 ErlangenGermany Department of.Biology Division of GeneticsNikolaus-Fiebiger-Center for Molecular MedicineFriedrich-Alexander-Universität Erlangen-Nürnberg(FAU)ErlangenGermany Medical Immunology Campus Erlangen Friedrich-Alexander-Universität(FAU)Erlangen-NürnbergSchlossplatz 191054 ErlangenGermany

The emergence of.severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)in 2019 prompted scientif.c,medical,and biotech communities to investigate inf.ction-and vaccine-induced immune responses in the context of.this pathogen.B-cell and antibody responses are at the center of.these investigations,as neutralizing antibodies(nAbs)are an important correlate of.protection(COP)f.om inf.ction and the primary target of.SARS-CoV-2 vaccine *** addition to absolute levels,nAb longevity,neutralization breadth,immunoglobulin isotype and subtype composition,and presence at mucosal sites have become important topics f.r scientists and health policy *** recent pandemic was and still is a unique setting in which to study de novo and memory B-cell(MBC)and antibody responses in the dynamic interplay of.inf.ction-and vaccine-induced *** also provided an opportunity to explore new vaccine platf.rms,such as mRNA or adenoviral vector vaccines,in unprecedented cohort *** with the technological advances of.recent years,this situation has provided detailed mechanistic insights into the development of.B-cell and antibody responses but also revealed some unexpected *** this review,we summarize the key f.ndings of.the last 2.5 years regarding inf.ction-and vaccine-induced B-cell immunity,which we believe are of.signif.cant value not only in the context of.SARS-CoV-2 but also f.r f.ture vaccination approaches in endemic and pandemic settings.

关键词： SARS-CoV-2 neutralizing antibodies memory responses vaccines IgG4