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Beyond monogenetic rare variants:tackling the low rate of genetic diagnoses in predominantly antibody deficiency

Cellular & Molecular Immunology 2021年第3期18卷 588-603页

作者： Emily S.j.Edwards julian j.bosco Samar Ojaimi Robyn E.O’Hehir Menno C.van Zelm Department of Immunology and Pathology Central Clinical SchoolMonash UniversityMelbourneVICAustralia The jeffrey Modell Diagnostic and Research Centre for Primary Immunodeficiencies MelbourneVICAustralia Department of Allergy Immunology and Respiratory MedicineCentral Clinical SchoolMonash University and AllergyAsthma and Clinical Immunology ServiceAlfred HospitalMelbourneVICAustralia Department of Infectious Diseases Monash HealthClaytonVICAustralia Centre for Inflammatory Diseases Monash HealthClaytonVICAustralia Department of Allergy and Immunology Monash HealthClaytonVICAustralia

Predominantly antibody deficiency(PAD)is the most prevalent form of primary immunodeficiency,and is characterized by broad clinical,immunological and genetic *** the current gold standard of whole exome sequencing for diagnosis,pathogenic gene variants are only identified in less than 20% of *** elucidation of the causal genes underlying PAD has provided many insights into the cellular and molecular mechanisms underpinning disease pathogenesis,many other genes may remain as yet undefined to enable definitive diagnosis,prognostic monitoring and targeted therapy of *** that many patients display a relatively late onset of disease presentation in their 2^(nd) or 3^(rd) decade of life,it is questionable whether a single genetic lesion underlies disease in all ***,combined effects of other gene variants and/or non-genetic factors,including specific infections can drive disease *** this review,we define(1)the clinical and immunological variability of PAD,(2)consider how genetic defects identified in PAD have given insight into B-cell immunobiology,(3)address recent technological advances in genomics and the challenges associated with identifying causal variants,and(4)discuss how functional validation of variants of unknown significance could potentially be translated into increased diagnostic rates,improved prognostic monitoring and personalized medicine for PAD patients.A multidisciplinary approach will be the key to curtailing the early mortality and high morbidity rates in this immune disorder.

关键词： Predominantly antibody deficiency Genetic diagnosis Genomics Functional validation

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Single-shot 3D incoherent imaging with diffuser endoscopy

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Light(Advanced Manufacturing) 2024年第2期5卷 64-74页

作者： julian Lich Tom Glosemeyer jürgen Czarske Robert Kuschmierz Laboratory of Measurement and Sensor System Technique TU DresdenGermany Competence Center for Biomedical Computational Laser Systems(BIOLAS) TU DresdenGermany Else Kroner Fresenius Center for Digital Health(EKFZ) TU DresdenGermany Cluster of Excellence Physics of Life TU DresdenGermany

Minimally invasive endoscopy offers a high potential for biomedical imaging ***,conventional fiberoptic endoscopes require lens systems which are not suitable for real-time 3D ***,a diffuser is utilized for passively encoding incoherent 3D objects into 2D speckle *** networks are employed for fast computational image reconstruction beyond the optical memory *** this paper,we demonstrate single-shot 3D incoherent fiber imaging with keyhole access at video *** the diffuser fiber endoscope for fluorescence imaging is promising for in vivo deep brain diagnostics with cellular resolution.

关键词： Endoscopy Diffuser Deep learning Coherent fiber bundle Multicore fiber Neural networks Fiber optics Speckle patterns Fluorescence imaging

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Solvation Effects on the Dielectric Constant of 1 M LiPF6 in Ethylene Carbonate:Ethyl Methyl Carbonate 3:7

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Energy & Environmental Materials 2024年第1期7卷 253-256页

作者： julian Self Nathan T.Hahn Kristin A.Persson Department of Chemical Engineering Polytechnique MontrealMontrealQC H3T 1J4Canada Department of Materials Science and Engineering University of CaliforniaBerkeleyCA 94720USA Energy Technologies Area Lawrence Berkeley National LaboratoryBerkeleyCA 94720USA Material Physical and Chemical Sciences CenterSandia National LaboratoriesAlbuquerqueNM 87185USA

We report the dielectric constant of 1 M LiPF_(6)in EC:EMC 3∶7 w/w(ethylene carbonate/ethyl methyl carbonate)in addition to neat EC:EMC 3∶7 w/*** three Debye relaxations,the static permittivity value,or dielectric constant,is extrapolated to 18.5,which is compared to 18.7 for the neat solvent *** EC solvent is found to strongly coordinate with the Li^(+)cations of the salt,which results in a loss of dielectric contribution to the ***,the small amplitude and large uncertainty in relaxation frequency for EMC cloud definitive identification of the Li^(+)solvation ***,the loss of the free EC permittivity contribution due to Li^(+)solvation is almost completely balanced by the positive contribution of the associated LiPF_(6)salt,demonstrating that a significant quantity of dipolar ion pairs exists in 1 M LiPF_(6)in EC:EMC 3∶7.

关键词： batteries dielectric relaxation spectroscopy electrolytes

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Piezo1 expression in chondrocytes controls endochondral ossification and osteoarthritis development

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Bone Research 2024年第1期12卷 199-214页

作者： Laura j.Brylka Assil-Ramin Alimy Miriam E.A.Tschaffon-Müller Shan jiang Tobias Malte Ballhause Anke Baranowsky Simon von Kroge julian Delsmann Eva Pawlus Kian Eghbalian Klaus Püschel Astrid Schoppa Melanie Haffner-Luntzer David j.Beech Frank Timo Beil Michael Amling johannes Keller Anita Ignatius Timur A.Yorgan Tim Rolvien Thorsten Schinke Department of Osteology and Biomechanics University Medical Center Hamburg-Eppendorf20246 HamburgGermany Department of Trauma and Orthopedic Surgery University Medical Center Hamburg-Eppendorf20246 HamburgGermany Institute of Orthopedic Research and Biomechanics University Medical Center UlmBadenWürttemberg89081 UlmGermany Department Legal Medicine University Medical Center Hamburg-Eppendorf20246 HamburgGermany Leeds Institute of Cardiovascular and Metabolic Medicine School of MedicineUniversity of LeedsLS29JT LeedsUK

Piezo proteins are mechanically activated ion channels,which are required for mechanosensing functions in a variety of cell *** we and others have previously demonstrated that the expression of Piezo1 in osteoblast lineage cells is essential for boneanabolic processes,there was only suggestive evidence indicating a role of Piezo1 and/or Piezo2 in *** we addressed the question if and how chondrocyte expression of the mechanosensitive proteins Piezo1 or Piezo2 controls physiological endochondral ossification and pathological osteoarthritis(OA)*** with chondrocyte-specific inactivation of Piezo1(Piezo1^(Col2a1Cre)),but not of Piezo2,developed a near absence of trabecular bone below the chondrogenic growth plate ***,all Piezo1^(Col2a1Cre) animals displayed multiple fractures of rib bones at 7 days of age,which were located close to the growth *** skeletal growth was only mildly affected in these mice,OA pathologies were markedly less pronounced compared to littermate controls at 60 weeks of ***,when OA was induced by anterior cruciate ligament transection,only the chondrocyte inactivation of Piezo1,not of Piezo2,resulted in attenuated articular cartilage ***,osteophyte formation and maturation were also reduced in Piezo1^(Col2a1Cre) *** further observed increased Piezo1 protein abundance in cartilaginous zones of human ***,we identified Ptgs2 and Ccn2 as potentially relevant Piezo1 downstream genes in ***,our data do not only demonstrate that Piezo1 is a critical regulator of physiological and pathological endochondral ossification processes,but also suggest that Piezo1 antagonists may be established as a novel approach to limit osteophyte formation in OA.

关键词： Piezo1 markedly ossification

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Versus—A tool for evaluating visualizations and image quality using a 2AFC methodology

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Visual Informatics 2018年第4期2卷 225-234页

作者： jenny Vuong Sandeep Kaur julian Heinrich bosco K.Ho Christopher j.Hammang Benedetta F.Baldi Seán I.O’Donoghue CSIRO Data61Australia Garvan Institute of Medical Research Australia University of New South Wales Australia University of Tübingen Germany University of Sydney Australia

Novel visualization methods and strategies are necessary to cope with the deluge of datasets present in any scientific field to make discoveries and find answers to previously unanswered *** methods and strategies should not only present scientific findings as images in a concise way but also need to be effective and expressive,which often remain ***,we present Versus,a tool to enable easy image quality assessment and image ranking,utilizing a two-alternative forced choice methodology(2AFC)and an efficient ranking algorithm based on a binary *** tool provides a systematic way of setting up evaluation experiments via the web without the necessity to install any additional software or require any programming ***,Versus can easily interface with crowdsourcing platforms,such as Amazon’s Mechanical Turk,or can be used as a stand-alone system to carry out evaluations with *** demonstrate the use of Versus by means of an image evaluation study,aiming to determine if hue,saturation,brightness,and texture are good indicators of uncertainty in three-dimensional protein *** from the power of crowdsourcing,we argue that there is demand and also great potential for this tool to become a standard for simple and fast image evaluations,with the aim to test the effectiveness and expressiveness of scientific visualizations.

关键词： Evaluation Visualization Visual analytics Image comparison Crowdsourcing Evaluation methods 2AFC Image evaluation Tool Visualization evaluation

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Traditional Healer’s Medicinal Practice Survey and Clinical Evidence Assessment of “YIKI”: An Antimalarial Phytomedicine Recipe from Bobo-Dioulasso, Burkina Faso

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Pharmacology & Pharmacy 2024年第7期15卷 248-267页

作者： Zachari Kabre Rakiswende Serge Yerbanga Aminata Fofana Roland Nâg-Tiero Meda Anyirekun Fabrice Some Alassane Haro Natacha A. j. A. Boro Fazhira B. Ye Benjamin Kouliga Koama Sami Eric Kam Eliasse Zongo Hadidjatou Belèm Windmi Kagambèga Florence Fournet Georges A. Ouedraogo jean-bosco Ouedraogo Institut de Recherche en Sciences de la Sant Direction Rgionale de lOuest Bobo-Dioulasso Burkina Faso Laboratoire de Recherche et dEnseignement en Sant et Biotechnologies Animales Universit Nazi BONI Bobo-Dioulasso Burkina Faso Institut des Sciences et Techniques Bobo-Dioulasso Burkina Faso Laboratoire de Recherche en Bactriologie INSP/Centre MURAZ Bobo-Dioulasso Burkina Faso Maladies Infectueuses et Vecteurs: Ecologie Gntique Evolution et Contrle (MIVEGEC) Institut de Recherche pour le Dveloppement Universit de Monpellier Montpellier France

YIKI is an antimalarial phytomedicine used by a traditional healer to treat malaria in Bobo-Dioulasso. However, there is no scientific evidence to support its use by local populations. The aim of this study was to identify the medicinal practices of the healer holder of YIKI and assess the clinical evidence of its phytomedicine in the uncomplicated malaria treatment. Ethnomedical survey based on a semi-structured and open questionnaire was conducted from October to December 2019 with the healer. Malaria knowledge and diagnosis methods, patient treatment and monitoring, and recipe formulation steps were surveyed. Moreover, thick and thin blood smears were taken, haemoglobin levels and temperature of consenting patients were measured before treatment, mid-treatment and at the end of treatment. The survey revealed that the healer has a good knowledge of malaria symptoms and his diagnosis is based on observation and physical examination of patients. The healer’s malaria diagnosis was rudimentary and had accuracy problems, with only 62.79% of malaria cases confirmed by microscopy. The formulation of YIKI and its use to treat malaria follow a standard process for plant harvesting, powder quantities and posology, but do not use any reproducible parameters for dose adjustment. Forty-three patients diagnosed and treated by the healer participated in the study. Laboratory results revealed 27 Plasmodium falciparum infection cases, including 2 with parasitaemia ≥ 200,000 p/µl blood. 25 patients were selected for therapeutic evidence assessment. There was a 48% elimination of parasites, a 28% parasitaemia decrease without complete cure, with gametocytogenesis in some patients, and a 24% parasitaemia increase. Haemoglobin and temperature results suggested that YIKI was not cytotoxic and reduced fever. Encouraging preliminary results have been obtained, but in view of the low number of patients, further YIKI efficacy and toxicity studies will be necessary for patient safety.

关键词： Antimalarial-Phytomedicine Efficacy Traditional Practices Bobo-Dioulasso

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Modeling and Control of Parallel Hybrid Electric Vehicle Using Sea-Lion Optimization

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Intelligent Automation & Soft Computing 2023年第2期35卷 1441-1454页

作者： j.Leon bosco Raj M.Marsaline Beno Department of EEE St.Xavier’s Catholic College of EngineeringChunkankadaiAnna UniversityChennaiIndia St.Xavier’s Catholic College of Engineering ChunkankadaiAnna UniversityChennaiIndia

This paper develops a parallel hybrid electric vehicle(PHEV)propor-tional integral controller with driving *** improve fuel efﬁciency and reduce hazardous emissions in hybrid electric vehicles(HEVs)combine an electric motor(EM),a battery and an internal combustion engine(ICE).The electric motor assists the engine when accelerating,driving longer highways or climbing *** enables the use of a smaller,more efﬁcient *** also makes use of the concept of regenerative braking to maximize energy efﬁ*** a Hybrid Electric Vehicle(HEV),energy dissipated while braking is utilized to charge the *** proportional integral controller was used in this paper to analyze engine,motor performance and the New European Driving Cycle(NEDC)was used in the vehicle driving test using Matlab/*** proportional integral controllers were designed to track the desired vehicle speed and manage the vehi-cle’s energyﬂ*** Sea Lion Optimization(SLnO)methods were created to reduce fuel consumption in a parallel hybrid electric vehicle and the results were obtained for the New European Driving Cycle.

关键词： Hybrid electric vehicle(HEV) proportional integral controller parallel HEV fuel efﬁciency new European driving cycle(NEDC) sea lion optimization(SLnO)

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Watershed management index based on the village watershed model (VWM) approach towards sustainability

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International Soil and Water Conservation Research 2020年第1期8卷 35-46页

作者： Ignatius Sriyana j.G.De Gijt Sri Kumala Parahyangsari john bosco Niyomukiza Department of Civil Engineering Faculty of EngineeringDiponegoro UniversitySemarangIndonesia Department of Hydraulic Engineering and Flood Risk Delft University of Technology TUNetherlands Member of the Watershed Coordination Forum in Central java Province Central JavaIndonesia Department of Civil Engineering Faculty of EngineeringNdejje UniversityUganda

In the current study,we examine the Indonesian government's watershed management program,which was established in *** 2005,the Coordination Team for Rescue of Water Resources(CTRWR)was established to execute the program on a national ***,at the time,field implementation was a sectoral interest due to the lack of program *** this end,the Indonesian government pro-moted integrated watershed management in 2009,which since then has been implemented by all stakeholders(in Top-Down management form),with application limited to preparing and planning *** is mainly driven by the stakeholders'lack of understanding with regard to watershed systems as integrated management *** implementation results have not yet been realized,including the promotion of community-based watershed management(through Bottom-Up manage-ment).The purpose of our research was to determine the index numbers by measuring the level of cooperation between watershed management workers based on the Village Watershed Model(VWM)specifically surface water which includes six variables:planning,participation,institutional,fund sharing,gender,and management *** method used was an ordinal measure with the Likert *** data showed successful watershed management,in which five of the six VWM varia-bles—planning,participation,institutional,fund sharing,and management systems—were in the"good"category with indices ranging from 73.08 to *** gender variable index(69.12)was in the"me-dium"category.

关键词： Management Index Village watershed Model Sustainability

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Using emotions for the development of human-agent societies

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Frontiers of Information Technology & Electronic Engineering 2016年第4期17卷 325-337页

作者： j.A.RINCON j.BAjO A.FERNANDEZ V.julian C.CARRASCOSA Department of Computer Systems and Computation Universitat Politecnica de Valencia Departamento de Inteligencia Artificial Universidad Politecnica de Madrid Centre for Intelligent Information Technologies Universidad Rey Juan Carlos

Human-agent societies refer to applications where virtual agents and humans coexist and interact transparently into a fully integrated environment. One of the most important aspects in this kind of applications is including emotional states of the agents(humans or not) in the decision-making process. In this sense, this paper presents the applicability of the jaCalIVE(jason Cartago implemented intelligent virtual environment) framework for developing this kind of society. Specifically, the paper presents an ambient intelligence application where humans are immersed into a system that extracts and analyzes the emotional state of a human group. A social emotional model is employed to try to maximize the welfare of those humans by playing the most appropriate music in every moment.

关键词： Multi-agent systems Virtual environments Emotional agents

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Multiple forest structural elements are needed to promote beetle biomass, diversity and abundance

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Forest Ecosystems 2022年第5期9卷 585-595页

作者： Nolan j.Rappa Michael Staab julian Frey Nathalie Winiger Alexandra-Maria Klein Nature Conservation and Landscape Ecology University of FreiburgTennenbacherstrasse 479106Freiburg im BreisgauGermany Ecological Networks Technical University DarmstadtSchnittspahnstrasse 364287DarmstadtGermany Forest Growth and Dendroecology University of FreiburgTennenbacherstrasse 479106Freiburg im BreisgauGermany Wildlife Ecology and Management University of FreiburgTennenbacherstrasse 479106Freiburg im BreisgauGermany

Background:Retention forestry is a management strategy aiming to mitigate biodiversity loss by retaining structural elements such as dead trees that would otherwise be *** we analyze the biomass,diversity and abundance among forest beetles collected using window traps on 1281-ha forest sites reflecting gradients in the amount of structural elements in southwestern ***:We found that beetle biomass increased with mean diameter at breast height(a measure of tree size),and decreased with stand structural *** of individual feeding guilds responded differently to forest structural elements,namely lying deadwood,understory complexity,tree basal area and stand structural *** family diversity increased with the effective number of layers,i.e.1-m forest strata occupied by vegetation assessed via terrestrial laser *** of feeding guilds responded to only elevation and share of deciduous *** composition in terms of biomass was structured by forest elements similar to biomass of individual feeding guilds,with the addition of lying *** differed from community composition in terms of abundance of feeding guilds,which was structured by primarily standing deadwood volume and share of deciduous ***:Our results show that biomass,diversity and abundance respond differently to forest structural *** suggests that the concurrent prioritization of multiple forest elements is needed to promote forest beetles,with more focus placed on the differing resource needs among feeding *** addition,retention strategies should also consider the varying responses of beetle biodiversity metrics when assessing the importance of forest structural elements.

关键词： Allometry Biodiversity Coleoptera Forest management Terrestrial laser scanning Window trap

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