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Targeted therapy for capillary-venous malformations

Signal Transduction and Targeted Therapy 2024年第7期9卷 3024-3039页

作者： Lola Zerbib Sophia Ladraa Antoine Fraissenon Charles Bayard Marina Firpion Quitterie Venot Sanela Protic Clément Hoguin Amandine Thomas Sylvie Fraitag Jean-Paul Duong Sophie Kaltenbach Estelle Balducci coline Lefevre Patrick Villarese Vahid Asnafi Christine Broissand Nicolas Goudin Ivan Nemazanyy Gwennhael Autret Bertrand Tavitian Christophe Legendre Nadia Arzouk veronique minard-colin Caroline Chopinet Michael Dussiot Denise MAdams Tristan Mirault Laurent Guibaud Paul Isenring Guillaume Canaud UniversitéParis Cité ParisFrance INSERM U1151 Institut Necker-Enfants MaladesParisFrance Service d’Imagerie Pédiatrique Hôpital Femme-Mère-EnfantHCLBronFrance CREATIS UMR 5220 Villeurbanne 69100France Service de Radiologie Mère-Enfant Hôpital NordSaint EtienneFrance Service d’Anatomie pathologique Hôpital Necker-Enfants MaladesAP-HPParisFrance Laboratoire d’Oncohématologie Hôpital Necker-Enfants MaladesAP-HPParisFrance Pharmacie Hôpital Necker-Enfants MaladesAP-HPParisFrance Necker Bio-Image Analysis INSERM US24/CNRS UMS 3633ParisFrance Platform for Metabolic Analyses Structure Fédérative de Recherche NeckerINSERM US24/CNRS UMS 3633ParisFrance Plateforme Imageries du Vivant Universitéde ParisPARCCINSERMParisFrance Service de Néphrologie Transplantation AdultesHôpital Necker-Enfants MaladesAP-HPParisFrance Service de Transplantation Hôpital La PitiéSalpétrièreAP-HPParisFrance Department of Pediatric and Adolescent Oncology INSERM 1015Gustave RoussyUniversitéParis-SaclayVillejuifFrance Service de Physiologie&Explorations Fonctionnelles Cardiovasculaires CHU de LilleLille 59000France INSERM U1163 Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutic ImplicationsLaboratoire d’Excellence GR-ExParisFrance Division of Oncology Comprehensive Vascular Anomalies ProgramChildren’s Hospital of PhiladelphiaPhiladelphiaPAUSA Service de Médecine Vasculaire hôpital Européen Georges-PompidouParisFrance Nephrology Research Group L’Hôtel-Dieu de Québec Research CenterDepartment of MedicineFaculty of MedicineLaval UniversityQuebecQC G1R2J6Canada Unitéde médecine translationnelle et thérapies ciblées Hôpital Necker-Enfants MaladesAP-HPParisFrance CNRS UMR8253 Institut Necker-Enfants MaladesParisFrance

Sporadic venous malformations are genetic conditions primarily caused by somatic gain-of-function mutation of PIK3CA or TEK,an endothelial transmembrane receptor signaling through *** malformations are associated with pain,bleedings,thrombosis,pulmonary embolism,esthetic deformities and,in severe cases,life-threatening *** authorized medical treatment exists for patients with venous ***,we created a genetic mouse model of PIK3CA-related capillary venous malformations that replicates patient *** showed that these malformations only partially signal through AKT *** compared the efficacy of different drugs,including rapamycin,a mTORC1 inhibitor,miransertib,an AKT inhibitor and alpelisib,a PI3Kαinhibitor at improving the lesions seen in the mouse *** demonstrated the effectiveness of alpelisib in preventing vascular malformations’occurrence,improving the already established ones,and prolonging *** these findings,we were authorized to treat 25 patients with alpelisib,including 7 children displaying PIK3CA(n=16)or TEK(n=9)-related capillary venous malformations resistant to usual therapies including sirolimus,debulking surgical procedures or percutaneous *** assessed the volume of vascular malformations using magnetic resonance imaging(MRI)for each *** demonstrated improvement in all 25 *** malformations previously considered intractable were reduced and clinical symptoms were *** showed a decrease of 33.4%and 27.8%in the median volume of PIK3CA and TEK malformations respectively,over 6 months on *** conclusion,this study supports PI3Kαinhibition as a promising therapeutic strategy in patients with PIK3CA or TEK-related capillary venous malformations.

关键词： PIK3CA malformations venous

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Defense and senescence interplay in legume nodules

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Plant Communications 2024年第4期5卷 112-126页

作者： Fathi Berrabah Farouk Benaceur Chaoyan Yin Dawei Xin Ke´vin Magne Marie Garmier veronique Gruber Pascal Ratet Faculty of Sciences University Amar Telidji03000 LaghouatAlgeria Research Unit of Medicinal Plants(RUMP) National Center of Biotechnology ResearchCRBt25000 ConstantineAlgeria Universite´Paris-Saclay CNRSINRAEUniversity of EvryInstitute of Plant Sciences Paris-Saclay(IPS2)91190 Gif-sur-YvetteFrance Universite´Paris Cite´ Institute of Plant Sciences Paris-Saclay(IPS2)91190 Gif-sur-YvetteFrance Key Laboratory of Soybean Biology in the Chinese Ministry of Education College of AgricultureNortheast Agricultural UniversityHarbin 150030China These authors contributed equally to this article.

Immunity and senescence play a crucial role in the functioning of the legume symbiotic *** miss-regulation of one of these processes compromises the symbiosis leading to death of the endosymbiont and the arrest of the nodule *** relationship between immunity and senescence has been exten-sively studied in plant organs where a synergistic response can be ***,the interplay be-tween immunity and senescence in the symbiotic organ is poorly discussed in the literature and these phe-nomena are often mixed *** studies revealed that the cooperation between immunity and senescence is not always observed in the nodule,suggesting complex interactions between these two pro-cesses within the symbiotic ***,we discuss recent results on the interplay between immunity and senescence in the nodule and the specificities of this relationship during legume-rhizobium symbiosis.

关键词： immunity senescence symbiosis nodules legumes rhizobia

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Nearest Neighbor Sampling of Point Sets Using Rays

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Communications on Applied Mathematics and Computation 2024年第2期6卷 1131-1174页

作者： Liangchen Liu Louis Ly colin B.Macdonald Richard Tsai Department of Mathematics The University of Texas at Austin2515 SpeedwayAustinTX 78712USA Oden Institute for Computational Engineering and Sciences The University of Texas at Austin201 E24th StAustinTX 78712USA Department of Mathematics University of British Columbia1984 Mathematics RdVancouverBCV6T1Z2Canada

We propose a new framework for the sampling,compression,and analysis of distributions of point sets and other geometric objects embedded in Euclidean *** approach involves constructing a tensor called the RaySense sketch,which captures nearest neighbors from the underlying geometry of points along a set of *** explore various operations that can be performed on the RaySense sketch,leading to different properties and potential *** information about the data set can be extracted from the sketch,independent of the ray *** integrals on point sets can be efficiently computed using the *** also present several examples illustrating applications of the proposed strategy in practical scenarios.

关键词： Point clouds Sampling Classification Registration Deep learning Voronoi cell analysis

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Recent Progress of the Solar Wind Magnetosphere Ionosphere Link Explorer (SMILE) Mission

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空间科学学报 2024年第4期44卷 692-698页

作者： WANG Chi ESCOUBET Philippe FORSYTH colin LI Jing AGNOLON David State Key Laboratory of Space Weather National Space Science CenterChinese Academy of SciencesBeijing 100190 European Space Agency/European Space Research and Technology Centre Noordwijk 2201 AZ Mullard Space Science Laboratory University College LondonLondon RH56 NT

The SMILE(Solar wind Magnetosphere Ionosphere Link Explorer)mission is a joint space science mission between the Chinese Academy of Sciences(CAS)and the European Space Agency(ESA),aiming to understand the interaction of the solar wind with the Earth’s magnetosphere in a global *** of May 2024,the SMILE mission is in phase-D with an expected launch date of September *** report summarizes developments in the mission during the past two years.

关键词： SMILE Soft X-ray Imager Ultra-Violet Imager Light Ion Analyzer Magnetometer

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Chitin-glucan improves important pathophysiological features of irritable bowel syndrome

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World Journal of Gastroenterology 2024年第16期30卷 2258-2271页

作者： Caroline Valibouze Caroline Dubuquoy Philippe Chavatte Michaël Genin veronique Maquet Salvatore Modica Pierre Desreumaux Christel Rousseaux Department of Digestive Surgery and Transplantation Lille UniversityLille 59037France Intestinal Biotech Development Facultéde MédicineLille 59045France U1286-INFINITE-Institute for Translational Research in Inflammation Universitéde LilleLille 59000France ULR 2694-METRICS Évaluation des Technologies de santéet des Pratiques MédicalesUniversity of LilleLille 59000France KitoZyme SA Institution SociétéAnonymeZone 2Parc des Hauts SartsRue de MilmortHerstal 4040Belgium Hepato-Gastroenterology Department Lille University HospitalLille 59037France

BACKGROUND Irritable bowel syndrome(IBS)is one of the most frequent and debilitating conditions leading to gastroenterological ***,recommended treatments remain limited,yielding only limited therapeutic ***-glucan(CG)is a novel dietary prebiotic classically used in humans at a dosage of 1.5-3.0 g/d and is considered a safe food ingredient by the European Food Safety *** provide an alternative approach to managing patients with IBS,we performed preclinical molecular,cellular,and animal studies to evaluate the role of chitin-glucan in the main pathophysiological mechanisms involved in *** To evaluate the roles of CG in visceral analgesia,intestinal inflammation,barrier function,and to develop computational molecular *** Visceral pain was recorded through colorectal distension(CRD)in a model of long-lasting colon hypersensitivity induced by an intra-rectal administration of TNBS[15 milligrams(mg)/kilogram(kg)]in 33 Sprague-Dawley *** pressure was regularly assessed during the 9 wk-experiment(weeks 0,3,5,and 7)in animals receiving CG(n=14)at a human equivalent dose(HED)of 1.5 g/d or 3.0 g/d and compared to negative control(tap water,n=11)and positive control(phloroglucinol at 1.5 g/d HED,n=8)*** anti-inflammatory effect of CG was evaluated using clinical and histological scores in 30 C57bl6 male mice with colitis induced by dextran sodium sulfate(DSS)administered in their drinking water during 14 ***-29 cells under basal conditions and after stimulation with lipopolysaccharide(LPS)were treated with CG to evaluate changes in pathways related to analgesia μ-opioid receptor(MOR),cannabinoid receptor 2(CB2),peroxisome proliferator-activated receptor alpha,inflammation[interleukin(IL)-10,IL-1b,and IL-8]and barrier function[mucin 2-5AC,claudin-2,zonula occludens(ZO)-1,ZO-2]using the real-time PCR *** modelling of CG,LPS,lipoteichoic acid(LTA),and phospholipomannan(PLM)was developed,and the abili

关键词： Chitin-glucan Irritable bowel syndrome Abdominal pain Inflammation Intestinal barrier Molecular modelling Microbial cell walls chelation

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Thin-film flow technology in controlling the organization of materials and their properties Special Collection:Distinguished Australian Researchers

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Aggregate 2024年第1期5卷 115-129页

作者： Clarence Chuah Xuan Luo Javad Tavakoli Youhong Tang colin L.Raston Flinders Institute for Nanoscale Science and Technology College of Science and EngineeringFlinders UniversityAdelaideSouth AustraliaAustralia Centre for Health Technologies School of Biomedical EngineeringFaculty of Engineering and Information TechnologyUniversity of Technology SydneySydneyNew South WalesAustralia

Centrifugal and shear forces are produced when solids or liquids *** systems and devices that use these forces,such as dynamic thin-film flow technology,are evolving continuously,improve material structure-property relationships at the nanoscale,representing a rapidly thriving and expanding field of research high with green chemistry metrics,consolidated at the inception of *** vortex fluidic device(VFD)provides many advantages over conventional batch processing,with fluidic waves causing high shear and producing large surface areas for micro-mixing as well as rapid mass and heat transfer,enabling reactions beyond diffusion *** these abilities allows for a green and innovative approach to altering materials for various research and industry applications by controlling small-scale flows and regulating molecular and macromolecular chemical reactivity,self-organization phenomena,and the synthesis of novel *** review highlights the aptitude of the VFD as clean technology,with an increase in efficiency for a diversity of top-down,bottom-up,and novel material transformations which benefit from effective vortex-based processing to control material structure-property relationships.

关键词： material nanoscale structure-property relationship thin film flow technology vortex fluidic device

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Chemical-assisted MMP reduction on methane-oil systems:Implications for natural gas injection to enhanced oil recovery

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Petroleum 2024年第1期10卷 101-108页

作者： Mohamed Almobarak Matthew B.Myers colin D.Wood Yongbing Liu Ali Saeedi Quan Xie Discipline of Petroleum Engineering WA School of Mines:MineralsEnergy and Chemical EngineeringCurtin UniversityWestern AustraliaAustralia Commonwealth Scientific and Industrial Research Organization Kensington6151Australia Curtin University Oil and Gas Innovation Centre Management Kensington6151Australia State Key Laboratory of Oil and Gas Reservoir Geology and Exploitation No.8 Xindu AvenueXindu DistrictChengdu 610500PR China

***,the main challenge in this process is the high minimum miscibility pressure(MMP)between natural gas and crude oil,which limits its application and recovery factor,especially in hightemperature ***,we present a novel investigation to quantify the effect of chemicalassisted MMP reduction on the oil recovery ***,we measured the interfacial tension(IFT)of the methane-oil system in the presence of chemical or CO_(2) to calculate the MMP reduction at a constant temperature(373K)using the vanishing interfacial tension(VIT)***,we performed three coreflooding experiments to quantify the effect of MMP reduction on the oil recovery factor under different injection *** interfacial tension measurements show that adding a small fraction(1.5 wt%)of the tested surfactant(SOLOTERRA ME-6)achieved 9%of MMP reduction,while adding 20 wt%of CO_(2) to the methane yields 13%of MMP ***,the coreflooding results highlight the significance of achieving miscibility during gas injection,as the ultimate recovery factor increased from 65.5%under immiscible conditions to 77.2%using chemical-assisted methane,and to 79%using gas mixture after achieving near miscible *** results demonstrate the promising potential of the MMP reduction to signifi-cantly increase the oil recovery factor during gas ***,these results will likely expand the application envelop of the miscible gas injection,in addition to the environmental benefits of utilizing the produced gas by re-injection/recycling instead of flaring which contributes to reducing the greenhouse gas emissions.

关键词： Gas injection Enhanced oil recovery Miscibility Coreflooding

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The Influence of Variable (Monsoon) Rainfall on Sedimentation in the Roaches Grit and Other Upper Carboniferous Delta Sequences in the UK Pennine Basin

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International Journal of Geosciences 2024年第8期15卷 632-666页

作者： colin Michael Jones Retired Cambridge UK

The Roaches Grit in the UK Pennine Basin was a complex deep water deltaic sequence deposited during the Late Carboniferous glacial period. The channels of the upper part of the Roaches Grit, deposited towards the end of the cyclothem after the eustatic minimum, contain evidence for very high seasonal discharges related to strong monsoon rainfall in the catchment areas. In some channels, intense turbulence near the delta front, led to knick point recession and deep incision. These channels were filled with sediments during reduced discharge, including very large sets of cross-bedding up to 16 m thick. Channels were short-lived with frequent avulsions. Over time slightly lower discharges formed laterally migrating channels dominated by bar forms. Different discharge-controlled processes operated on the reactivated delta slope. Incised channels generated turbidity currents during floods which transported sediments directly into the basin far from the delta. Migrating channels built mouth bars;resedimentation during floods formed density currents which then deposited sediment on the lower parts of the slope.

关键词： Carboniferous Delta Climate Monsoon Rainfall ITCZ Precession

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Dissolved Organic Matter Features of Three Adjacent Eastern Mediterranean Urbanized Watersheds

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Open Journal of Modern Hydrology 2024年第3期14卷 153-173页

作者： Nour Abboud Elias Michel Maatouk Zeinab Matar veronique Kazpard Research and Analysis Platform for Environmental Sciences (PRASE) Doctoral School of Sciences and Technology (EDST) The Lebanese University Rafic Hariri Campus Hadat Lebanon Laboratory of Georessources Geosciences and Environment (L2GE) Faculty of Sciences The Lebanese University Fanar Lebanon Department of Earth and Life Sciences Faculty of Sciences The Lebanese University Hadat Lebanon

Landscape urbanization broadly affects ecosystems in coastal watersheds, but, until now, the influence of nonpoint source urban inputs on dissolved organic matter (DOM) amount, composition, and source is poorly understood. To understand how DOM composition varied with urbanization, fluorescence excitation-emission matrices (EEMs) were determined for urban and non-urban waters from upstream to downstream sites along three adjacent coastal watersheds that flow into the Mediterranean Sea. Two humic DOM fluorescent components (humic-like and fulvic-like peaks) and two proteinic components (tyrosine-like and tryptophane-like peaks) were identified by EEM fluorescence. The results indicated that urbanization had an important influence on DOM concentration and composition, with urban waters having a high degree of DOM variation due to different land uses surrounding each body of water. Urban waters show a higher DOM fluorescence index (FI), the highest fluorescence intensity of protein-like manifested also by BIX values, and a lower value of the humification index (HIX) than non-urban waters which were dominated by allochthonous inputs. In addition, the EEM was compared in dry and wet season where higher DOM amounts and FI appeared in summer due to autochthonous production coming from algae growth compared to allochthonous input from rainfall dominated in wet season. The concentration of DOC increased from upstream to downstream for the three rivers, especially Beirut River. The increase in DOC values was observed in both dry and wet seasons by 39 and 19 times respectively compared to upstream (0.93 - 0.91 mgC/L).

关键词： Dissolved Organic Matter DOM EEM Fluorescence Spectroscopy Autochthonous/Allochthonous DOM Urbanization Upstream/Downstream Wet/Dry Season

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Comparative Study of the Types of Photovoltaic Panels Used in Solar Pumping Systems in Dry Tropical Zones: Case of Adamawa Region in Cameroon

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Open Journal of Energy Efficiency 2024年第3期13卷 79-87页

作者： Boussaibo Andre Waoudowa Kande veronique Ndjiya Ngasop Stephane Gautier Kitmo Department of Electrical Engineering University Institute of Ngaoundr Ngaoundr Cameroon Department of Electrical Energy and Automation National School of Agro-Industrial Sciences Ngaoundr Cameroon Department of Physics University of Ngaoundr Ngaoundr Cameroon

In the dry tropical zone where access to water is increasingly difficult for populations, solar pumping units are increasingly installed to provide water to population. In the local market, there are essentially two types of solar panels, namely monocrystalline and polycrystalline. However, the part of the local market is more dominated by the polycrystalline panel. In this work, comparative studies are carried out in order to characterize the two types of solar panels with regard to local constraints. Tests were carried out over the course of the sun to establish the performance of each type. The panels used have the same electrical characteristics and are connected to loads with same characteristics. Under the set operating conditions, the monocrystalline panel presents more performance than the polycrystalline panel. Although the local market is dominated by the polycrystalline panel, dust deposition tests on the surface of the panels show that the performance of the polycrystalline panel is more affected compared to the performance of the monocrystalline panel.

关键词： Solar Pumping Monocrystalline Cell Polycrystalline Cell Dry tropical Zone Dustdeposit

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