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Targeted therapy for capillary-venous malformations

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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

Wireless and batteryless SAW sensors,a promising solution for harsh environments

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电子测量与仪器学报 2011年第7期25卷 582-590页

作者： H.M'Jahed T.Aubert G.Prieur O.Elmazria institut jean lamour UMR 7198 CNRS-Nancy University 54506 Vandoeuvre lès NancyFrance

In this paper,general principle of the Surface Acoustic Wave(SAW) sensor in wired and wireless con-figurations will be developed and a review of recent works concerning the field of high temperature applications will be *** first part will be devoted to aspects of data transmission and *** configurations of SAW de-vice,delay line and resonator,will be discussed as well as the remote interrogation techniques used to collect and to proc-ess *** second part will be devoted to the material ***,knowing that the conventional piezoelectric substrates such as quartz or lithium niobate cannot be used at high temperature,the choice of the material constituting the SAW device(substrate & electrodes) is one of the challenges to *** will focus our discussion on the Langasite,the current reference for high temperature applications,and on the AlN/Sapphire structure,the very promising alternative for application where the use of high frequency is required.

关键词：摘要编辑部编辑工作读者作者

Building 2D quasicrystals from 5-fold symmetric corannulene molecules

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Nano Research 2018年第4期11卷 2129-2138页

作者： Nataliya Kalashnyk Julian Ledieu Emilie Gaudry Can Cui An-Pang Tsai Vincent Fournee institut jean lamour umr 7198 cnrs - Universite de Lorraine Campus ARTEM - 2 allde Andre Guinier F-54000 NANCY France Department of Physics Zhejiang Sci-Tech University Hangzhou 310018 China institute of Multidisciplinary Research for Advanced Materials (IMRAM) Tohoku University 2-1-1 Katahira Aoba-ku Sendai 980-8577 Japan

The formation of long-range ordered aperiodic molecular films on quasicrystalline substrates is a new challenge that provides an opportunity for further surface functionalization. This aim can be realized through the smart selection of molecular building blocks, based on symmetry-matching between the underlying quasicrystal and individual molecules. It was previously found that the geometric registry between the C60 molecules and the 5- and 10-fold surfaces was key to the growth of quasiperiodic organic layers. However, an attempt to form a quasiperiodic C60 network on i-Ag-In-Yb substrates was unsuccessful, resulting in disordered molecular films. Here we report the growth of 5-fold symmetric corannulene C20H10 molecules on the 5-fold surfaces of i-Ag-In-Yb quasicrystals. Low-energy electron diffraction （LEED） and scanning tunneling microscopy （STM） revealed long-range quasiperiodic order and 5-fold rotational symmetry in self-assembled corannulene films. Recurrent decagonal molecular rings were seen, resulting from the decoration of specific adsorption sites with local pentagonal symmetry by corannulenes, adsorbed with their bowl-openings pointing away from the surface. They were identified as （Ag, In）-containing rhombic triacontahedral （RTH） duster centers and pentagonal Yb motifs, which cannot be occupied simultaneously due to steric hindrance. It is proposed that symmetry-matching between the molecule and specific substrate sites drives this organization. Alteration of the molecular rim by the introduction of CH substituents appeared to increase molecule mobility on the potential energy surface and facilitate trapping at these specific sites. This finding suggests that rational selection of molecular moiety enables the templated self-assembly of molecules leading to an ordered aperiodic corannulene layer.

关键词： quasicrystal,surface science 5-fold symmetry molecular self-assembly,corannulene

Modeling of a Wireless SAW Temperature Sensor and Associated Antenna

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Instrumentation 2014年第1期 8-14页

作者： Laurent ALLIES Eloi BLAMPAIN Hamid M'JAHED Gérard PRIEUR Omar ELMAZRIA institut jean lamour UMR 7198 CNRS-Université de Lorraine 54506 Vandoeuvre lès NancyFrance

Surface acoustic wave( SAW) resonator used as wireless sensor was characterized and the parameters of its MBVD(Modified Butterworth-Van Dyke) model were extracted versus temperature. The extracted parameters lead toevaluate the resonator performancesin terms of Temperature coefficient of frequency(TCF) and quality factor(Q). An antenna was then associated with the SAW resonator and the entire system has been characterized and modeled. The good agreement experiment-simulation allows to define the optimum operating conditions of the wireless sensor.

关键词： SAW resonator MBVD model Wireless sensor

Recent Advances on Graphene Quantum Dots for Electrochemical Energy Storage Devices

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Energy & Environmental Materials 2022年第1期5卷 201-214页

作者： Noura Zahir Pierre Magri Wen Luo jean Jacques Gaumet Philippe Pierrat cnrs L2CM UMR 7053 Universit e de Lorraine LCP-A2MC EA4632 Institut Jean Barriol Universit e de Lorraine State Key Laboratory of Advanced Technology for Materials Synthesis and Processing Wuhan University of Technologywuhan 430070china

Graphene quantum dots(GQDs)which are nanofragments of graphene with an average size between 2 and 50 nm have attracted much attention due to their outstanding properties such as high conductivity,high surface area,and good solubility in various *** combine the quantum confinement and edges effects and the properties of ***,GQDs offers a broad range of applications in various fields(medicine,energy conversion,and energy storage devices).This review will present the recent research based on the introduction of GQDs in batteries,supercapacitors,and microsupercapacitors as electrodes materials or mixed with an active material as an auxiliary ***,discussed on selected examples,summarize the electrochemical performances and finally,challenges and perspectives are recalled for the subsequent optimization strategy of electrode *** review is expected to appeal a broad interest on functional GQDs materials and promote the further development of high-performance energy storage device.

关键词： energy storage graphene quantum dots lithium ion batteries sodium ion batteries supercapacitor

Wafer-level vapor cells filled with laser-actuated hermetic seals for integrated atomic devices

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Microsystems & Nanoengineering 2022年第6期8卷 225-235页

作者： Vincent Maurice Clément Carlé Shervin Keshavarzi Ravinder Chutani Samuel Queste Ludovic Gauthier-Manuel jean-Marc Cote Rémy Vicarini Moustafa Abdel Hafiz Rodolphe Boudot Nicolas Passilly IEMN-institut d’Electronique de Microélectronique et de Nanotechnologie UMR8520 CNRSUniversitéLilleCentrale LilleF-59000 LilleFrance FEMTO-ST institute UMR6174 CNRSUniversitéBourgogne Franche-ComtéF-25030 BesançonFrance

Atomic devices such as atomic clocks and optically-pumped magnetometers rely on the interrogation of atoms contained in a cell whose inner content has to meet high standards of purity and ***-blowing techniques and craftsmanship have evolved over many decades to achieve such standards in macroscopic vapor *** the emergence of chip-scale atomic devices,the need for miniaturization and mass fabrication has led to the adoption of microfabrication techniques to make millimeter-scale vapor ***,many shortcomings remain and no process has been able to match the quality and versatility of glass-blown ***,we introduce a novel approach to structure,fill and seal microfabricated vapor cells inspired from the century-old approach of glass-blowing,through opening and closing single-use zero-leak microfabricated *** valves are actuated exclusively by laser,and operate in the same way as the“make-seals”and“break-seals”found in the filling apparatus of traditional *** structures are employed to fill cesium vapor cells at the *** make-seal structure consists of a glass membrane that can be locally heated and deflected to seal a *** break-seal is obtained by breaching a silicon wall between *** new approach allows adapting processes previously restricted to glass-blown *** can also be extended to vacuum microelectronics and vacuum-packaging of micro-electro-mechanical systems(MEMS)devices.

关键词： filled vacuum heated

Influence of Electromagnetic Stirring on the Macrosegregation in Arc Remelted Ti and Zr Alloy Ingots

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Journal of Iron and Steel Research(International) 2012年第S1期19卷 199-202页

作者： Alain Jardy Mathieu Revil-Baudard Hervé Combeau Universite de Lorraine Institut Jean Lamour-Dépt.SI2MUMR CNRS 7198Ecole des MinesParc de SauruptNancy F-54042France

A numerical model of the VAR process has been developed in close collaboration with titanium and zirconium *** model is based on the solution of the coupled heat,mass and momentum transport equations in the whole ingot(liquid pool,mushy zone and solidified part)using a finite volume *** accounts for electromagnetic stirring, buoyancy flows and turbulence *** mechanisms implemented in the model include a full coupling between energy and solute transport in the mushy *** numerical model has been applied to simulate the remelting of *** influence of the applied magnetic field on the fluid flow and segregation behaviour,for a number of different stirring practices,is presented and ***,the macrosegregation in Zy4 ingots is *** comparison between the predicted segregation and the experimental results shows the importance of accounting for both the stirring and thermosolutal convection to forecast properly the segregation in remelted ingots.

关键词： Vacuum Arc Remelting simulation zirconium titanium macrosegregation stirring

Stable NiO-CeO_(2) nanoparticles with improved carbon resistance for methane dry reforming

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Journal of Rare Earths 2022年第1期40卷 57-62页

作者： Andrea Cárdenas-Arenas Esther Bailón-García Dolores Lozano-Castelló Patrick Da Costa Agustín Bueno-López Department of Inorganic Chemistry University of AlicanteCarretera de San Vicente s/nE03080AlicanteSpain Sorbonne Universite Institut Jean Le Rond d'AlembertCNRS UMR 71902 place de la gare de ceinture78210Saint Cyr L'EcoleFrance

High surface area mixed oxide 8.7%NiO-CeO_(2) nanoparticles(122 m2/g;6-7 nm)were prepared using a reversed microemulsion method,and were tested for dry methane reforming(DRM).The catalytic activity of these nanoparticles remains stable under the severe conditions of DRM(700℃),and they show better carbon resistance than conventional NiO-CeO_(2) catalysts prepared without control of the *** activity and selectivity of nanoparticles and reference catalyst are similar,but nanoparticles reduce the accumulation of carbon by 63%during the DRM tests,which is a key feature for this *** and H_(2)-TPR suggest that the improved carbon resistance of the nanoparticles is due to the better interaction and cooperation between NiO and CeO_(2) mixed *** nanoparticles,the participation of cerium cations in the redox processes taking place during DRM stabilizes cationic species of *** the contrary,the catalyst prepared without control of the size suffers segregation of Ni during DRM reaction,and segregated Ni explains the higher catalytic formation of carbon.

关键词： Methane dry reforming Nickel Ceria Nanoparticle Rare earths

Best practices for model-based and simulation-aided engineering of power transmission and motion control systems

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Chinese Journal of Aeronautics 2019年第1期32卷 186-199页

作者： jean-Charles MARE institut Clément Ader (cnrs umr 5312)

This paper deals with the modelling and simulation of aircraft systems, in particular for power transmission and control. It is intended to review, propose and disseminate best practices for making model-based/simulation-aided engineering more efficient at any phase of the system life cycle. The proposals are aimed at creating value, not only by increasing the performance of the product under study but also by shortening the time to market, capitalizing knowledge, mitigating risks and facilitating concurrent engineering. The needs associated with the engineering activities are firstly identified to define a set of requirements for the models. Then, these requirements are used to drive the considerations leading to model development, focusing in particular on the process,modelled physical effects, modelling level, model architecting and concurrent engineering. The third part deals with the model implementation, giving special consideration to the different types of models, causalities, parameterization, implementation and verification. Each part is illustrated by examples related to safety critical actuators.

关键词： Actuator Aerospace Architecture Causality Modelling Motion control Power transmission Simulation