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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

Design of Metal-Organic Assemblies via Shape Complementarity and Conformational Constraints in Dual Curvature Ligands

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CCS Chemistry 2023年第11期5卷 2506-2518页

作者： Cui-Lian Liu Eduard O.Bobylev Sébastien Dauriac Brice Kauffmann Koen Robeyns Yann Garcia Joost N.H.Reek Michael L.Singleton Institute of Condensed matter and Nanosciences Universitécatholique de LouvainLouvain-la-Neuve 1348 Van’t Hoff Institute for Molecular Sciences University of AmsterdamAmsterdam 1098 XH Universitéde Bordeaux cnrs INSERMUMS3033Institut Européen de Chimie et Biologie(IECB)33600 Pessac

While common in biological systems,building blocks with low symmetry and flexibility pose numerous problems for synthetic self-assembly,such as the formation of isomers of assemblies that are difficult to distinguish and *** this work,three aromatic amide-based ligands(L1–L3)with a central 1,8-diazatriptycene core were designed and used for selfassembly with Pd^(2+).While hundreds of stereoisomers based on the conformational flexibility around the amides and the unsymmetrical nonplanar structure of the core are possible upon coordination with the metal,the constraints designed into the ligands direct the self-assembly toward only a single Pd_(2)L_(4)cage(L1)or Pd_(4)L_(8)double-walled metallomacrocycle(L2)structure,even in mixtures of the *** structural approach and the modularity of the ligand synthesis affords ready access to deep cavitands with endohedral functionalization(L3).These results highlight the potential of this new design strategy and open the door to selectively functionalized cavity-based architectures for numerous applications.

关键词： metal-organic cageopis isomer selectivity low-symmetry ligands self-sorting self-assembly

Comment on “Locating the source field lines of Jovian decametric radio emissions” by YuMing Wang et al.

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Earth and Planetary Physics 2022年第1期6卷 10-12页

作者： Laurent Lamy Baptiste Cecconi Stéphane Aicardi C.K.Louis LESIA Observatoire de ParisUniversitéPSLCNRSSorbonne UniversitéUniversitéde Paris5 place Jules Janssen92195 MeudonFrance Station de Radioastronomie de Nançay Observatoire de ParisUniversitéPSLCNRSUniv.Orléans18330 NançayFrance LAM PythéasAix Marseille UniversitéCNRSCNES38 Rue Frédéric Joliot Curie13013 MarseilleFrance DIO UMS2201 CNRSObservatoire de ParisUniversitéPSL61 avenue de l’Observatoire75014ParisFrance School of Cosmic Physics DIAS Dunsink ObservatoryDublin Institute for Advanced StudiesDublinIreland

In this comment on the article“Locating the source field lines of Jovian decametric radio emissions”by Wang YM et al.,2020,we discuss the assumptions used by the authors to compute the beaming angle of Jupiter’s decametric emissions induced by the moon *** method,relying on multi-point radio observations,was applied to a single event observed on 14th March 2014 by Wind and both STEREO A/B spacecraft from~5 to~16 *** have erroneously identified the emission as a northern(Io-B type)instead of a southern one(Io-D type).We encourage the authors to update their results with the correct hemisphere of origin and to test their method on a larger sample of Jupiter-Io emissions.

关键词： planetary magnetosphere Jupiter auroral radio emissions planet-moon interaction

Nanoporous Vesicular Membranes of Amphiphilic Polymers Containing Trans/Cis Isomers

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CCS Chemistry 2022年第8期4卷 2651-2661页

作者： Hui Chen Xia Yu Yujiao Fan Xiangjun Xing Sylvain Trépout Min-Hui Li Beijing Advanced Innovation Center for Soft matter Science and Engineering Beijing University of Chemical TechnologyChaoyang DistrictBeijing 100029 Chimie ParisTech PSL University ParisCNRSInstitut de Recherche de Chimie ParisUMR824775231 Paris Cedex 05 Wilczek Quantum Center School of Physics and AstronomyShanghai Jiao Tong UniversityShanghai 200240 Institut Curie PSL UniversityUniversitéParis-SaclayCNRS UMS2016Inserm US43Multimodal Imaging Center91400 Orsay

Nanoporous membranes and vesicles are interesting systems with potential in applications offering channels for material ***,nanoporous membranes and polymersomes are developed by self-assembly of trans-and cis-stereoisomers of amphiphilic *** polymers,PEG550-TPEChol and PEG550-SS-TPE-SS-Chol,containing a central tetraphenylethene(TPE),a cholesterol(Chol),and a poly(ethylene glycol)(PEG550)are studied.

关键词： amphiphilic polymers stereoisomers aggregation-induced emission self-assembly porous membrane

Benchmarks of 3D Laplace Equation Solvers in a Cubic Configuration for Streamer Simulation

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Plasma Science and Technology 2016年第5期18卷 538-543页

作者： Joseph-Marie PLEWA Olivier DUCASSE Philippe DESSANTE Carolyn JACOBS Olivier EICHWALD Nicolas RENON Mohammed YOUSFI Universite de Toulouse UPS INPT LAPLACE (Laboratoire Plasma et Conversion d'Energie) Toulouse Cedex 9 F-31062 France Group of Electrical Engineering-Paris (GeePs) UMR 8507 CNRS CentraleSupelec Universite Paris-Sud-UPMC 11 rue Joliot-Curie Plateau de Moulon 91192 Gif-sur-Yvette Cedex F-91192 France CALMIP Universite de Toulouse-CNRS-INPT-INSA-UPS UMS 3667 France cnrs LAPLACE Toulouse F-31062 France

The aim of this paper is to test a developed SOR R＆B method using the Chebyshev accelerator algorithm to solve the Laplace equation in a cubic 3D configuration. Comparisons are made in terms of precision and computing time with other elliptic equation solvers proposed in the open source LIS library. The first results, obtained by using a single core on a HPC, show that the developed SOR R＆B method is efficient when the spectral radius needed for the Chebyshev acceleration is carefully pre-estimated. Preliminary results obtained with a parallelized code using the MPI library are also discussed when the calculation is distributed over one hundred cores.

关键词： numerical methods for elliptic equations high performance computing 3Dstreamer simulation SOR IDR BiCGSTAB

The SlSHN2 transcription factor contributes to cuticle formation and epidermal patterning in tomato fruit

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Molecular Horticulture 2022年第1期2卷 186-205页

作者： Cecile Bres Johann Petit Nicolas Reynoud Lysiane Brocard Didier Marion Marc Lahaye Benedicte Bakan Christophe Rothan UMR 1332 BFP INRAEUniversite de Bordeaux33140 Villenave d’OrnonFrance Unite Biopolymeres InteractionsAssemblagesINRAEBP7162744316 Nantes Cedex 3France Univ.Bordeaux CNRSINSERMBordeaux Imaging CenterBICUMS 3420US 433000 BordeauxFrance INRA UMR1332 Biologie du Fruit Et Pathologie71 Av Edouard Bourlaux33140 Villenave d’OrnonFrance

Tomato(Solanum lycopersicum)is an established model for studying plant cuticle because of its thick cuticle covering and embedding the epidermal cells of the *** this study,we screened an EMS mutant collection of the miniature tomato cultivar Micro-Tom for fruit cracking mutants and found a mutant displaying a glossy fruit *** using an established mapping-by-sequencing strategy,we identified the causal mutation in the SlSHN2 transcription factor that is specifically expressed in outer epidermis of growing *** point mutation in the shn2 mutant intro-duces a K to N amino acid change in the highly conserved‘mm’domain of SHN *** cuticle from shn2 fruit showed a~fivefold reduction in cutin while abundance and composition of waxes were barely *** addition to alterations in cuticle thickness and properties,epidermal patterning and polysaccharide composition of the cuticle were *** analysis further highlighted the altered expression of hundreds of genes in the fruit exocarp of shn2,including genes associated with cuticle and cell wall formation,hormone signaling and response,and transcrip-tional *** conclusion,we showed that a point mutation in the transcriptional regulator SlSHN2 causes major changes in fruit cuticle formation and its coordination with epidermal patterning.

关键词： Mutant SHINE Cuticle Epidermis Cell wall Ethylene

Correction:COVID-19 and flu:conserved or specific immune signature?

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Cellular & Molecular Immunology 2022年第12期19卷 1439-1439页

作者： Christophe Paget François Trottein Centre d’Etude des Pathologies Respiratoires INSERM U1100 Université de Tours Faculté de Médecine de Tours 37000 Tours France Centre d’Infection et d’Immunité de Lille INSERM U1019 CNRS UMR 9017 Université de Lille CHU Lille UMS 2014 - PLBS U1019 Institut Pasteur de Lille 59000 Lille France

Correction to:Cellular&Molecular Immunology https://***/10.1038/s41423-020-00595-3,published online 11 January 2021 The licence information was missing from this article and should *** original article has been correc... 详细信息

关键词： signature conserved corrected

COVID-19 and flu:conserved or specific immune signature?

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Cellular & Molecular Immunology 2021年第2期18卷 245-246页

作者： Christophe Paget François Trottein Centre d’Etude des Pathologies Respiratoires INSERM U1100Universitéde ToursFacultéde Médecine de Tours37000ToursFrance Centre d’Infection et d’Immunitéde Lille INSERM U1019CNRS UMR 9017Universitéde LilleCHU LilleUMS 2014-PLBSU1019Institut Pasteur de Lille59000LilleFrance

Respiratory viruses are a major cause of infectious disease and mortality *** respiratory viruses,emerging and well-known coronaviruses and influenza viruses cause seasonal epidemics and pandemics and rank as very important *** clinical signs of viral respiratory infections range from asymptomatic forms to life-threatening acute respiratory distress syndrome(ARDS,associated with a high mortality rate).Another reported cause of mortality is multiple organ *** ongoing pandemic of coronavirus disease 19(COVID-19,caused by severe acute respiratory syndrome coronavirus 2(SARS-CoV-2))is a serious public health ***-CoV-2 infection induces to a clinical signature distinct from that associated with other types of pneumonia,and SARS-CoV-2 differs from influenza viruses with regard to target cells and replicative *** morbidity and mortality rates are higher for COVID-19 than for seasonal influenza.

关键词： mortality acute respiratory

Sliding walls: a new paradigm for fluidic actuation and protocol implementation in microfluidics

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Microsystems & Nanoengineering 2020年第1期6卷 1093-1102页

作者： Bastien Venzac Yang Liu Ivan Ferrante Pablo Vargas Ayako Yamada Remi Courson Marine Verhulsel Laurent Malaquin Jean-Louis Viovy Stephanie Descroix Laboratoire Physico Chimie Curie Institut CuriePSL Research UniversityCNRS UMR16875005 ParisFrance Sorbonne Universites UPMC Univ Paris 0675005 ParisFrance Institut Pierre-Gilles de Gennes PSL Research University75005 ParisFrance Institut Curie PSL Research UniversityCNRS UMR 14475005 ParisFrance LAAS-cnrs Universitéde ToulouseCNRS31400 ToulouseFrance

Currently,fluidic control in microdevices is mainly achieved either by external pumps and valves,which are expensive and bulky,or by valves integrated in the *** types of internal valves or actuation methods have been proposed,but they generally impose difficult compromises between performance and fabrication *** propose here a new paradigm for actuation in microfluidic devices based on rigid or semi-rigid walls with transversal dimensions of hundreds of micrometres that are able to slide within a microfluidic chip and to intersect microchannels with hand-driven or translation stage-based *** this new concept for reconfigurable microfluidics,the implementation of a wide range of functionalities was facilitated and allowed for no or limited dead volume,low cost and low *** demonstrate here several fluidic operations,including on/off or switch valving,where channels are blocked or reconfigured depending on the sliding wall *** valves sustain pressures up to *** and reversible compartmentalisation of large microfluidic chambers were also *** last possibility was applied to a“4D”migration assay of dendritic cells in a collagen ***,sliding walls containing a hydrogel-based membrane were developed and used to concentrate,purify and transport biomolecules from one channel to another,such functionality involving complex fluidic transport patterns not possible in earlier microfluidic ***,this toolbox is compatible with“soft lithography”technology,allowing easy implementation within usual fabrication workflows for polydimethylsiloxane *** new technology opens the route to a variety of microfluidic applications,with a focus on simple,hand-driven devices for point-of-care or biological laboratories with low or limited equipment and resources.

关键词： walls hundreds operations