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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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Low phase noise K-band signal generation using polarization diverse single-soliton integrated microcombs

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Photonics Research 2024年第6期12卷 1175-1185页

作者： ALWALEED ALDHAFEERI HSIAO-HSUAN CHIN tristan MELTON DONG IL LEE ALLEN CHU WENTING WANG MINGBIN YU PATRICK GUO-QIANG LO DIM-LEE KWONG CHEE WEI WONG Fang Lu Mesoscopic Optics and Quantum Electronics Laboratory University of CaliforniaLos AngelesCalifornia 90095USA Institute of Microelectronics A*STARSingapore 117865Singapore State Key Laboratory of Functional Materials for Informatics Shanghai Institute of Microsystem and Information Technologyand Shanghai Industrial Technology Research InstituteShanghai 200050China

Frequency microcombs with microwave and millimeter-wave repetition rates provide a compact solution for coherent communication and information *** implementation of these microcombs using a CMOS-compatible platform further paves the way for large-scale photonic integration and ***,we demonstrate free-running soliton microcombs with K-band repetition rates with very low phase noise over a 4 GHz pump detuning range reaching−117(−123)dBc/Hz at 10 kHz offset for a 19.7(10)GHz carrier without active pump stabilization,exceeding commercial electronic microwave oscillators at frequency offsets above 40 *** minimum laser noise to soliton microwave signal transduction factor observed is−*** noise performance is achieved using a hybridized dual-mode for soliton generation to achieve passive thermal stabilization and minimal soliton spectrum shift from prior Raman scattering and dispersive wave *** further examine the locking of the repetition rate to an external ultrastable photonic oscillator to illustrate the feasibility of phase noise suppression below the thermorefractive noise limits of microresonator frequency combs.

关键词： pump polarization millimeter

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Rare dentin defects:Understanding the pathophysiological mechanisms of COLXVA1 mutations

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Genes & Diseases 2024年第5期11卷 1-5页

作者： Isaac Maximiliano Bugueno tristan Rey Alexandra Jimenez-Armijo Marzena Kawczynski Naji Kharouf Marie-Cecile Maniere Yann Herault Agnes Bloch-Zupan Virginie HaushalterLaugel Institute of Genetics and Molecular and Cellular Biology(IGBMC) CNRS-UMR7104INSERM U1258Universitede StrasbourgIllkirch 67400France Faculty of Dentistry Universitede StrasbourgStrasbourg 67000France Institute of Advanced Studies(USIAS) Universitede StrasbourgStrasbourg 67045France Reference Center for Rare Oral and Dental Diseases(CRMR-O-Rares) Oral Medicine and Surgery DepartmentHopitaux Universitaires de Strasbourg(HUS)Strasbourg 67000France Rare Diseases Health Network“TETE COU”&European Reference Network ERN CRANIO Strasbourg 67000France “Institut de Genetique Medicale d’Alsace” Laboratory of Genetic DiagnosticHopitaux Universitaires de Strasbourg(HUS)Strasbourg 67000France Biomaterials and Bioengineering Laboratory Inserm UMR_S 1121Strasbourg 67000France Orofacial Development and Regeneration Faculty of MedicineInstitute of Oral BiologyCentre of Dental MedicineUniversity of ZurichZurich 8032Switzerland

Dentin is a mineralized tissue with a chemical composition similar to bone but with a higher mineralized density and *** constitutes the central structure of the tooth between the internal pulp and external enamel toward the oral cavity or cementum toward the underlying *** dentin defects occur in a variety of rare genetic *** can manifest as“isolated”occurrences such as in dentinogenesis imperfecta(DI)or dentin dysplasia(DD)or can be associated with other symptoms in diseases such as osteogenesis imperfecta,Goldblatt syndrome,microcephalic osteodysplastic primordial dwarfism type Ⅱ,among others.

关键词： diseases den rigidity

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Photoelectrochemical Iron-Catalyzed C(sp^(3))-H Borylation of Alkanes in a Position-Selective Manner

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CCS Chemistry 2024年第6期6卷 1430-1438页

作者： Wen Wei Bin Wang Simon L.Homölle Jinbin Zhu Yanjun Li tristan von Münchow Isaac Maksso Lutz Ackermann Institut für Organische und Biomolekulare Chemie Georg-August-Universität Göttingen37077 Göttingen

Catalytic C-H borylation is of prime topical importance,since easily available feedstock chemicals can thereby be transformed into valuable transient functional groups in the absence of directing ***,we disclose an iron-catalyzed C(sp^(3))-H borylation of simple,non-prefunctionalized alkanes,providing access to value-added products in a single step by means of *** power of merging photo-and electrochemistry was mirrored by ample scope and exceedingly mild reaction ***,an outstanding position-selectivity in favor of primary C(sp^(3))-H proved viable within the photoelectrochemical borylation through a chemo-selective anodic overoxidation *** ferro-photoelectrochemistry strategy avoids toxic precious transition metals,enabling C(sp^(3))-H borylations in a site-selective fashion.

关键词： photoelectrocatalysis C(sp^(3))-H borylation iron catalysis position-selectivity chlorine radical

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Two adjacent NLR genes conferring quantitative resistance to clubroot disease in Arabidopsis are regulated by a stably inherited epiallelic variation

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Plant Communications 2024年第5期5卷 148-162页

作者： Antoine Gravot Benjamin Lie gard Leandro Quadrana Florian Veillet Yoann Aigu tristan Bargain Juliette Benejam Christine Lariagon Jocelyne Lemoine Vincent Colot Maria J.Manzanares-Dauleux Melanie Jubault IGEPP Institut Agro INRAEUniversitede Rennes35650 Le RheuFrance Institut de Biologie de l’Ecole Normale Superieure(IBENS) Ecole Normale SuperieureCentre National de la Recherche Scientifique(CNRS)Institut National de la Santeet de la Recherche Medicale(INSERM)75005 ParisFrance IGEPP INRAE Institut AgroUniversitede Rennes29260 PloudanielFrance Present address:Aix-Marseille University CEACNRSInstitute of Biosciences and BiotechnologiesBIAM Cadarache13108 Saint-Paul-lez-DuranceFrance.

Clubroot caused by the protist Plasmodiophora brassicae is a major disease affecting cultivated *** a combination of quantitative trait locus(QTL)fine mapping,CRISPR-Cas9 validation,and extensive analyses of DNA sequence and methylation patterns,we revealed that the two adjacent neigh-boring NLR(nucleotide-binding and leucine-rich repeat)genes AT5G47260 and AT5G47280 cooperate in controlling broad-spectrum quantitative partial resistance to the root pathogen *** in Arabidopsis and that they are epigenetically *** variation in DNA methylation is not associated with any nucleotide variation or any transposable element presence/absence variants and is stably ***-ations in DNA methylation at the Pb-At5.2 QTL are widespread across Arabidopsis accessions and corre-late negatively with variations in expression of the two *** study demonstrates that natural,stable,and transgenerationally inherited epigenetic variations can play an important role in shaping resistance to plant pathogens by modulating the expression of immune receptors.

关键词： methylation clubroot Plasmodiophora brassicae AT5G47260 AT5G47280 ADR1-L3 ddm1

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Animal personality and the ecological impacts of freshwater non-native species

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Current Zoology 2014年第3期60卷 417-427页

作者： tristan JUETTE CNRS Universite Paul Sabatier ENFA UMR 5174 EDB (Laboratoire Evolution & Diversite Biologique) Universite Toulouse 3 Paul Sabatier CNRS

While the ecological impacts of invasive species have been demonstrated for many taxonomic groups, the potential effects of behavioural variation among nonnative individuals （i.e. personality） on these impacts have been largely overlooked. This is despite the fact that recent studies have demonstrated that, by nature, the three first stages of biological invasions （i.e. transport, establishment and spread） can lead to personalitybiased populations. Freshwater ecosystems provide a unique oppor tunity to investigate this issue, notably because the ecological impacts of nonnative species have been extensively documented and because animal personality has been widely studied using freshwater model species. Here, we aim at developing some per spectives on the potential effects of animal personality on the ecological impacts of freshwater nonnative species across levels of biological organizations. At the individual level, personality types have been demonstrated to affect the physiolo gy, metabolism, life history traits and fitness of individuals. We used these effects to discuss how they could subsequently impact invaded popula tions and, in turn, recipient communities. We also discussed how these might translate into changes in the structure of food webs and the functioning of invaded ecosystems. Finally we discussed how these perspectives could interact with the management of invasive species .

关键词： Biological invasions Behavioural syndromes Aquatic ecosystems Temperaments Behavioural types Cascadingeffects

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Mock catalogues for large surveys:Lessons from Gaia and implications for CSST

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Science China(Physics,Mechanics & Astronomy) 2023年第11期66卷 239-240页

作者： tristan Cantat-Gaudin Max-Planck-Institut für Astronomie Heidelberg69117Germany

Experience in large stellar surveys,such as Gaia,has shown that synthetic catalogues are very useful for preparing the data and evaluating scientific results(see the Gaia Universe model snapshot,ref.[1],as an example).This experience holds especially for complex datasets that provide not only positions and apparent magnitudes,but also proper motions,multi-band photometry,and spectroscopy.

关键词： spectroscopy survey holds

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Spatial and Temporal Validation of In-Situ and Satellite Weather Data for the South West Agricultural Region of Australia

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Journal of Meteorological Research 2022年第1期36卷 175-192页

作者： tristan CAMPBELL Peter FEARNS Centre for Mine Site Restoration Curtin University of TechnologyWA 6102Australia Remote Sensing and Satellite Research Group Curtin University of TechnologyWA 6102Australia

Seasonal variations in weather have significant impacts on crop *** accuracy of weather data is an important consideration for crop yield *** study uses an independent in-situ weather station network to validate the accuracy of monthly temperature and precipitation data from the in-situ weather station network operated by the Bureau of Meteorology(BOM),interpolated gridded data from this network,and satellite weather data for the South West Agricultural Region of *** region covers five classes of the K?ppen-Geiger climate classification system and is responsible for 10 billion AUD of agricultural produce annually.A strong bias was found for the maximum temperatures in the Copernicus LST(land surface temperature)satellite *** bias was linearly correlated with the in-situ temperature and exceeded 20℃in warmer *** to the bias’s linear nature,a linear correction was able to reduce the root-mean-square error(RMSE)of the Copernicus LST product by 82%.This process was tested for other regions of Australia and,despite some regional differences,a linear correction consistently reduced RMSE by 80%.The validation process demonstrated that the dataset with reliably the lowest RMSE is the gridded weather data calculated from BOM’s in-situ weather *** neighbor in-situ weather stations generally had the next lowest RMSE,followed by weather-station corrected satellite products and lastly the non-weather station corrected satellite *** the in-situ gridded product generally had the lowest RMSE,there were spatial and seasonal *** maximum temperatures were more accurately measured by the bias-corrected Copernicus LST product in the northern and eastern extents(where there is a lower density of BOM in-situ stations).Monthly minimum temperatures from the Copernicus LST product had similar to slightly better RMSE than the Australian Water Availability Project(AWAP)product for the southern half of the study ar

关键词： Australian Water Availability Project land surface temperature satellite precipitation validation

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Practical assessment of rock damage due to blasting

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International Journal of Mining Science and Technology 2019年第3期29卷 379-385页

作者： Jhon Silva tristan Worsey Braden Lusk Department of Mining Engineering University of KentuckyLexingtonKY 40508USA Department of Mining and Nuclear Engineering Missouri University of Science and TechnologyRollaMO 65409USA

Blasting is the most cost effective methodology to break rock for mining or civil engineering applications.A good production blast will break only the rock that is needed to be removed,leaving the host rock with minimal *** control of rock damage due to blasting is very important when it comes to mine or construction design,safety,and *** to the host rock due to a production blast could result in failures,overbreak and unstable *** how far the fractures generated by a production blast will go into the host rock is a valuable tool for engineers to design a safe highwall while keeping the actual excavation close to the ***,there are several methods available to predict damage due to *** accuracy of many of these methods is questionable,and in most cases,the methodologies over predict the *** often leads to inefficient mines and poor construction *** the current methodologies are reviewed,each one presents sound approaches,but in many cases they also lack consideration of other variables that,according to the authors,need to be included when predicting blast *** paper presents a practical methodology to assess the rock damage from blasting by combining other *** proposed method allows consideration of more variables when compared to available methods,resulting in a more accurate rock damage *** method uses the estimation of the generated levels of peak particle velocity with the distance from a production blast presented by Persson and Holmberg,the peak particle velocity damage ranges proposed by Forsyth and the relationship between the static compressive strength and dynamic compressive strength of rocks from *** new methodology was validated using the data published in a large-scale study performed in granite by Siskind.

关键词： Blasting Fracture Fracture extension Peak particle velocity

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Discontinuous Galerkin Methods for a Class of Nonvariational Problems

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Communications on Applied Mathematics and Computation 2022年第2期4卷 634-656页

作者： Andreas Dedner tristan Pryer Mathematics Institute University of WarwickCoventry CV47ALUK Department of Mathematical Sciences University of BathBath BA27AYUK

We extend the fnite element method introduced by Lakkis and Pryer(SIAM ***.33(2):786–801,2011)to approximate the solution of second-order elliptic problems in nonvariational form to incorporate the discontinuous Galerkin(DG)*** is done by viewing the“fnite element Hessian”as an auxiliary variable in the *** the fnite element Hessian in a discontinuous setting yields a linear system of the same size and having the same sparsity pattern of the compact DG methods for variational elliptic ***,the system matrix is very easy to assemble;thus,this approach greatly reduces the computational complexity of the discretisation compared to the continuous *** conduct a stability and consistency analysis making use of the unifed frameworkset out in Arnold et al.(SIAM ***.39(5):1749–1779,2001/2002).We also give an a posteriori analysis of the method in the case where the problem has a strong *** analysis applies to any consistent representation of the fnite element Hessian,and thus is applicable to the previous works making use of continuous Galerkin *** evidence is presented showing that the method works well also in a more general setting.

关键词： Nonvariational problems Discontinuous Galerkin Error estimates Adaptivity

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