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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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Indirect Water Consumption:The Scope 3 of Water?Estimation of This Indicator in Sectors Relying on Agriculture

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Journal of Environmental Science and Engineering(A) 2024年第3期13卷 77-85页

作者： Grégory schneider-Maunoury Nour Emam Maria Fernanda Estrada Cabeza de Vaca Anass Fathi Ricardo Henao Martinez Kahan Korkmaz Omar Mghaieth Christianah Ramanalinjatovo Syrine Saidi Department of Economics and Management UniversitéSorbonne Paris Nord99 Av.Jean Baptiste ClémentVilletaneuse 93430France

The aim of this research project is to assess indirect water consumption,that represents the water consumed in the upstream part of the production life *** are provided for agriculture related companies,as agriculture represents 70%of water consumption on *** consumption varies greatly according to the countries where supply chain is *** estimation is attempted for 27 European listed companies in sectors relying on agriculture in its supply chain.A data is estimated for 22 companies,showing that indirect water consumption is much more important than direct *** questions raised through the estimation of indirect water consumption in various subsectors show the interest in this data,which represents the equivalent of Carbon Scope 3 for water issues.

关键词： Water consumption water footprint financial materiality impact materiality sustainability reporting

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Copy-Move Geometric Tampering Estimation Through Enhanced SIFT Detector Method

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Computer Systems Science & Engineering 2023年第1期44卷 157-171页

作者： J.S.Sujin S.sophia Department of Electronics and Communication Engineering Sri Krishna College of TechnologyCoimbatore641042India Department of Electronics and Communication Engineering Sri Krishna College of Engineering and TechnologyCoimbatore641008India

Digital picture forgery detection has recently become a popular and sig-nificant topic in image *** to advancements in image processing and the availability of sophisticated software,picture fabrication may hide evidence and hinder the detection of such criminal *** practice of modifying origi-nal photographic images to generate a forged image is known as digital image forging.A section of an image is copied and pasted into another part of the same image to hide an item or duplicate particular image elements in copy-move *** order to make the forgeries real and inconspicuous,geometric or post-processing techniques are frequently performed on tampered regions during the tampering *** Copy-Move forgery detection,the high similarity between the tampered regions and the source regions has become crucial *** most frequent way for detecting copy-move forgeries is to partition the images into overlapping square blocks and utilize Discrete cosine transform(DCT)com-ponents as block *** to the high dimensionality of the feature space,Gaussian Radial basis function(RBF)kernel based Principal component analysis(PCA)is used to minimize the dimensionality of the feature vector repre-sentation,which improves feature matching *** this paper,we propose to use a novel enhanced Scale-invariant feature transform(SIFT)detector method called as RootSIFT,combined with the similarity measures to mark the tampered areas in the *** proposed method outperforms existing state-of-the-art methods in terms of matching time complexity,detection reliability,and forgery location accuracy,according to the experimental *** F1 score of the proposed method is 92.3%while the literature methods are around 90%on an average.

关键词： Multi sensor data fusion discriminator orientation pose position mean average precision recall

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Segmentation Based Real Time Anomaly Detection and Tracking Model for Pedestrian Walkways

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Intelligent Automation & Soft Computing 2023年第6期36卷 2491-2504页

作者： B.sophia D.Chitra Department of Computer Science and Engineering Sri Krishna College of Engineering and TechnologyCoimbatoreTamilnaduIndia Department of Computer Science and Engineering P.A College of Engineering and TechnologyPollachiTamilnaduIndia

Presently,video surveillance is commonly employed to ensure security in public places such as traffic signals,malls,railway stations,etc.A major chal-lenge in video surveillance is the identification of anomalies that exist in it such as crimes,thefts,and so ***,the anomaly detection in pedestrian walkways has gained significant attention among the computer vision communities to enhance pedestrian *** recent advances of Deep Learning(DL)models have received considerable attention in different processes such as object detec-tion,image classification,*** this aspect,this article designs a new Panoptic Feature Pyramid Network based Anomaly Detection and Tracking(PFPN-ADT)model for pedestrian *** proposed model majorly aims to the recognition and classification of different anomalies present in the pedestrian walkway like vehicles,skaters,*** proposed model involves panoptic seg-mentation model,called Panoptic Feature Pyramid Network(PFPN)is employed for the object recognition *** object classification,Compact Bat Algo-rithm(CBA)with Stacked Auto Encoder(SAE)is applied for the classification of recognized *** ensuring the enhanced results better anomaly detection performance of the PFPN-ADT technique,a comparison study is made using Uni-versity of California San Diego(UCSD)Anomaly data and other benchmark data-sets(such as Cityscapes,ADE20K,COCO),and the outcomes are compared with the Mask Recurrent Convolutional Neural Network(RCNN)and Faster Convolu-tional Neural Network(CNN)*** simulation outcome demonstrated the enhanced performance of the PFPN-ADT technique over the other methods.

关键词： Panoptic segmentation object detection deep learning tracking model anomaly detection pedestrian walkway

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ESCRT machinery:role of membrane repair mechanisms in escaping cell death

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Signal Transduction and Targeted Therapy 2022年第8期7卷 2655-2657页

作者： Lisa Gregor sophia Stock Sebastian Kobold Division of Clinical Pharmacology Department of Medicine IVUniversity HospitalLudwig Maximilian University(LMU)of MunichLindwurmstrasse 2a80337 MunichGermany Department of MedicineⅢ University HospitalLudwig Maximilian University(LMU)of MunichMarchioninistrasse 1581377 MunichGermany German Cancer Consortium(DKTK) Partner Site MunichPettenkoferstrasse 8a80336 MunichGermany Einheit für Klinische Pharmakologie(EKLiP) Helmholtz Zentrum MünchenGerman Research Center for Environmental Health(HMGU)Ingolstädter Landstrasse 185764 NeuherbergGermany

A recent research article published in Science by Ritter et *** that endosomal sorting complexes required for transport(ESCRT)proteins mediate repair of lesions in the cell membrane caused by the pore-forming toxin perforin at sites of cytotoxic T cell engagement.1 Subsequent entry of granzymes and initiation of apoptosis of cancer cells is thus limited,leading to reduced sensitivity against T cell‑mediated cytotoxicity.1 Along these lines,this could constitute a new critical resistance mechanism for T cellbased treatment approaches.

关键词： al. cytotoxicity death

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Insights into manganese transport: A matter of phosphorylation

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Molecular Plant 2022年第3期15卷 385-387页

作者： Anja schneider Plant Science Faculty of BiologyLudwig-Maximilians-Universität MünchenMunichGermany

Manganese(Mn)is an essential element required for the growth of all known living *** plants acquire Mn from the rhizosphere,and its translocation into,and distribution within,the plant involves a multitude of transporters(Alejandro et al.,2020).In the aerial part of the green plant,the most prominent reaction that depends on Mn is the water-splitting reaction of photosystem II,which employs a tetra-Mn cluster located in the thylakoid lumen of ***,no less important reactions take place in mitochondria,where a Mn superoxide dismutase acts as a scavenger of reactive oxygen species,and in the Golgi apparatus,where Mn-dependent glycosyltransferases are involved in cell wall biosynthesis.

关键词： manganese apparatus splitting

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The role of host refuge and strong Allee effects in a host-parasitoid system

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生物数学学报:英文版 2023年第5期16卷 111-135页

作者： Xiaochuan Hu sophia R.-J.Jang Vinli Inc. DallasTX75206USA Department of Mathematics and Statistics Teras Tech University LubbockTX 79409USA

We propose and study a discrete host-parasitoid model of difference equations with a spatial host refuge where hosts in the refuge patch are free from parasitism but undergo a demographic strong Allee *** the growth rate of hosts in the non-refuge patch is less than one,a host Allee threshold is derived below which both populations become *** is proven that both populations can persist indefinitely if the host growth rate in the non-refuge patch exceeds one and the maximum reproductive number of parasitoids is greater than *** simulations reveal that the host refuge can either stabilize or destabilize the host-parasitoid interactions,depending on other model parameters.A large number of parasitoid turnover from a parasitized host may be detrimental to the parasitoids due to Allee effects in the hosts within the refuge ***,it is demonstrated numerically that if the host growth rate is not small,the population level of parasitoids may suddenly drop to a small value as some parameters are varied.

关键词： Host refuge strong Allee effect stabilizing and destabilizing Beverton-Holt growth rate Allee threshold maximum reproductive number

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Enterocolic phlebitis:a rare cause of bowel ischemia and review of the literature

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Gastroenterology Report 2023年第1期11卷 24-31页

作者： Stefanie Bowee sophia B.Matter Heather Dawson Roman A.Inglin Department of General Surgery Spital InterlakenUnterseenSwitzerland Institute of Pathology University of BernBernSwitzerland

Enterocolic phlebitis(EP)is a rare cause of bowel ischemia due to isolated venulitis of the bowel wall and mesentery without arterial *** is often misdiagnosed as inﬂammatory bowel disease,carcinoma,or diverticulitis due to non-speciﬁc symptoms as well as non-speciﬁc clinical and radiologicalﬁ*** unresponsive to pharmacotherapy,surgical resection of the affected bowel appears to be the only successful therapy with a very low recurrence *** of EP remains *** report a case of EP with rare presentation in the left hemicolon and unusual histologicalﬁndings emphasizing the heterogeneity of this cause of enterocolic *** review and comparison of the three enti-ties—EP,mesenteric inﬂammatory veno-occlusive disease(MIVOD),and idiopathic myointimal hyperplasia of mesenteric veins(IMHMV),all describing patterns of bowel ischemia due to isolated pathology of mesenteric veins—reveal that the current terminology is *** and MIVOD are very similar and may be considered the same ***,though,differs in localization,symptom duration,and histologicalﬁndings but also shares features with EP and *** studies and harmonized terminology are inevitable for better understanding of the disease,prevention of unnecessary pharmacotherapy,and reduction in time to diagnosis.

关键词： phlebitis acute abdomen mesenteric vascular occlusion mesenteric ischemia colectomy case report

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Tribological mechanisms of slurry abrasive wear

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Friction 2023年第6期11卷 1079-1093页

作者： Yulong LI Paul SCHREIBER Johannes schneider Christian GREINER Institute for Applied Material(IAM) Karlsruhe Institute of Technology(KIT)Karlsruhe76131Germany KIT IAM-ZM MicroTribology Center(μTC) Karlsruhe76131Germany

Abrasive wear mechanisms—including two-body and three-body abrasion—dominate the performance and lifespan of tribological systems in many engineering fields,even of those operating in lubricated *** steel(100Cr6)pins and discs in a flat-on-flat contact were utilized in experiments together with 5 and 13 μm Al_(2)O_(3)-based slurries as interfacial media to shed light on the acting *** results indicate that a speed-induced hydrodynamic effect occurred and significantly altered the systems'frictional behavior in tests that were performed using the 5 μm *** experiments revealed that a speed-dependent hydrodynamic effect can lead to a 14% increase in film thickness and a decrease in friction of around 2/3,accompanied by a transition from two-body abrasion to three-body abrasion and a change in wear mechanism from microcutting and microploughing to fatigue ***,no correlation could be found between the total amount of wear and the operating state of the system during the experiment;however,the wear distribution over pin and disc was observed to change *** paper studies the influence of the hydrodynamic effect on the tribological mechanism of lubricated abrasive wear and also highlights the importance to not only consider a tribological systems'global amount of wear.

关键词： abrasive wear two-body abrasion three-body abrasion hydrodynamic effect steel

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Therapeutic regulation of autophagy in hepatic metabolism

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Acta Pharmaceutica Sinica B 2022年第1期12卷 33-49页

作者： Katherine Byrnes sophia Blessinger Niani Tiaye Bailey Russell Scaife Gang Liu Bilon Khambu Department of Pathology and Laboratory Medicine Tulane University School of MedicineNew OrleansLA 70112USA

Metabolic homeostasis requires dynamic catabolic and anabolic processes. Autophagy, an intracellular lysosomal degradative pathway, can rewire cellular metabolism linking catabolic to anabolic processes and thus sustain homeostasis. This is especially relevant in the liver, a key metabolic organ thatgoverns body energy metabolism. Autophagy’s role in hepatic energy regulation has just begun to emerge and autophagy seems to have a much broader impact than what has been appreciated in the field. Though classically known for selective or bulk degradation of cellular components or energy-dense macromolecules, emerging evidence indicates autophagy selectively regulates various signaling proteins to directly impact the expression levels of metabolic enzymes or their upstream regulators. Hence, we review three specific mechanisms by which autophagy can regulate metabolism: A) nutrient regeneration, B) quality control of organelles, and C) signaling protein regulation. The plasticity of the autophagic function is unraveling a new therapeutic approach. Thus, we will also discuss the potential translation of promising preclinical data on autophagy modulation into therapeutic strategies that can be used in the clinic to treat common metabolic disorders.

关键词： Autophagy Liver metabolism Signaling proteins Lysosome Nutrient regeneration Quality control Farnesoid X receptor Cryptochrome 1

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