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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

HACD1,a regulator of membrane composition and fluidity,promotes myoblast fusion and skeletal muscle growth

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分子细胞生物学报:英文版 2015年第5期7卷 429-440页

作者： Jordan Blondelle Yusuke Ohno Vincent Gache Stephane Guyot Sebastien Storck Nicolas Blanchard-Gutton Ines Barthelemy Gemma Walmsley Anaelle Rahier Stephanie Gadin Marie Maurer laurent Guillaud Alexandre Prola Arnaud Ferry Genevieve Aubin-Houzelstein Jean Demarquoy Frederic Relaix Richard JPiercy Stephane Blot Akio kihara laurent Tiret Fanny Pilot-Storck Inserm IMRB U955-E1094000 CreteilFrance universite Paris-Est Ecole nationale veterinaire d’Alfort(EnvA)94700 Maisons-AlfortFrance universite Paris-Est Creteil Faculte de medecine94000 CreteilFrance Laboratory of Biochemistry Faculty of Pharmaceutical SciencesHokkaido UniversitySapporo 060-0812Japan universite de Bourgogne UMR A 02.102 PAM-EPMBAgroSup Dijon21000 DijonFrance Institut Necker-Enfants Malades INSERM U1151-CNRS UMR 8253Sorbonne Paris CiteUniversite Paris DescartesFaculte de Medecine-Site Broussais75015 ParisFrance Comparative Neuromuscular Disease Laboratory Department of Clinical Sciences and ServicesRoyal Veterinary CollegeLondon NW10TUUK Therapie des maladies du muscle strie INSERM u974-CNRS uMR7215-uPMC uM76-Institut de Myologie Universite Pierre et Marie Curie-Universite Paris Descartes75000 ParisFrance universite de Bourgogne Faculte des Sciences GabrielBio-PeroxIL21000 DijonFrance

The reduced diameter of skeletal myofibres is a hallmark of several congenital myopathies,yet the underlying cellular and molecular mechanisms remain *** this study,we investigate the role of HACD1/PTPLA,which is involved in the elongation of the very long chain fatty acids,in muscle fibre *** humans and dogs,HACD1 deficiency leads to a congenital myopathy with fibre size disproportion associated with a generalized *** of HACD1-deficient Labradors,Hacd1-knockout mice,and Hacd1-deficient myoblasts,we provide evidence that HACD1 promotes myoblast fusion during muscle development and *** further demonstrate that in normal differentiating myoblasts,expression of the catalytically active HACD1 isoform,which is encoded by a muscle-enriched splice variant,yields decreased lysophosphatidylcholine content,a potent inhibitor of myoblast fusion,and increased concentrations of≥C18 and monounsaturated fatty acids of *** lipid modifications correlate with a reduction in plasma membrane *** conclusion,we propose that fusion impairment constitutes a novel,non-exclusive pathological mechanism operating in congenital myopathies and reveal that HACD1 is a key regulator of a lipid-dependent muscle fibre growth mechanism.

关键词： centronuclear myopathy LPC MuFA PTPLA VLCFA

Teaching Polymer Chemistry: Revisiting the Syllabus

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Open Journal of Polymer Chemistry 2012年第4期2卷 132-143页

作者： Thierry Hamaide Yves Holl laurent Fontaine Jean-Luc Six Armand Soldera Centre Québécois des Matériaux Fonctionnels Département de Chimie U. Sherbrooke J1K2R1 Qc Canada Institut Charles Sadron CNRS 23 Rue du Loess Strasbourg Cedex France Lorraine université Laboratoire de Chimie-Physique Macromoléculaire Nancy France université de Lyon Université Claude Bernard Lyon Ingénierie des Matériaux Polymères Villeurbanne France université du Maine Unité de Chimie Organique Moléculaire et Macromoléculaire Le Mans cedex France

As anyone who has taught polymer chemistry to undergraduate students might already appreciate, we are faced with inherent difficulties in clearly communicating the subject matter due its interdisciplinary character. This paper is thus aimed at proposing a new educational plan to teach this fundamental course in order to overcome some inherent obstacles. It is especially devoted to professors in charge of teaching a first course in polymer chemistry. We intend to emphasize polymerization by beginning with the simplest chemical pathway, namely the living anionic polymerization. In addition, some pedagogical difficulties are outlined all along this paper. This novel and distinct way to teach polymer chemistry makes the students with a background in organic chemistry more equipped to face difficulties arising from the introduction of new concepts.

关键词： Teaching Polymer Chemistry Chain Polymerization Step-Growth Polymerization Polycondensation Polyaddition

updated Inventory of Carbon Monoxide in the Taurus Molecular Cloud

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天文和天体物理学研究:英文版 2023年第9期23卷 56-65页

作者： Yan Duan Di Li laurent Pagani Paul F.Goldsmith Tao-Chung Ching Chen Wang Jinjin Xie National Astronomical Observatories Chinese Academy of SciencesBeijing 100101China university of Chinese Academy of Sciences Beijing 100049China Research Center for Intelligent Computing Platforms Zhejiang LaboratoryHangzhou 311100China NAOC-ukZN Computational Astrophysics Centre University of KwaZulu-NatalDurban4000South Africa LERMA&uMR 8112 du CNRS Observatoire de ParisPSL UniversitySorbonne UniversitésCNRSF-75014 ParisFrance Jet Propulsion Laboratory California Institute of Technology4800 Oak Grove DrivePasadenaCA91109USA Jansky Fellow National Radio Astronomy Observatory1003 Lopezville RoadSocorroNM 87801USA Shanghai Astronomical Observatory Chinese Academy of Sciences National Astronomical ObservatoriesChinese Academy of SciencesShanghai 200030China

The most extensive survey of carbon monoxide(CO)gas in the Taurus molecular cloud relied on ^(12)CO and ^(13)CO J=1→0 emission only,distinguishing the region where ^(12)CO is detected without ^(13)CO(named mask 1 region)from the one where both are detected(mask 2 region)(Goldsmith et al.2008;Pineda et al.2010).We have taken advantage of recent ^(12)CO J=3→2 James Clerk Maxwell Telescope observations,where they include mask 1regions to estimate density,temperature,and N(CO)with a large velocity gradient *** represents 1395 pixels out of~1.2 million in the mark 1 *** to Pineda et al.(2010)results and assuming a Tkin of 30 k,we find a higher volume density of molecular hydrogen of 3.3×10^(3) cm^(-3),compared to their 250-700 cm^(-3),and a CO column density of 5.7×10^(15)cm^(-2),about a quarter of their *** differences are important and show the necessity to observe several CO transitions to better describe the intermediate region between the dense cloud and the diffuse atomic *** observations to extend the ^(12)CO J=3→2 mapping further away from the ^(13)COdetected region comprising mask 1 are needed to revisit our understanding of the diffuse portions of dark clouds.

关键词： submillimeter ISM-ISM clouds-ISM molecules

Engineering immunosuppressive drug-resistant armored(IDRA)SARS-CoV-2 T cells for cell therapy

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Cellular & Molecular Immunology 2023年第11期20卷 1300-1312页

作者： Qi Chen Adeline Chia Shou kit Hang Amy Lim Wee kun koh Yanchun Peng Fei Gao Jili Chen Zack Ho Lu-En Wai kamini kunasegaran Anthony Tanoto Tan Nina Le Bert Chiew Yee Loh Yun Shan Goh laurent Renia Tao Dong Anantharaman Vathsala Antonio Bertoletti Emerging Infectious Disease Program Duke-NUS Medical SchoolSingaporeSingapore National university Centre for Organ Transplantation National University HospitalSingaporeSingapore MRC Human Immunology unit MRC Weatherall Institute of Molecular MedicineRadcliffe Department of MedicineUniversity of OxfordOxfordUK Lion TCR Pte Ltd SingaporeSingapore A*STAR ID labs Agency for ScienceTechnology and ResearchSingaporeSingapore Lee kong Chian School of Medicine Nanyang Technological UniversitySingaporeSingapore Department of Medicine Yong Loo Lin School of MedicineNational University of SingaporeSingaporeSingapore Singapore Immunology Network A*STARSingaporeSingapore

Solid organ transplant(SOT)recipients receive immunosuppressive drugs(ISDs)and are susceptible to developing severe ***,we analyze the Spike-specific T-cell response after 3 doses of mRNA vaccine in a group of SOT patients(n=136)treated with different *** demonstrate that a combination of a calcineurin inhibitor(CNI),mycophenolate mofetil(MMF),and prednisone(Pred)treatment regimen strongly suppressed the mRNA vaccine-induced Spike-specific cellular *** defects have clinical consequences because the magnitude of vaccine-induced Spike-specific T cells was directly proportional to the ability of SOT patients to rapidly clear SARS-CoV-2 after breakthrough *** then compensate for the T-cell defects induced by immunosuppressive treatment and to develop an alternative therapeutic strategy for SOT patients,we describe production of 6 distinct SARS-CoV-2 epitope-specific ISD-resistant T-cell receptor(TCR)-T cells engineered using the mRNA electroporation method with reactivity minimally affected by mutations occurring in Beta,Delta,Gamma,and Omicron *** strategy with transient expression characteristics marks an improvement in the immunotherapeutic field and provides an attractive and novel therapeutic possibility for immunosuppressed COVID-19 patients.

关键词： T cell therapy Transplantation SARS-CoV-2 Immunosuppressive drug resistant T cells

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用次级束流对硅望远镜的时间和能量定标

核技术 1993年第12期16卷 705-709页

作者：贺智勇 Steckmeyer JC kerambrun A Angelique JC Bizard G Brou R Cussol D Patry JP Peter J Regimbart R Tamain B Vient E Cabot C Auger G Crema E Peghaire A Saint-laurent F Gonin M Hagel k Wada R Eudes P Lebrun C Rosato E Masri Y El 中国科学院近代物理研究所 LPC Caen ISMRAIN2P3-CNRSF-14050 CaenFrance GANIL BP5027F-14021 CaenFrance Cyclotron Institute Texas A and M UniversityCollege StationTX77843USA LPN Universite de Names44072 Nantes Cedex 03France Dipartimento di Scienze Fisiche Universita di NapoliI-80125 NaplesItaly uLB BruxellesBelgium

利用95MeV/u的^(16)O束产生的次级束流对半导体硅探测器进行了时间和能量定标。实验结果表明,由于次级束流提供的次级粒子种类多,飞行距离长,因此,能给探测器带来非常精确的定标。

关键词：时间定标能量定标探测器定标

中能区64Zn+27Al反应中集体流的实验研究

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中能区64Zn+27Al反应中集体流的实验研究