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A mutation in the ZNf687 gene that is responsible for the severe form of Paget's disease of bone causes severely altered bone remodeling and promotes hepatocellular carcinoma onset in a knock-in mouse model

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Bone Research 2023年第1期11卷 201-214页

作者： Sharon Russo federica Scotto di carlo Antonio Maurizi Giorgio fortunato Anna Teti Danilo Licastro Carmine Settembre Tommaso Mello fernando Gianfrancesco Institute of Genetics and Biophysics“Adriano Buzzati-Traverso” National Research Council of ItalyNaplesItaly Department of Environmental Biological and Pharmaceutical Sciences and Technologies(DiSTABiF)University of Campania Luigi VanvitelliCasertaItaly Department of Biotechnological and Applied Clinical Sciences University of L’AquilaL’AquilaItaly AREA Science Park PadricianoTriesteItaly Telethon Institute of Genetics and Medicine(TIGEM) PozzuoliItaly Gastroenterology Unit Department of Experimental and Clinical Biochemical Sciences“Mario Serio”University of FlorenceFlorenceItaly IRCCS INM Neuromed PozzilliISItaly

Paget’s disease(PDB)is a late-onset bone remodeling disorder with a broad spectrum of symptoms and *** of the most aggressive forms is caused by the P937R mutation in the ZNf687 *** the genetic involvement of ZNf687 in PDB has been extensively studied,the molecular mechanisms underlying this association remain ***,we describe the first Zfp687 knock-in mouse model and demonstrate that the mutation recapitulates the PDB phenotype,resulting in severely altered bone *** microcomputed tomography analysis,we observed that 8-month-old mutant mice showed a mainly osteolytic phase,with a significant decrease in the trabecular bone volume affecting the femurs and the ***,osteoblast activity was deregulated,producing disorganized ***,this phenotype became pervasive in 16-month-old mice,where osteoblast function overtook bone resorption,as highlighted by the presence of woven bone in histological analyses,consistent with the PDB ***,we detected osteophytes and intervertebral disc degeneration,outlining for the first time the link between osteoarthritis and PDB in a PDB mouse *** sequencing of wild-type and Zfp687 knockout RAW264.7 cells identified a set of genes involved in osteoclastogenesis potentially regulated by Zfp687,e.g.,Tspan7,Cpe,Vegfc,and Ggt1,confirming its role in this ***,in this mouse model,the mutation was also associated with a high penetrance of hepatocellular ***,this study established an essential role of Zfp687 in the regulation of bone remodeling,offering the potential to therapeutically treat PDB,and underlines the oncogenic potential of ZNf687.

关键词： RAW264.7 Paget remodeling

Absolute parameters of the three totally-eclipsing W UMa stars NSVS 2443858,NSVS 780649 and V1098 Her

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Research in Astronomy and Astrophysics 2021年第9期21卷 124-134页

作者： Velimir Popov francesco Acerbi carlo Barani Department of Physics and Astronomy Shumen University115 Universitetska str.9700 ShumenBulgaria Via Zoncada 51 26845 Codogno(LO)Italy Via Molinetto 35 26845 Triulza di Codogno(LO)Italy

We present the first photometric analysis of three totally-eclipsing W UMa binaries,NS VS2443858,NSVS 780649 and V1098 *** absolute astrophysical parameters of the stellar components were determined by means of Gaia distances and light curve *** results show that:(ⅰ)Two of the systems,NSVS 2443858 and V1098 Her,are of A subtype while the obtained temperature of the secondary component of NSVS 780649 indicates that it is a W-subtype system;(ⅱ)The estimated mass ratios approach the lower limit of the mass ratio assumed by researchers in recent years so our targets could be classified as extreme mass ratio binary(EMRB)systems;(ⅲ)All the systems have deep contact configurations,so they also are deep low mass ratio(DLMR)systems;(ⅳ)The components of our systems are stars of f and G spectral type and undergo total eclipses;(ⅴ)The sum 0.871 M⊙of the component masses of NSVS 780649 is below the mass limit of 1.0-1.2 M⊙assumed for the known contact binary stars.

关键词： techinques:photometric binaries:eclipsing stars:fundamental parameters stars:individual

DZ Lyn:a contact binary with components in poor thermal contact

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Research in Astronomy and Astrophysics 2009年第11期9卷 1270-1276页

作者： Massimiliano Martignoni francesco Acerbi carlo Barani Via Don G. Minzoni 26/D 20020 Magnago(MI)Italy Via Zoncada 51 26845 Codogno(LO)Italy Via Molinetto 35 26845 Triulza di Codogno(LO)Italy

CCD photometric observations of the short-period eclipsing binary DZ Lyn were obtained during seven nights in March-April 2008 in the B, V, Rc and Ic bands; these observations confirm the short-period （P = 0.378 d） of the system. The presented light curves are analyzed by means of the 2003 version of the Wilson-Devinney （WD） program. A grid of solutions for several fixed values of mass ratio was calculated. The best fitting possible is for a mass ratio of q -- 0.885, a degree of contact f = 0.18 and a low orbital inclination of i = 57.6°. The results show that DZ Lyn is an A-subtype W Ursae Majoris contact binary in poor thermal contact; in fact the difference between the mean temperatures of the components is about 1800 K. The star DZ Lyn seems to be another member of the class of poorly understood close binaries in or near geometrical contact but far from thermal contact. The absolute dimensions are estimated and its dynamical evolution is inferred.

关键词： binaries eclipsing -- stars fundamental parameters -- stars individual DZ Lyn

Photometric study and preliminary elements of the low-mass ratio W UMa system ASAS 021209+2708.3

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Research in Astronomy and Astrophysics 2011年第7期11卷 843-850页

作者： francesco Acerbi carlo Barani Massimiliano Martignoni Via Zoncada 51 26845 Codogno(LO)Italy Via Molinetto 35 26845 Triulza di Codogno(LO)Italy Via Don G.Minzoni 26/D 20020 Magnago(MI)Italy

We present CCD B and V light curves,obtained in the year 2006,and a photometric solution of the low-mass ratio contact binary ASAS 021209＋2708.3. With our data we were able to determine six new times of minimum light and refine the orbital period of the system to 0.3181963 *** light curves are analyzed using the 2003 version of the Wilson-Devinney program and the analysis was performed with and without adding a spot on the surface of one star because the light curves appear to exhibit a typical O’Connell effect,with Maximum I brighter than Maximum *** results show that ASAS 021209＋2708.3 may be classified as an A-subtype W Ursae Majoris system with a small mass ratio q=0.1889,a large over-contact degree of f= 0.587,a very small difference between the component temperatures of Δ T=53 K and an orbital inclination of i=81°.It is known that deep（f50%） ,low-mass ratio（q0.25） overcontact binary stars are a very important resource for understanding the phenomena of Blue Straggler/fK Com-type *** formations of Blue Straggler stars and fK Com-type stars are unsolved problems in stellar *** of the possible explanations for their formation is from the coalescence of W UMa-type overcontact binary *** absolute dimensions of ASAS 021209＋2708.3 are estimated and its dynamical evolution is inferred.

关键词： binaries:eclipsing–stars:fundamental parameters–stars:individual:ASAS 021209+2708.3

Estimation of the Lyman-α signal of the EfILE diagnostic under static or radiofrequency electric field in vacuum

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Plasma Science and Technology 2018年第7期20卷 1-6页

作者： carlo poggi théo guillaume fabrice doveil laurence chérigier-kovacic Consorzio RfX Corso Stati Uniti1-35127 PadovaItaly Aix-Marseille Universite CNRSPIIM UM R 7345F-13397 Marseille Cedex 20France

The electric field induced Lyman-a emission diagnostic aims to provide a non intrusive and precise measurement of the electric field in plasma, using a beam of hydrogen atoms prepared in the metastable 2s state. The metastable particles are obtained by means of a proton beam extracted from a hydrogen plasma source, and neutralised by interaction with vaporised caesium. When a 2s atom enters a region where an electric field is present, it undergoes a transition to the 2p state （Stark mixing）. It then quickly decays to the ground level, emitting Lyman-a radiation, which is collected by a photomultiplier. The 2s → 2p transition rate is proportional to the square of the magnitude of the electric field, and depends on the field oscillation frequency （with peaks around l GHz）. By measuring the intensity of the Lyman-a radiation emitted by the beam it is possible to determine the magnitude of the field in a defined region. In this work, an analysis of the behaviour of the diagnostic under static or radiofrequency electric field is presented. Electric field simulations obtained with a finite element solver of Maxwell equations, combined with theoretical calculations of the Stark mixing transition rate, are used to develop a model for the interpretation of photomultiplier data. This method shows good agreement with experimental results for the static field case, and allows to measure the field magnitude for the oscillating case.

关键词： electric field measurement electromagnetic simulations Stark effect plasma diagnostics

Criteria influencing the selection and design of HV and UHV DC cables in new network applications

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High Voltage 2018年第2期3卷 90-95页

作者： Gian carlo Montanari Peter H.f.Morshuis Mingyu Zhou Gary CStevens Alun SVaughan Zhengyi Han Duo Li University of Bologna BolognaItaly Solid Dielectric Solutions LeidenThe Netherlands Global Energy Interconnection Research Institute Europe GmbH BerlinGermany Gnosys Global Ltd GuildfordUK ECS University of SouthamptonSouthamptonUK

This study sets out to discuss the challenges that need to be addressed effectively to facilitate the adoption of long distance high-voltage and ultra-high-voltage direct current(UHVDC)power transmission *** in the alternating current case,the desire to minimise the losses has involved the use of increasingly high transmission voltages,which presents particular problems where UHVDC cables are ***,this study presents the case for a novel,integrated approach to the problem,which links three distinct aspects of the problem,namely:(i)cable ratings and network operability;(ii)cable design and constituent insulating material requirements;and(iii)potential strategies for the design of novel insulation materials,where the required properties are actively‘designed in’.The study concludes that the impact of these factors acting in concert on network operation/performance requires a more integrated approach to the problem than has traditionally been the case.A number of complementary strategies for the development of novel material technologies and UHVDC cable designs are required that can meet the service performance and ultimate service lifetimes expected for new network applications.

关键词： network service cable

Pledget-assisted hemostasis to fix residual access-site bleedings after double pre-closure technique

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World Journal of Cardiology 2022年第5期14卷 297-306页

作者： francesco Burzotta Cristina Aurigemma Mila Kovacevic Enrico Romagnoli Stefano Cangemi francecso Bianchini Marialisa Nesta Piergiorgio Bruno carlo Trani Dipartimento di Scienze Cardiovascolari Fondazione Policlinico Universitario A Gemelli IRCCSRome 00168Italy UniversitàCattolica del Sacro Cuore Rome 00168Italy faculty of Medicine University of Novi SadNovi SadSerbia Institute of Cardiovascular Diseases of Vojvodina Cardiology ClinicSremska KamenicaSerbia

BACKGROUND The use of pre-closure suture-based devices represents a widely access-site hemostasis technique in percutaneous transfemoral transcatheter-aortic-valvereplacement(Tf-TAVR);yet this technique is associated with the risk of a device failure that may result in clinically relevant residual ***,a bailout intervention is *** far,the best management of pre-closure device failure has not been *** To report the first clinical results obtained using a novel bailout hemostasis technique for patients with double suture-based vascular closure device failure in the setting of *** We developed a“pledget-assisted hemostasis”technique to manage residual access-site *** consists of the insertion of a surgical,non-absorbable,polytetrafluoroethylene pledget over the sutures of the two ProGlide(Abbott Vascular,CA,United States).The ProGlide’s knot-pushers are used to push down the pledget and the hand-made slipknot to seal the femoral artery *** technique was used as a bailout strategy in patients undergoing Tf-TAVR with a systematic double pre-closure ***-procedural access-site angiography was systematically ***-hospital complications were systematically detected and classified according to Valve Academic Research Consortium-2 *** Out of 136 consecutive patients who underwent Tf-TAVR,15 patients(mean age 80.0±7.2 years,66.7%female)with access-site bleeding after double pre-closure technique failure were treated by pledget-assisted *** the majority of patients,16f sheath was used(n=12;80%).In 2 cases(13%),a peripheral balloon was also inflated in the iliac artery to limit blood loss during pledget ***-confirmed hemostasis(primary efficacy endpoint)was achieved in all *** the procedure,1 patient required blood transfusion(2 units),and no other bleeding or major ischemic complication was *** The“pledget assisted hemostasis”might be con

关键词： Transcatheter aortic valve replacement Transcatheter aortic valve implantation Vascular complications Preclosure device Pledget Hemostasis Personalized medicine

Raman spectra and vibrational analysis of CsPbI3:A fast and reliable technique to identify lead halide perovskite polymorphs

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Journal of Materiomics 2021年第1期7卷 127-135页

作者： Jessica Satta Claudio Melis carlo Maria Carbonaro Andrea Pinna Manuel Salado Daniel Salazar Pier carlo Ricci Department of Physics University of CagliariS.p.No.8 Km 0.70009042MonserratoCAItaly Department of Mechanical Chemical and Materials EngineeringCINSAINSTMUniversity of CagliariVia Marengo 2CagliariI-09123Italy BCMaterials Basque Center for MaterialsApplications and NanostructuresUPV/EHU Science Park48940LeioaSpain

A major issue in the development of Lead halide perovskites is the assessment of the crystal structure of the samples,due to their typically limited time-stability,and the understanding of the role of external factors that can induce a crystal phase transformation(such as humidity,intense light flux,temperature,etc.).In this perspective,it is of utmost importance to have at disposal a fast and reliable experimental tool able to give an immediate indication of the polymorph of the sample with the possibility to integrate in-situ measurements for constant *** this paper we propose Raman spectroscopy as the ideal technique to solve this *** vibrational analysis of CsPbI3 in the a-phase and 5-phase and of the Cs4PbI6 secondary phase is reported and all the vibrational modes are assigned by comparing experimental spectra of the phases to Raman modes calculated within the DfT ***,the mechanism of laser induced phase degradation was studied using in-situ Raman measurements providing new insights on the secondary phase generated during the process.

关键词： Raman spectroscopy CsPb13 Inorganic lead halide perovskites Phase transformation

Gene expression profiling improves prognostication by nomogram in patients with soft-tissue sarcomas

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Cancer Communications 2022年第6期42卷 563-566页

作者： Amandine Crombé Mariella Spalato-Ceruso Audrey Michot Yech’an Laizet carlo Lucchesi Maud Toulmonde Kevin Bourcier francois Le Loarer Antoine Italiano Department of Imaging Institut BergoniéBordeauxNouvelle-Aquitaine F-33076France Department of Medicine Institut BergoniéBordeauxNouvelle-Aquitaine F-33076France Department of Surgery Institut BergoniéBordeauxNouvelle-Aquitaine F-33076France Bioinformatics Unit BordeauxNouvelle-Aquitaine F-33076France Department of Pathology Institut BergoniéBordeauxNouvelle-Aquitaine F-33076France University of Bordeaux BordeauxNouvelle-Aquitaine F-33000France

Dear Editor,Surgery remains the cornerstone of treatment for softtissue sarcomas(STS)[1].However,despite adequate locoregional treatment,30%-40%of the patients eventually develop metastases[2,3].Accurate risk assessment is crucial to tailor therapeutic strategies and to identify high-risk patients who may benefit from perioperative ***,several efforts have been made to develop prognostic nomograms that enable individual prognosis prediction;representing an important decision-making aid for oncologists and surgeons involved in sarcoma care[4-6].

关键词： patients sarcoma Surgery