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Gene replacement therapy in Bietti crystalline corneoretinal dystrophy:an open-label,single-arm,exploratory trial

Signal Transduction and Targeted Therapy 2024年第5期9卷 2275-2284页

作者： jinyuan wang jinlu Zhang Shicheng Yu Hongyan Li Shaohong Chen jingting Luo Haibo wang Yuxia Guan Haihan Zhang Shiyi Yin Huili wang Heping Li junle Liu jingyuan Zhu Qiong Yang Ying Sha Chuan Zhang Yuhang Yang Xuan Yang Xifang Zhang Xiuli Zhao Likun wang Liping Yang Wenbin Wei Beijing Tongren Eye Center Beijing Key Laboratory of Intraocular Tumor Diagnosis and TreatmentBeijing Ophthalmology&Visual Sciences Key LabMedical Artificial Intelligence Research and Verification Key Laboratory of the Ministry of Industry and Information TechnologyBeijing Tongren HospitalCapital Medical University100730 BeijingChina School of Clinical Medicine Tsinghua University100084 BeijingChina Chigenovo Co. Ltd.102206 BeijingChina department of Ophthalmology Peking University Third HospitalBeijing Key Laboratory of Restoration of Damaged Ocular NervePeking University Third Hospital100191 BeijingChina department of Ophthalmology The Third People’s Hospital of Dalian116091 DalianChina department of Biomedical Informatics School of Basic Medical SciencesPeking University Health Science Center100191 BeijingChina Institute of Systems Biomedicine Department of PathologySchool of Basic Medical SciencesPeking University Health Science Center100191 BeijingChina

Bietti crystalline corneoretinal dystrophy is an inherited retinal disease caused by mutations in CYP4V2,which results in blindness in the working-age population,and there is currently no available ***,we report the results of the first-in-human clinical trial(NCT04722107)of gene therapy for Bietti crystalline corneoretinal dystrophy,including 12 participants who were followed up for 180-365 *** open-label,single-arm exploratory trial aimed to assess the safety and efficacy of a recombinant adeno-associated-virus-serotype-2/8 vector encoding the human CYP4V2 protein(rAAV2/8-hCYP4V2).Participants received a single unilateral subretinal injection of 7.5×10^(10)vector genomes of rAAV2/***,73 treatment-emergent adverse events were reported,with the majority(98.6%)being of mild or moderate intensity and considered to be procedure-or corticosteroid-related;no treatment-related serious adverse events or local/systemic immune toxicities were *** with that measured at baseline,77.8%of the treated eyes showed improvement in best-corrected visual acuity(BCVA)on day 180,with a mean±standard deviation increase of 9.0±10.8 letters in the 9 eyes analyzed(p=0.021).By day 365,80%of the treated eyes showed an increase in BCVA,with a mean increase of 11.0±10.6 letters in the 5 eyes assessed(p=0.125).Importantly,the patients’improvement observed using multifocal electroretinogram,microperimetry,and Visual Function Questionnaire-25 further supported the beneficial effects of the *** conclude that the favorable safety profile and visual improvements identified in this trial encourage the continued development of rAAV2/8-hCYP4V2(named ZVS101e).

关键词： cor dystrophy crystalline

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Retaining the self-released chalcogenate at reconstructed cobalt sites by self-transformed carbonate regulation for boosted oxygen evolution

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journal of Energy Chemistry 2024年第10期97卷 46-54,I0002页

作者： jingxuan Zhao Zhe Xue Qing wang Xiangyang Li Shoujie Liu Xu Zhao School of Chemical Engineering and Technology Xi'an Jiaotong UniversityXi'an 710049ShaanxiChina Key Laboratoryof Materials Physics Instituteof Solid State PhysicsHefei Institutes of Physical Science(HFIPS)Chinese Academy of SciencesHefei 23031 Anhui China School of Materials Science and Engineering Anhui UniversityHefei 230601AnhuiChina

The in-situ generated oxyanions at electrochemically reconstructed catalysts from metal-based nonoxide compounds have been proven to significantly accelerate oxygen evolution reaction(OER)***,it remains a challenge to retain these self-released oxyanions at reconstructed catalysts,hindering its utilization as a tool to develop efficient OER ***,we demonstrate a versatile selftransformed carbonate regulation strategy to efficiently retain the self-released chalcogenate at Co oxyhydroxides reconstructed from carbon-incorporated Co selenides under OER *** selftransformed CO_(3)^(2-)can induce electron accumulation and narrow d bond at Co sites to facilitate the Co3d-O 2p orbital hybridization between Co sites and SeO_(x)^(2-)for enhanced SeO_(x)^(2-)retention,which can accelerate the rate-limiting step for^(*)OOH formation during *** to CoOOH-SeO_(x)^(2-)with limited SeO_(x)^(2-)residues,CoOOH-CO_(3)^(2-)/SeO_(x)^(2-)with elevated SeO_(x)^(2-)retention by CO_(3)^(2-)regulation exhibited a 5.6-fold increase in current density and a remarkable lower Tafel slope towards *** strategy paves a rational avenue to design efficient catalysts for electrooxidation reactions through finely regulating self-released oxyanions at reconstructed structures.

关键词： Electrochemical reconstruction Chalcogenate retention Carbonate regulation Orbital hybridization Oxygen evolution reaction

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MIdNet:deblurring Network for Material Microstructure Images

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Computers, Materials & Continua 2024年第4期79卷 1187-1204页

作者： jiaxiang wang Zhengyi Li Peng Shi Hongying Yu dongbai Sun National Center for Materials Service Safety University of Science and Technology BeijingBeijing100083China School of Materials Sun Yat-Sen UniversityShenzhen518107China School of Materials Science and Engineering Southern Marine Science and Engineering Guangdong Laboratory(Zhuhai)Sun Yat-Sen UniversityGuangzhou510006China

Scanning electron microscopy(SEM)is a crucial tool in the field of materials science,providing valuable insightsinto the microstructural characteristics of ***,SEM images often suffer from blurrinesscaused by improper hardware calibration or imaging automation errors,which present challenges in analyzingand interpretingmaterial ***,rectifying the blurring of these images assumes paramountsignificance to enable subsequent *** address this issue,we introduce a Material Images deblurringNetwork(MIdNet)built upon the foundation of the Nonlinear Activation Free Network(NAFNet).MIdNetis meticulously tailored to address the blurring in images capturing the microstructure of *** keycontributions include enhancing the NAFNet architecture for better feature extraction and representation,integratinga novel soft attention mechanism to uncover important correlations between encoder and decoder,andintroducing newmulti-loss functions to improve training effectiveness and overallmodel *** conducta comprehensive set of experiments utilizing the material blurry dataset and compare them to several state-of-theartdeblurring *** experimental results demonstrate the applicability and effectiveness of MIdNet in thedomain of deblurring material microstructure images,with a PSNR(Peak Signal-to-Noise Ratio)reaching 35.26 dBand an SSIM(Structural Similarity)of *** dataset is available at:https://***/woshigui/MIdNet.

关键词： Image deblurring material microstructure attention mechanism deep learning

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Obstructive sleep apnea:Overlooked comorbidity in patients with diabetes

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World journal of diabetes 2024年第7期15卷 1448-1460页

作者： Eric d Tenda joshua Henrina jin H Cha Muhammad R Triono Ersananda A Putri dahliana j Aristy dicky L Tahapary division of Respirology and Critical Care Department of Internal MedicineFaculty of Medicine Universitas Indonesia-Cipto Mangunkusumo HospitalDKI JakartaJakarta Pusat 10430Indonesia Head of Research Group Artificial Intelligence and digital Health Indonesian Medical Education and Research InstituteFaculty of Medicine University of IndonesiaDKI JakartaJakarta Pusat 10430Indonesia division of Endocrinology Metabolism and DiabetesDepartment of Internal MedicineFaculty of Medicine Universitas Indonesia-Cipto Mangunkusumo HospitalDKI JakartaJakarta Pusat 10430Indonesia

In this review article,we explore the interplay between obstructive sleep apnea(OSA)and type 2 diabetes mellitus(T2dM),highlighting a significant yet often overlooked *** delve into the pathophysiological links between OSA and diabetes,specifically how OSA exacerbates insulin resistance and disrupts glucose *** research examines the prevalence of OSA in diabetic patients and its role in worsening diabetes-related *** the importance of comprehensive management,including weight control and positive airway pressure therapy,the study advocates integrated approaches to improve outcomes for patients with T2dM and *** review underscores the necessity of recognizing and addressing OSA in diabetes care to ensure more effective treatment and better patient outcomes.

关键词： Obstructive sleep apnea Type 2 diabetes mellitus Macrovascular Microvascular Weight loss Positive airway pressure

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Graphene-calcium carbonate coating to improve the degradation resistance and mechanical integrity of a biodegradable implant

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journal of Magnesium and Alloys 2024年第1期12卷 394-404页

作者： Lokesh Choudhary Parama Chakraborty Banerjee R.K.Singh Raman derrek E.Lobo Christopher d.Easton Mainak Majumder Frank Witte jörg F.Löffler department of Mechanical and Aerospace Engineering Monash UniversityClayton CampusMelbourneVIC 3800Australia department of Chemical and Biological Engineering Monash UniversityClayton CampusMelbourneVIC 3800Australia Nanoscale Science and Engineering Laboratory(NSEL) Bldg 37Monash UniversityClayton CampusMelbourneVIC 3800Australia CSIRO Materials Science and Engineering Bayview AvenueClaytonVIC 3168Australia julius Wolff Institute and Berlin Brandenburg Center for Regenerative Therapies Charité–Universitätsmedizin BerlinAugustenburger Platz 113353 BerlinGermany Laboratory of Metal Physics and Technology Department of MaterialsETH Zurich8093 ZurichSwitzerland

Biodegradable implants are critical for regenerative orthopaedic procedures,but they may suffer from too fast corrosion in human-body *** necessitates the synthesis of a suitable coating that may improve the corrosion resistance of these implants without compromising their mechanical *** this study,an AZ91 magnesium alloy,as a representative for a biodegradable Mg implant material,was modified with a thin reduced graphene oxide(RGO)-calcium carbonate(CaCO_(3))composite *** analytical and in-vitro electrochemical characterization reveals that this coating significantly improves the corrosion resistance and mechanical integrity,and thus has the potential to greatly extend the related application field.

关键词： Graphene coating Biodegradable implant Hydroxyapatite Corrosion Magnesium alloy

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PSRdP:A Parallel Processing Method for Pulsar Baseband data

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Research in Astronomy and Astrophysics 2024年第1期24卷 300-310页

作者： Ya-Zhou Zhang Hai-Long Zhang jie wang Xin-Chen Ye Shuang-Qiang wang Xu du Han Wu Ting Zhang Shao-Cong Guo Meng Zhang Xinjiang Astronomical Observatory Chinese Academy of SciencesUrumqi 830011China University of Chinese Academy of Sciences Beijing 100049China Key Laboratory of Radio Astronomy Chinese Academy of SciencesNanjing 210008China National Astronomical data Center Beijing 100101China Southeast University Nanjing 211189China School of Cyber Science and Engineering Qufu Normal UniversityQufu 273165China

To address the problem of real-time processing of ultra-wide bandwidth pulsar baseband data,we designed and implemented a pulsar baseband data processing algorithm(PSRdP)based on GPU parallel computing *** can perform operations such as baseband data unpacking,channel separation,coherent dedispersion,Stokes detection,phase and folding period prediction,and folding integration in GPU *** tested the algorithm using the j0437-4715 pulsar baseband data generated by the CASPSR and Medusa backends of the Parkes,and the j0332+5434 pulsar baseband data generated by the self-developed backend of the Nan Shan Radio *** obtained the pulse profiles of each baseband *** experimental analysis,we have found that the pulse profiles generated by the PSRdP algorithm in this paper are essentially consistent with the processing results of digital Signal Processing Software for Pulsar Astronomy(dSPSR),which verified the effectiveness of the PSRdP ***,using the same baseband data,we compared the processing speed of PSRdP with dSPSR,and the results showed that PSRdP was not slower than dSPSR in terms of *** theoretical and technical experience gained from the PSRdP algorithm research in this article lays a technical foundation for the real-time processing of QTT(Qi Tai radio Telescope)ultra-wide bandwidth pulsar baseband data.

关键词： (stars:)pulsars:general methods:data analysis techniques:miscellaneous

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Effect of Al_(2)O_(3)on the Mechanical Properties of(B_(4)C+Al_(2)O_(3))/Al Neutron Absorbing Materials

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Acta Metallurgica Sinica(English Letters) 2024年第8期37卷 1411-1420页

作者： j.X.Cai B.M.Shi N.Li Y.Liu Z.G.Zhang Y.N.Zan Q.Z.wang B.L.Xiao Z.Y.Ma Shi‑Changxu Innovation Center for Advanced Materials Institute of Metal ResearchChinese Academy of SciencesShenyang 110016China Key Laboratory of Nuclear Materials and Safety Assessment Institute of Metal ResearchChinese Academy of SciencesShenyang 110016China Nuclear Equipment division Beijing Institute of Nuclear EngineeringChina Nuclear Power Engineering Co.Ltd.Beijing 100840China department of Materials Science School of Mechanical EngineeringShenyang UniversityShenyang 110044China

B_(4)C/Al composites are widely utilized as neutron absorbing materials for the storage and transportation of spent nuclear *** order to improve the high-temperature mechanical properties of B_(4)C/Al composites,in-situ nano-Al_(2)O_(3)was introduced utilizing oxide on Al powder *** this study,the Al_(2)O_(3)content was adjusted by utilizing spheroid Al powder with varying diameters,thereby investigating the impact of Al_(2)O_(3)content on the tensile properties of(B_(4)C+Al_(2)O_(3))/Al *** was found that the pinning effect of Al_(2)O_(3)on the grain boundaries could hinder the recovery of dislocations and lead to dislocation accumulation at high *** the result,with the increase in Al_(2)O_(3)content and the decrease in grain size,the high-temperature strength of the composites increased *** finest Al powder used in this investigation had a diameter of 1.4μm,whereas the resultant composite exhibited a maximum strength of 251 MPa at room temperature and 133 MPa at 350℃,surpassing that of traditional B_(4)C/Al composites.

关键词： Al matrix composites Neutron absorbing materials (B_(4)C+Al_(2)O_(3))/Al composites High-temperature strength

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Solid-liquid extraction for yttrium recovery using porous polymeric resin(XAd-7) functionalized with d_(2)EHPA

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journal of Rare Earths 2024年第9期42卷 1764-1773,I0005页

作者： P.A.Martínez-Montoya R.G.Sanchez-Alvarado d.Y.Medina-Velazquez M.L.Carrera-jota P.Garnica-Chávez A.de j.Morales-Ramirez Higher School of Chemical Engineering and Extractive Industries ESIQIEIPNDepartment of Metallurgical EngineeringUPALM S/NCol.LindavistaGustavo A.Madero Cd.de MéxicoC.P07738Mexico Metropolitan Autonomous University Department of MaterialsBasic Sciences and EngineeringAv.San Pablo No.180Col.Reynosa TamaulipasCd.de MéxicoC.P02200Mexico Center for Research and Technological Innovation CIITECIPNCda.de CECATIs/nSanta CatarinaAzcapotzalcoC.P02250CDMXMexico

The recovery of yttrium is proposed by applying a solid-liquid extraction process using di-2-ethyl hexylphosphoric acid(d2EHPA) as extracting *** extracting agents were supported on a macro porous polymeric resin XAd-7(solid phase).Yttrium ions extraction and discharge tests were performed,firstly from a synthetic aqueous solution of 100 mg/L Y(liquid phase) at 25℃ with *** effects of pH of aqueous solutions bearing yttrium,volume fraction of extracting agents and the solid/liquid(S/L)ratio on the yttrium recovery were *** most favorable conditions for yttrium ions extraction are;20 vol% d_(2)EHPA functionalized resin,pH=1.5 and an S/L ratio of 10 mg/mL The discharge of yttrium ions was done under the same conditions of extraction stage,using a 2 mol/L [H_(2)SO_(4)] as stripping *** to 80% yttrium is extracted,while 75% yttrium is recovered in the striping *** all experiments,the reaction equilibrium is reached after 20 min,and the kinetics for the extraction stage was determined as a second-order ***,experiments were carried out to discharge the yttriumloaded resins,and it has been determined that the best pH value to strip the Y ions is *** tests of extraction and discharge for yttrium ions show that the functionalized resin can work at least five cycles without decreasing its ***,the proposed process was tested in a real solution with Y ions fro m a waste fluorescent la mp powder leached in H_(2)SO_(4),demonstrating the ability to effectively recover yttrium,separating it from various metals from the studied residue.

关键词： Yttrium recovery Rare earth solid-liquid extraction XAd-7 resin Fluorescent lamps recycling Solid-liquid kinetics extraction

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Highly sensitive ratiometric fluorescent fiber matrices for oxygen sensing with micrometer spatial resolution

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Bio-design and Manufacturing 2024年第3期7卷 292-306页

作者： Giuliana Grasso Valentina Onesto Stefania Forciniti Eliana d’Amone Francesco Colella Lara Pierantoni Valeria Famà Giuseppe Gigli Rui L.Reis j.Miguel Oliveira Loretta L.del Mercato nstitute of Nanotechnology National Research Council(CNR-NANOTEC)c/o Campus Ecoteknevia Monteroni73100 LecceItaly department of Mathematics and Physics“Ennio de Giorgi” University of Salentoc/o Campus Ecoteknevia Monteroni73100 LecceItaly 3B’s Research Group I3Bs–Research Institute on BiomaterialsBiodegradables and BiomimeticsHeadquarters of the European Institute of Excellence on Tissue Engineering and Regenerative MedicineUniversity of MinhoAveParkZona Industrial da Gandra4805-017 BarcoGuimarãesPortugal ICVS/3B’s-PT Government Associate Laboratory 4806-909 Braga/GuimarãesPortugal department of Experimental Medicine University of Salentoc/o Campus Ecoteknevia Monteroni73100 LecceItaly

Oxygen(O_(2))-sensing matrices are promising tools for the live monitoring of extracellular O_(2) consumption levels in long-term cell *** this study,ratiometric O_(2)-sensing membranes were prepared by electrospinning,an easy,low-cost,scalable,and robust method for fabricating ***(ε-caprolactone)and poly(dimethyl)siloxane polymers were blended with tris(4,7-diphenyl-1,10-phenanthroline)ruthenium(II)dichloride,which was used as the O_(2)-sensing probe,and rhodamine B isothiocyanate,which was used as the reference *** functionalized scaffolds were morphologically characterized by scanning electron microscopy,and their physicochemical profiles were obtained by Fourier transform infrared spectroscopy,thermogravimetric analysis,and water contact angle *** sensing capabilities were investigated by confocal laser scanning microscopy,performing photobleaching,reversibility,and calibration curve studies toward different dissolved O_(2)(dO)*** sensing nanofibers showed a high response to changes in dO concentrations in the physiological-pathological range from 0.5%to 20%and good stability under ratiometric *** addition,the sensing systems were highly biocompatible for cell growth promoting adhesiveness and growth of three cancer cell lines,namely metastatic melanoma cell line SK-MEL2,breast cancer cell line MCF-7,and pancreatic ductal adenocarcinoma cell line Panc-1,thus recreating a suitable biological environment in *** O_(2)-sensing biomaterials can potentially measure alterations in cell metabolism caused by changes in ambient O_(2)content during drug testing/validation and tissue regeneration processes.

关键词： Electrospinning Ruthenium(II)dichloride Oxygen sensors Ratiometric imaging Fluorescence

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Changes in human settlement environments and their drivers in valley cities located in arid and semi-arid regions:A case study of Lanzhou in Western China

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Research in Cold and Arid Regions 2024年第3期16卷 149-158页

作者： Rong Zhu YouYan jiang Bo wang YaHui Zhang Gansu Institute of Architectural design and Research LanzhouGansu 730000China College of Earth and Environmental Sciences Lanzhou UniversityLanzhouGansu 730000China Lanzhou Regional Climate Center LanzhouGansu 730020China School of Human Settlements and Civil Engineering Xi’An Jiaotong UniversityXi’AnShanxi 712000China

development of urban human settlement environments(HSEs)is an integral part of promoting high-quality and sustainable regional development and constructing a beautiful *** city of Lanzhou,located at the geometric center of China,is the only provincial capital traversed by the Yellow *** the constraints posed by the valley topography and the need for economic development,the development of this HSE,which is located within an arid region,poses considerable ***,an understanding of the evolution of HSEs and drivers of changes in them contributes to high-quality,sustainable urban development in arid and semi-arid *** analytical model was developed using the parameters of relief degree of land surface,human comfort days,the land cover index,nighttime light index,and *** model was used in combination with population density and the gross domestic product to analyze the spatial distribution of Lanzhou's HSE and its *** results showed that landscapes in Lanzhou underwent significant changes between 2000 and 2022,with an increase in building-up land(+0.946%),cultivated land(+0.134%),and forest land(+0.018%)and a decrease in grassland(-1.10%).There was significant outward expansion of the main urban zone of Lanzhou and of various county towns,with the increase in building-up land being most *** this period,there were significant changes in the periphery of the core urban area and county towns in Lanzhou,with decreases moving from the urban center(the highest value)to the surrounding areas(Yongdeng County had the lowest value).The correlation between the HSE and population density grew stronger in Anning and Chengguan districts but became weaker in Xigu and Qilihe *** variations in the HSE were primarily caused by climate change,followed by human activities,and were also influenced by the valley ***,the spatial distribution of population density and the HSE in Lanzhou d

关键词： Human settlement environment Vegetation index Nighttime light index Human comfort days

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