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Mucopolysaccharidosis typeⅢB:a current review and exploration of the AAV therapy landscape

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neural Regeneration Research 2024年第2期19卷 355-359页

作者： Courtney J.Rouse Victoria n.Jensen Coy D.Heldermon Lacerta Therapeutics AlachuaFLUSA University of Florida College of Medicine GainesvilleFLUSA

Mucopolysaccharidoses typeⅢB is a rare genetic disorder caused by mutations in the gene that encodes for *** results in the aggregation of heparan sulfate polysaccharides within cell lysosomes that leads to progressive and severe debilitating neurological *** treatment options are expensive,limited,and presently there are no approved cures for mucopolysaccharidoses typeⅢ***-associated virus gene therapy has significantly advanced the field forward,allowing researchers to successfully design,enhance,and improve potential *** group recently published an effective treatment using a codon-optimized triple mutant adeno-associated virus 8 vector that restores n-acetyl-alpha-glucosaminidase levels,auditory function,and lifespan in the murine model for mucopolysaccharidoses typeⅢB to that seen in healthy ***,we review the current state of the field in relation to the capsid landscape,adeno-associated virus gene therapy and its successes and challenges in the clinic,and how novel adenoassociated virus capsid designs have evolved research in the mucopolysaccharidoses typeⅢB field.

关键词： adeno-associated virus central nervous system gene therapy heparan sulfate immune response mucopolysaccharidoses type IIIB n-acetyl-alpha-glucosaminidase newborn screening

Detailed seismic zoning of the East Kazakhstan region in the Republic of Kazakhstan

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Geodesy and Geodynamics 2024年第2期15卷 156-165页

作者： natalya V.Silacheva n.P.Stepanenko O.K.Kurilova A.D.Kudabayeva A.T.Danabayeva Institute of Seismology Ministry of Emergency SituationsAlmaty 050060Kazakhstan

Kazakhstan is currently drafting new construction regulations that comply with the major provisions of the *** regulations are created on the basis of seismic zoning maps of various degrees of detail,developed by our Institute of Seismology using a new methodological approach for *** article is about creating the first normative map of the Detailed Seismic Zoning on a probabilistic foundation for the Republic of Kazakhstan’s East Kazakhstan *** carried out the probabilistic assessment of seismic hazard using a methodology consistent with the main provisions of Eurocode 8and updated compared with that used in developing maps of Kazakhstan’s General Seismic Zoning and seismic microzoning of *** most thorough and current data accessible for the area under consideration were combined with contemporary analytical *** have been done to not only the databases being used but also the way seismic sources were shown,including active faults *** a scale of 1:1000000,precise seismic zoning maps of the East Kazakhstan region were created for two probabilities of exceedance:10%and 2%in 50 years in terms of peak ground accelerations and macroseismic *** obtained seismic hazard distribution is generally consistent with the General Seismic Zoning of Kazakhstan’s previous ***,because active faults were included and a thoroughly revised catalog was used,there are more pronounced zones of increased danger along the fault in the western part of the *** the west of the territory,acceleration values also increased due to a more accurate consideration of seismotectonic *** maps are the basis for developing new state building regulations of the Republic of Kazakhstan.

关键词： Probabilistic seismic hazard assessment Detailed seismic zoning Peak ground acceleration Microseismical intensity

Changes in children’s cardiorespiratory fitness and body mass index over the course of the COVID‑19 pandemic:a 34‑month longitudinal study of 331 primary school children

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World Journal of Pediatrics 2024年第2期20卷 185-188页

作者： Gerald Jarnig Reinhold Kerbl Mireille n.M.van Poppel Institute of Human Movement Science Sport and HealthUniversity of GrazMozartgasse 148010 GrazAustria Department of Pediatrics and Adolescent Medicine LKH Hochsteiermark8700 LeobenAustria

In late 2019,heavy restrictions were enforced on public activities worldwide to diminish the spread of the severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)virus[1].These restrictions,which included school closures and strictly limited sports and leisure opportunities,particularly impacted children and adolescents[2].Lifestyle changes in children and adolescents led to reduced physical activity levels[2,3],increased sedentary behavior[3,4],changes in eating habits,and changes in sleep habits[3].

关键词： respiratory acute children

A concise review on surface and structural modification of porous zeolite scaffold for enhanced hydrogen storage

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Chinese Journal of Chemical Engineering 2024年第6期70卷 33-53页

作者： B.A.Abdulkadir R.S.R.Mohd Zaki A.T.Abd Wahab S.n.Miskan Anh-Tam nguyen Dai-Viet n.Vo H.D.Setiabudi Centre for Research in Advanced Fluid&Processes Universiti Malaysia Pahang Al-Sultan AbdullahLebuh Persiaran Tun Khalil YaakobGambangPahang 26300Malaysia Faculty of Chemical&Process Engineering Technology Universiti Malaysia Pahang Al-Sultan AbdullahLebuh Persiaran Tun Khalil YaakobGambangPahang 26300Malaysia Center of Excellence for Green Energy and Environmental nanomaterials(CE@GrEEn) Nguyen Tat Thanh University300A Nguyen Tat ThanhDistrict 4Ho Chi Minh City 755414Viet Nam

Investigating zeolites as hydrogen storage scaffolds is imperative due to their porous nature and favorable physicochemical ***,the storage capacity of the unmodified zeolites has been rather unsatisfactory(0.224%-1.082%(mass))compared to its modified ***,the contemporary focus on enhancing hydrogen storage capacities has led to significant attention towards the utilization of modified zeolites,with studies exploring surface modifications through physical and chemical treatments,as well as the integration of various active *** enhanced hydrogen storage properties of zeolites are attributed to the presence of aluminosilicates from alkaline and alkaline-earth metals,resulting in increased storage capacity through interactions with the charge density of these ***,there is a great demand to critically review their role such as well-defined topology,pore structure,good thermal stability,and tunable hydrophilicity in enhanced hydrogen *** article aimed to critically review the recent research findings based on modified zeolite performance for enhanced hydrogen *** of the factors affecting the hydrogen storage capacities of zeolites that can affect the rate of reaction and the stability of the adsorbent,like pressure,structure,and morphology were studied,and ***,future perspectives,recommendations,and directions for modified zeolites were discussed.

关键词： Zeolites Hydrogen storage Surface modification Adsorption Active metal

A nano-plasmonic HMIM waveguide based concurrent dual-band BPF using circular ring resonator

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Optoelectronics Letters 2024年第3期20卷 152-156页

作者： Miriyala Sridhar Surendra Kumar Bitra TS n Murthy Koppireddi Padmaraju Department of Electronics and Communication Engineering Koneru Lakshmaiah Education FoundationGunturIndia Astra Microwave Products Ltd. HyderabadIndia Department of Electronics and Communication Engineering JNTU-GV College of EngineeringVizianagaramIndia Department of Electronics and Communication Engineering Jawaharlal Nehru Technological University KakinadaKakinadaIndia

This article analyzes the transmission line characteristics of plasmonic hybrid metal insulator metal(HMIM) wave-guide, circular ring resonator(CRR) based dual-band band-pass filters with two transmission poles in both pass-bands in the optical regime using coupled line feed. The transmission line characteristics of an HMIM waveguide, such as characteristic impedance(Z_(PV)), effective refractive index(n_(eff)) and propagation length(L_(spp)), have been obtained by using full wave simulation.

关键词： waveguide resonator circular

Wettability,reactivity,and interface structure in Mg/ni system

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Journal of Magnesium and Alloys 2024年第2期12卷 659-672页

作者： S.Terlicka n.Sobczak Ł.Maj P.Darłak J.J.Sobczak Institute of Metallurgy and Materials Science Polish Academy of Sciences25 Reymonta Str.KrakowPoland Faculty of Foundry Engineering AGH University23 Reymonta Str.KrakowPoland

The sessile drop method was applied to the experimental investigation of the wetting and spreading behaviors of liquid Mg drops on pure ni *** comparison,the experiments were performed in two variants:(1)using the Capillary Purification(CP)procedure,which allows the non-contact heating and squeezing of a pure oxide-free Mg drop;(2)by classical Contact Heating(CH)*** high-temperature tests were performed under isothermal conditions(CP:760℃for 30 s;CH:715℃for 300 s)using Ar+5 wt%H_(2) *** the sessile drop tests,images of the Mg/ni couples were recorded by CCD cameras(57 fps),which were then applied to calculate the contact angles of metal/substrate *** and transmission electron microscopy analyses,both coupled with energy-dispersive X-ray spectroscopy,were used for detailed structural characterization of the solidified *** was found that an oxide-free Mg drop obtained by the CP procedure showed a wetting phenomenon on the ni substrate(an average contact angleθnng and good wetting over the ni substrate(θ_((CP))~20°in 5 s)to form a final contact angle ofθ_(f(CP))~18°.In contrast,a different wetting behavior was observed for the CH procedure,where the unavoidable primary oxide film on the Mg surface blocked the spreading of liquid Mg showing apparently non-wetting behavior after 300 s contact at the test ***,in both cases,the deep craters formed in the ni substrates under the Mg drops and significant change in the structure of initially pure Mg drops to Mg-ni alloys suggest a strong dissolution of ni in liquid Mg and apparent values of the final contact angles measured for the Mg/ni system.

关键词： Magnesium-based alloys Sessile drop tests Capillary purification procedure Wettability Reactivity Contact angle

Robust hybrid bismuth perovskites as potential photocatalysts for overall water splitting

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nano Research 2024年第5期17卷 4593-4601页

作者： Antonio J.Chacón-García Herme G.Baldovi Artem A.Babaryk Antonio Rodríguez-Diéguez Sergio navalón Yolanda Pérez Hermenegildo García Patricia Horcajada Advanced Porous Materials Unit IMDEA Energy InstituteAvda.Ramón y Cajal 328935 MóstolesMadridSpain Departamento de Química Universitat Politècnica de València46022 ValènciaSpain Departamento Química Inorgánica Universidad de Granada1807GranadaSpain Departamento de Biología y Geología Física y Química Inorgánica.ESCET.Universidad Rey Juan Carlos28933 MóstolesMadridSpain Instituto de tecnología química CSIC-UPV Universitat Politècnica de València(UPV)46022Spain

Organic-inorganic hybrid perovskites have gained great attention as promising photocatalysts for hydrogen ***,due to their poor stability in water,the use of aqueous hydrohalic acid solutions is specifically required for an efficient hydrogen ***,three novel photoactive lead-free hybrid perovskites based on bismuth and triazolium cations(denoted as IEF-15,IEF-16,and IEF-17(IEF stands for IMDEA energy frameworks))were synthesized and fully characterized(structural,compositional,optical,etc.).Further,these solids were proposed as photocatalysts for the challenging gas phase overall water splitting(OWS)***,IEF-16 thin films exhibited a remarkable photocatalytic activity in both H_(2) and O_(2) evolution,as a consequence of its appropriate bandgap and energy-band alignment,achieving hydrogen evolution rates ofμmol·g_(H_(2))^(-)1846 and 360 after 24 h under ultraviolet-visible(UV-vis)irradiation or simulated solar irradiation,*** study additionally highlights the remarkable structural and photochemical stability of IEF-16 under different operational conditions(*** volume,irradiation and temperature),paving the way for green hydrogen production from OWS using perovskite-based photocatalysts.

关键词： bismuth-based hybrid perovskites thin films photocatalysis overall water splitting green hydrogen

Mathematical modeling and simulations of stress mitigation by coating polycrystalline particles in lithium-ion batteries

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Applied Mathematics and Mechanics(English Edition) 2024年第6期45卷 947-962页

作者： n.IQBAL J.CHOI S.F.SHAH C.LEE S.LEE Department of Mechanical Roboticsand Energy EngineeringDongguk University-SeoulSeoul 04620Republic of Korea

A chemo-mechanical model is developed to investigate the effects on the stress development of the coating of polycrystalline ni-rich LinixMnyCo_(z)O_(2)(x≥0.8)(nMC)particles with poly(3,4-ethylenedioxythiophene)(PEDOT).The simulation results show that the coating of primary nMC particles significantly reduces the stress generation by efficiently accommodating the volume change associated with the lithium diffusion,and the coating layer plays roles both as a cushion against the volume change and a channel for the lithium transport,promoting the lithium distribution across the secondary particles more ***,the lower stiffness,higher ionic conductivity,and larger thickness of the coating layer improve the stress *** paper provides a mathematical framework for calculating the chemo-mechanical responses of anisotropic electrode materials and fundamental insights into how the coating of nMC active particles mitigates stress levels.

关键词： lithium-ion battery(LIB) polycrystalline particle coating finite element simulation ni-rich LinixMnyCo_(z)O_(2)(x>0.8)(nMC)

The interaction of the structure-directing agent with the zeolite framework determines germanium distribution in SCM-15 germanosilicate

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Frontiers of Chemical Science and Engineering 2024年第5期18卷 105-114页

作者： Stoyan P.Gramatikov Petko St.Petkov Zhendong Wang Weimin Yang Georgi n.Vayssilov Faculty of Chemistry and Pharmacy University of Sofia1126 SofiaBulgaria State Key Laboratory of Green Chemical Engineering and Industrial Catalysis Sinopec Shanghai Research Institute of Petrochemical Technology Co.Ltd.Shanghai 201208China

We report results from computational modeling of the relative stability of germanosilicate SCM-15 structure due to different distribution of germanium heteroatoms in the double four-member rings(D4Rs)of the framework and the orientation of the structure directing agent(SDA)molecules in the as-synthesized *** calculated relative energies of the bare zeolite framework suggest that structures with germanium ions clustered in the same D4Rs,e.g.,with large number of Ge–O-Ge contacts,are the most *** simulations of various orientations of the SDA in the pores of the germanosilicate zeolite show different stability order-the most stable models are the structures with germanium spread among all ***,for SCM-15 the stabilization due to the presence of the SDA and their orientation,is thermodynamic factor directing both the formation of specific framework type and Ge distribution in the framework during the *** relative stability of bare structures with different germanium distribution is of minor *** differs from SCM-14 germanosilicate,reported earlier,for which the stability order is preserved in presence of ***,even for zeolites with the same chemical composition and SDA,the characteristics of their framework lead to different energetic preference for germanium distribution.

关键词： zeolite density functional theory structure-directing agent SCM-15