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Genes & Diseases 2024年第3期11卷 66-68页

作者： Aswathi n Jincy M.Jose Sreelata b.nair Prabha S.n Pappachan S Department of Medical Genetics Lifeline Super Specialty HospitalPathanamthittaKerala 691523India Lifeline Super Specialty Hospital PathanamthittaKerala 691523India

Alagille syndrome(ALGS;OMIM#118450)is a highlyvariable,multi-systemic,autosomal dominant disorder,caused by mutations in the Jagged Canonical notch Ligand 1(JAG1)gene(20p12.2)or in the neurogenic locus notch homolog protein 2(nOTCH2)gene(1p13).^(1)It primarily affects the liver,heart,eyes,face,kidney,skin,vertebrae,and skeleton.^(1,2)The JAG1 gene has 26 exons and about 90%of the ALGS cases result from pathogenic variants in the JAG1 and around 7%of cases have deletions in chromosome 20 that include this gene.

关键词： JAG1 cases notch

Design of biologically Active Macromolecular Ligands and Their Luminescent Complexes with Terbium Ions

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Chinese Journal of Polymer Science 2024年第5期42卷 604-611,I0007页

作者： Tatiana n.nekrasova Andrei I.Fischer natalia A.nesterova Anatoliy v.Dobrodumov Anzhelika v.Garshinina Olga D.Shuvaeva natalya v.Zakharova Evgenii F.Panarin Institute of Macromolecular Compounds Russian Academy of Sciences31 Bolshoy pr.St Petersburg199004Russia Smorodintsev Research Institute of Influenza Russian Ministry of Health15/17 ul.prof.PopovaSt Petersburg197376Russia

Water-soluble copolymers of p-methacrylamidobenzoic acid(MAbA)with neutral comonomers(n-vinylpyrrolidone(vP),n-methyl-n-vinylacetamide(MvAA),n-methacryloyl glucosamine(MAG))and anionic comononer sodium styrene sulfonate(naSS)were synthesized by radical *** interactions between the prepared copolymers and Tb^(3+)ions in aqueous solutions were studied;the significant influence of chemical structure of a comonomer on luminescence intensity of Tb^(3+)complexes with the copolymers was *** luminescence intensity of Tb^(3+)complexes with the copolymers containing n-vinylamide units(vP,MvAA)is three times more intense than that observed for the complexes between Tb^(3+)and MAG-containing *** the case of naSS-containing copolymers,the luminescence intensity is controlled by the values of binding constants between Tb^(3+)and MAbA and the content of MAbA units in a *** studied copolymers and their complexes with Tb^(3+)have low cytotoxicity and a pronounced antiviral activity against human respiratory syncytial virus.

关键词： Luminescence Water-soluble copolymers Lanthanide complexes Antiviral activity Cytotoxicity

Astrophysical S-factor and reaction rate for 15n(p,γ)16O within the modified potential cluster model

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Chinese Physics C 2024年第4期48卷 130-145页

作者： S.b.Dubovichenko A.S.Tkachenko R.Ya.Kezerashvili n.A.burkova b.M.Yeleusheva Fesenkov Astrophysical Institute 050020 AlmatyKazakhstan new York City College of Technology City University of New YorkBrooklyn11201 New YorkUSA Graduate School and University Center City University of New York10016 New YorkUSA al-Farabi Kazakh national University 050040 AlmatyKazakhstan

We study radiative p^(15)n capture on the ground state of ^(16)O at stellar energies within the framework of a modified potential cluster model(MPCM)with forbidden states,including low-lying *** investigation of the ^(15)n(p,γ0)^(16)O reaction includes the consideration of ^(3)S_(1) resonances due to E1 transitions and the contribution of the ^(3)P_(1) scattering wave in the p+^(15)n channel due to the ^(3)P_(1)→^(3)P_(0)M1 *** calculated the astrophysical low-energy S-factor,and the extrapolated S(0)turned out to be within 34.7−40.4 kev·*** important role of the asymptotic constant(AC)for the ^(15)n(p,γ0)16O process with interfering ^(3)S_(1)(312)and ^(3)S_(1)(962)resonances is elucidated.A comparison of our calculation for the S-factor with existing experimental and theoretical data is addressed,and a reasonable agreement is *** reaction rate is calculated and compared with the existing *** has negligible dependence on the variation of AC but shows a strong impact of the interference of ^(3)S_(1)(312)and ^(3)S_(1)(962)resonances in reference to the CnO Gamow windows,especially at low *** estimate the contribution of cascade transitions to the reaction rate based on the exclusive experimental data from ***.C.85,065810(2012).The reaction rate enhancement due to the cascade transitions is observed from T_(9)>0.3 and reaches the maximum factor~1.3 at T_(9)=*** present the Gamow energy window and a comparison of rates for radiative proton capture reactions ^(12)n(p,γ)^(13)O,^(13)n(p,γ)^(14)O,^(14)n(p,γ)^(15)O,and ^(15)n(p,γ)^(16)O obtained in the framework of the MPCM and provide the temperature windows,prevalence,and significance of each process.

关键词： low and astrophysical energies p^(15)n system thermonuclear reaction rate potential cluster model CnO-cycle

Output feedback adaptive super twisting sliding mode control for quadrotor UAvs

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Control Theory and Technology 2024年第1期22卷 92-105页

作者： Oscar Salas-Peña Jesus DeLeón-Morales Susana v.Gutiérrez-Martínez École Centrale de nantes-LS2n UMR 6004 CNRS44300NantesFrance Facultad de Ingeniería Mecánica y Eléctrica Universidad Autónoma de Nuevo León66451San Nicolás de Los GarzaMexico

In this paper, a sliding mode control with adaptive gain combined with a high-order sliding mode observer to solve the tracking problem for a quadrotor UAv is addressed, in presence of bounded external disturbances and parametric uncertainties. The high order sliding mode observer is designed for estimating the linear and angular speed in order to implement the proposed scheme. Furthermore, a Lyapunov function is introduced to design the controller with the adaptation law, whereas an analysis of finite time convergence towards to zero is provided, where sufficient conditions are obtained. Regarding previous works from literature, one important advantage of proposed strategy is that the gains of control are parameterized in terms of only one adaptive parameter, which reduces the control effort by avoiding gain overestimation. numerical simulations for tracking control of the quadrotor are given to show the performance of proposed adaptive control–observer scheme.

关键词： Adaptive super twisting controller High-order sliding modes observer UAv

Prompt neutron emission in ^(250)no spontaneous fission associated with ground and isomeric state decays

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Chinese Physics C 2024年第6期48卷 112-117页

作者： R.S.Mukhin A.v.Isaev A.v.Andreev M.L.Chelnokov v.I.Chepigin H.M.Devaraja I.n.Izosimov A.A.Kuznetsova O.n.Malyshev A.G.Popeko Yu.A.Popov A.Rahmatinejad b.Sailaubekov T.M.Shneidman E.A.Sokol A.I.Svirikhin M.S.Tezekbayeva M.A.bychkov A.v.Yeremin O.Dorvaux b.Gall K.Kessaci K.Hauschild A.Lopez-Martens Joint Institute for nuclear Research Dubna141980Russia Dubna State University Dubna141982Russia Institute of nuclear Physics Almaty050032Kazakhstan L.n.Gumilyov Eurasian national University Astana010008Kazakhstan Kazan Federal University Kazan420008Russia Universitéde Strasbourg CNRSStrasbourg67000France IJCLab IN2P3-CNRSUniversitéParis-SaclayOrsay91400France

The complete-fusion reaction^(204)Pb(^(48)Ca,2n)^(250)no was used to study two activities of^(250)no with distinct half-lives.A total of 1357 events were observed in the SFinx neutron detection *** average number of neutrons emitted per spontaneous fission of^(250)no was determined to be(4.1±0.1).The unusually symmetrical shape of the prompt neutron multiplicity distribution was restored and presented for the first *** tests were performed to compare the prompt neutron multiplicity distributions associated with the ground state and K-isomer state decays.

关键词： spontaneous fission prompt neutrons actinide

Old-growth mixed dipterocarp forests show variable losses and gains in aboveground biomass and standing carbon over forty years

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Forest Ecosystems 2024年第1期11卷 14-23页

作者： Sisira Ediriweera Champika bandara Tithira Lakkana Shanika Jayasinghe David Woodbury Arun Dayanandan b.M.P.Singhakumara Xiangcheng Mi I.A.U.n.Gunatilleke C.v.S.Gunatilleke Mark SAshton Department of Science and Technology Uva Wellassa UniversityBadullaSri Lanka The Forest School Yale School of the EnvironmentYale UniversityNew HavenCT06511USA Department of Forestry and Environmental Science University of Sri JayewardenepuraNugegodaSri Lanka State Key Laboratory of vegetation and Environmental Change Institute of BotanyChinese Academy of SciencesBeijing100093China botany Department University of PeradeniyaPeradeniyaSri Lanka

background:no studies have documented long-term trends in aboveground biomass(AGb)for mixed-dipterocarp forests(MDF),the dominant rain forest type in tropical wet equatorial *** our study,we sought to document such trends over forty years across three sites representing lowland to lower montane ***:To do this,we established fifty 100 m×25 m plots in 1978 across three sites sampled along an elevation gradient,identified as mature old-growth *** measured trees for diameter at breast height that we identified to species and *** took wood samples to calculate species wood-specific *** re-measured plots in 1998 and again in ***:We show standing AGb for all sites combined to be 517.52 Mg·ha^(-1)in 1978,but this declined by 17%over 40 years to 430.11 Mg·ha^(-1).no differences exist among sites in AGb primarily because of considerable within site variation;but interactions of time with site show declines across sites were not uniform,one remained about the *** few species represented a high proportion of the AGb with the top five species comprising between 34%and 65%,depending upon site and year *** species,Mesua nagassarium,represented a disproportionately large amount of AGb and decline over time,particularly at the low elevation ***:Our results are directly relevant to estimating AGb and standing carbon sequestered in *** study is the first to demonstrate varying but overall,declining trends in amounts of AGb among forests making predictions of biomass and standing carbon in MDF difficult over wide regions.

关键词： Western ghats Shorea spp. Southeast Asia Sri Lanka Tropical rain forest Wood specific gravity

Lithium:A review of applications,occurrence,exploration,extraction,recycling,analysis,and environmental impact

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Geoscience Frontiers 2024年第5期15卷 1-46页

作者： v.balaram M.Santosh M.Satyanarayanan n.Srinivas Harish Gupta CSIR-national Geophysical Research Institute(nGRI) Hyderabad 500007India School of Earth Sciences and Resources China University of GeosciencesBeijing 100083China Department of Earth Science University of AdelaideAdelaide SA 5005Australia Department of Environmental Studies GITAM Institute of ScienceGandhi Institute of Technology&Management(GITAM)Visakhapatnam 530045India Department of Civil Engineering Osmania UniversityHyderabad 500007India

The energy transition challenges faced by modern civilization have significantly enhanced the demand for critical metals like lithium resulting in improved methods to explore,extract,and utilize these *** this comprehensive review,we discuss the different types of lithium resources,factors,and mechanisms controlling lithium enrichment in various geological settings including terrestrial and marine *** exploration strategies including geological,geophysical,mineralogical,geochemical,and remote sensing techniques including drone-based techniques for lithium exploration studies in different terranes are *** overview of the mining techniques,including beneficiation and extraction,and their principles,mechanisms,operations,and comparison of the various approaches and compatibility with different types of lithium deposits for obtaining maximum yield are *** isotopic studies are useful in understanding geological processes such as past weathering events and riverine input into the oceans,as well as in understanding the source of lithium in diverse types of *** also highlight the recent developments in other areas such as recycling,environmental impact,and state-of-the-art analytical techniques for determining lithium in different lithium ore deposits and other geological *** overview provides the latest developments and insights in the various sectors related to lithium and prompt further developments to meet the growing demand for this valuable metal as the world transforms to clean energy.

关键词： Lithium minerals Pegmatite Lithium brine deposits Lithium sedimentary deposits Exploration techniques

Effect of proton irradiation on the optical properties of thermal control coating based on polystyrene with silica filler

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Propulsion and Power Research 2024年第1期13卷 1-11页

作者： n.I.Cherkashina v.I.Pavlenko M.M.Mikhailov A.n.Lapin S.A.Yuriev R.v.Sidelnikov D.S.Romanyuk belgorod State Technological University named after v.G.Shoukhov Kostyukov Str.46Belgorod 308012Russia Tomsk State University of Control Systems and Radioelectronics 40Prospect LeninaTomsk 634050Russia

This paper presents the results of studying the surface properties changes of a ther-moregulating coating based on polystyrene and silicaﬁller after proton irradiation with an en-ergy of 50 kev at afluence of 3�1015 cm�*** proton irradiation,the values of the contact angle of wetting with water increase by 3.5%and 14.9%for polystyrene and the coating,*** free surface energy(energy of the surface layer)of polystyrene and the coat-ings before and after proton irradiation was calculated using the Owens-Wendt-Rabel-Kaelble *** was a signiﬁcant increase in the polarity of the polystyrene surface(gp increased by a factor of 2.2)after proton *** the coating,an increase in gp by a factor of 3.89 was observed after proton *** changes in the coating were presented by IR Fourier spectroscopy.A slight decrease in the absorption intensity of all characteristic bands compared to the unirradiated sample was *** was found that the irradiation of the coating with protons led to the formation of macromolecules with hydroxyl,carbonyl,and carboxyl bonds,as well as the formation of dimeric and oligomeric siloxane *** was also found that after irradiation of a pure polystyrene sample with protons,the value of the solar absorption as increased by only 4.2%;whereas for the coating with silicaﬁller,the value of as increased by 28.6%.

关键词： Thermal control coating Proton irradiation Hydrophobicity Free surface energy Radiation crosslinking

Artificial enhancement of sturgeon stock in freshwater reservoirs:A case study on sterlet Acipenser ruthenus of the Kama reservoir

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Aquaculture and Fisheries 2024年第2期9卷 287-294页

作者： Pavel b.Mikheev Semyon n.Kazarinov Alla G.Melnikova Stanislav v.Ponosov nikolai G.Petrenko Andrei I.nikiforov Alexey Yu Puzik Olga n.Elchenkova Perm State national Research University Perm614990Russia Khabarovsk branch of the Federal State budget Scientific Institution“Russian Federal Research Institute of Fisheries and Oceanography”“KhabarovsknIRO” Khabarovsk680038Russia Perm branch of the Federal State budget Scientific Institution“Russian Federal Research Institute of Fisheries and Oceanography”“PermnIRO” Perm614991Russia Federal State budget Scientific Institution“Russian Federal Research Institute of Fisheries and Oceanography”“vnIRO” Moscow117454Russia Moscow State Institute of International Relations(University)of the Ministry for Foreign Affairs of Russia“MGIMO” Moscow117454Russia Department of State Control Supervision and Protection of Aquatic Biological Resources for Perm KraiMiddle-Volga Regional Office of the Federal Fishery AgencyPerm614990Russia

The paper presents the results of the sterlet sturgeon Acipenser ruthenus reintroduction into the Kama Reservoir(Perm Krai,Russia)by sterlet fry from hatcheries into the reservoir since *** effectiveness of hatchery releases was evaluated by monitoring gillnet sampling *** results showed that the species was reported throughout entire Kama Reservoir,which contrasts with 1988-1994 data,when the species was registered only at the upper part of the reservoir near the Kama River *** highest number and biomass values of catch per unit effort(CPUE)of the sterlet were found in submerged river channels-the deepest habitats with flow conditions which are close to the ecological requirements of the *** distribution of the sterlet within the channel biotopes of the reservoir is defined by reduced *** biggest individuals and the widest size range of the sterlet were typical for the upper area of the reservoir and Chusovskoy bay,which have the greatest *** smallest sizes were typical for fish from catches near the dam of the reservoir,which is consistent with the published data on the distribution of sterlet in reservoir *** presented results may indicate the artificial formation of the sterlet stock in the Kama Reservoir maintained by hatchery *** revealed distribution of the sterlet in the reservoir determines the direction of further research on natural reproduction and thus formation of an artificially formed ***,future studies are required to assess the possibility of hybridization of the sterlet of artificial origin with natural populations of the species,since the low genetic diversity of the sterlet released into the Kama Reservoir which can lead to irreversible loss of genetic individuality of the natural stocks of the species in the catchment of upper course of the Kama River.

关键词： Sterlet Acipenser ruthenus Kama reservoir Hatchery releases CPUE Artificial stock formation