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Genotypes and Phenotypes of Uygur Children with Xeroderma Pigmentosum: A Case Study in Xinj.ang, China

International j.urnal of Dermatology and Venereology 2024年第3期7卷 144-149页

作者： Qiu Y. wang.w.-j. Liang j.-Q. Yakeya B. wang P. Zhang j.-Z. Kang X.-j. Department of Dermatology and Venereology People's Hospital of Xinjiang Uygur Autonomous Region Xinjiang Uygur Autonomous Region Urumqi 830001 China Xinj.ang Clinical Research Center for Dermatologic Diseases Xinjiang Uygur Autonomous Region Urumqi 830001 China Xinj.ang Key Laboratory of Dermatology Research (Xj.S1707) Xinjiang Uygur Autonomous Region Urumqi 830001 China

Obj.ctive: Xeroderma pigmentosum (XP) is a rare autosomal recessive dermatosis caused by genetic defects of DNA repair. This study was performed to detect and analyze the genes of 2 Uygur patients with XP and their families and assess the patients' phenotypes, which may enrich the understanding of the genetic skin disorder spectrum in Xinj.ang area. Methods: we collected the clinical data from 2 patients with XP and peripheral blood samples from the patients and their family members. The patients' DNA was sequenced and detected by Sanger sequencing, and gene mutations were screened. Results: The proband in family 1 presented with brown maculae at the exposure site and squamous cell carcinoma secondary to a facial rash. The proband had a homozygous nucleotide variation of XPC c.2251-2A> G (A change from A to G in the penultimate position of the intron before the 2251 position in the coding region), which was a shear mutation. In this family, both parents were heterozygous, and no similar mutation detected in the sister. In family 2, the proband had scattered black brown spots and papules on the trunk and limbs. and his younger sister was also a patient. The proband and his younger sister had homonucleotide variation of XPA c.631C> T, which was nonsense mutation, resulting in the codon for Arg No.211 being changed into termination codon (***211X), thus terminating the peptide chain synthesis prematurely. All the normal individuals in the two families were heterozygotes, and homozygous mutations occurred in all the patients, which was consistent with the autosomal recessive inheritance. Conclusion: XP is rare in Uygur population. This study expanded the mutation spectrum of XP and provided a basis for early diagnosis, treatment, prognostic prediction, and prenatal genetic consultation. © 2022 Hospital for Skin Diseases (Institute of Dermatology), Chinese Academy of Medical Sciences, and Chinese Medical Association, published by wolters Kluwer, Inc.

关键词： gene mutation xeroderma pigmentosum XPA gene XPC gene

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Compressive property and energy absorption characteristic of interconnected porous Mg-Zn-Y alloys with adj.sting Y addition

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j.urnal of Magnesium and Alloys 2024年第1期12卷 171-185页

作者： j.A.Liu S.j.Liu B.wang.w.B.Sun X.j.Liu Z.w.Han College of Materials Science and Engineering Key Laboratory of Automobile Materials(Ministry of Education)Jilin UniversityChangchun 130022PR China Key Laboratory of Bionic Engineering(Ministry of Education) College of Biological and Agricultural EngineeringJilin UniversityChangchun130022PR China weihai Institute for Bionics-j.lin University Weihai 264205PR China

In this study,interconnected porous Mg-2Zn-xY alloys with different phase compositions were prepared by various Y additions(x=0.4,3,and 6 wt.%)to adj.st the compressive properties and energy absorption *** characterization methods were then applied to identify the microstructure of the porous Mg-Zn-Y and describe the details of the second *** tests were performed at room temperature(RT),200℃,and 300℃to study the impact of the Y addition and testing temperature on the compressive properties of the porous *** experimental results showed that a high Y content promotes a microstructure refinement and increases the volume fraction of the second *** the Y content increases,different Mg-Zn-Y ternary phases appear:I-phase(Mg_(3)Zn_(6)Y),w-phase(Mg_(3)Zn_(3)Y_(2)),and LPSO phase(Mg_(12)ZnY).when the Y content ranges between 0.4%and 6%,the compressive strength increases from 6.30MPa to 9.23 MPa,and the energy absorption capacity increases from 7.33 Mj.m^(3)to 10.97 Mj.m^(3)at RT,which is mainly attributed to the phase composition and volume fraction of the second ***,the average energy absorption efficiency is independent of the Y *** addition,the compressive deformation behaviors of the porous Mg-Zn-Y are altered by the testing *** compressive strength and energy absorption capacity of the porous Mg-Zn-Y decrease due to the softening effect of the high temperature on the *** deformation behaviors at different temperatures are finally observed to reflect the failure mechanisms of the struts.

关键词： Porous magnesium Rare earth elements Microstructure Compressive behavior Energy absorption characteristic

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Artificial-intelligent-powered safety and efficiency improvement for controlling and scheduling in integrated railway systems

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High-speed Railway 2024年第3期2卷 172-179页

作者： Liu j. Liu G. wang Y. Zhang w. Beij.ng National Railway Research & Design Institute of Signal & Communication Group Co. Ltd Beijing 100070 China Engineering Research Center of Railway Industry of Intelligent and Autonomous Train Control Beijing 100070 China

The multi-mode integrated railway system, anchored by the high-speed railway, caters to the diverse travel requirements both within and between cities, offering safe, comfortable, punctual, and eco-friendly transportation services. with the expansion of the railway networks, enhancing the efficiency and safety of the comprehensive system has become a crucial issue in the advanced development of railway transportation. In light of the prevailing application of artificial intelligence technologies within railway systems, this study leverages large model technology characterized by robust learning capabilities, efficient associative abilities, and linkage analysis to propose an Artificial-intelligent (AI)-powered railway control and dispatching system. This system is elaborately designed with four core functions, including global optimum unattended dispatching, synergetic transportation in multiple modes, high-speed automatic control, and precise maintenance decision and execution. The deployment pathway and essential tasks of the system are further delineated, alongside the challenges and obstacles encountered. The AI-powered system promises a significant enhancement in the operational efficiency and safety of the composite railway system, ensuring a more effective alignment between transportation services and passenger demands. © 2024 Beij.ng j.aotong University

关键词： Artificial intelligence High-speed railway Large-scale mode, system framework Multi-mode railway system Safety and efficiency improvement

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In situ digital testing method for quality assessment of soft soil improvement with polyurethane

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j.urnal of Rock Mechanics and Geotechnical Engineering 2024年第5期16卷 1732-1748页

作者： X.F.wang C.j.wang.w.V.Yue Z.j.Zhang Z.Q.Yue Department of Civil Engineering China University of Geosciences (Beijing)BeijingChina Department of water Science and Engineering Zhengzhou UniversityZhengzhouChina Department of Geotechnical Engineering Tongji UniversityShanghaiChina Department of Civil Engineering The University of Hong KongChina

This study purposes an in situ testing method on quality assessment of soil *** drilling data includes the spatial distribution and in situ strength of untreated and treated soil along three different drillholes measured by on-site drilling monitoring *** factual drilling data can characterize the degree of soil improvement by penetration inj.ction with permeable *** from on-site drilling monitoring shows that the linear zones represent constant drilling speeds shown in the plot of drill bit advancement *** drilling time,which indicates the spatial distributions of soil *** soil profile at the study site is composed of four layers,which includes fill,untreated silty clay,treated silty clay,and mucky *** results of soil profile are verified by the parallel site *** constant drilling speeds profile the coring-resistant strength of drilled *** comparing with the untreated silty clay,the constant drilling speeds of the treated silty clay have been decreased by 13.0-62.8%.Two drilling-speed-based indices of 61.2%and 65.6%are proposed to assess the decreased average drilling speed and the increased in situ strength of treated silty *** tests,*** compressive strength(UCS)test,have been performed with core sample to investigate and characterize in situ strength by comparing that with drilling *** show that the average predicted strengths of treated silty clay are 2.4-6.9 times higher than the average measured strength of untreated silty *** UCS-based indices of 374.5%and 344.2%verified the quality assessment(QA)results by this new in situ *** method provides a cost-effective tool for quality assessment of soil improvement by utilizing the digital drilling data.

关键词： Drilling process monitoring system Hydraulic rotary drilling process Constant drilling speed Soil improvement Quality assessment

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Advances in magnesium-containing bioceramics for bone repair

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Biomaterials Translational 2024年第1期5卷 3-20页

作者： Qi L. Zhao T. Yan j. Ge w. j.ang w. wang j. Gholipourmalekabadi M. Lin K. wang X. Zhang L. Department of Oral and Cranio-Maxillofacial Surgery Shanghai Ninth People’s Hospital College of Stomatology Shanghai Jiao Tong University School of Medicine National Clinical Research Center for Oral Diseases Shanghai Key Laboratory of Stomatology and Shanghai Research Institute of Stomatology Shanghai China Department’s Education Deputy Department of Medical Biotechnology Iran University of Medical Sciences Tehran Iran Institute of Translational Medicine Shanghai University Organoid Research Center Shanghai University Shanghai China

Reconstruction of bone defects or fractures caused by ageing, trauma and tumour resection is still a great challenge in clinical treatment. Although autologous bone graft is considered as gold standard, the source of natural bone is limited. In recent years, regenerative therapy based on bioactive materials has been proposed for bone reconstruction. Specially, numerous studies have indicated that bioactive ceramics including silicate and phosphate bioceramics exhibit excellent osteoinductivity and osteoconductivity, further promote bone regeneration. In addition, magnesium (Mg) element, as an indispensable mineral element, plays a vital role in promoting bone mineralisation and formation. In this review, different types of Mg-containing bioceramics including Mg-containing calcium phosphate-based bioceramics (such as Mg-hydroxyapatite, Mg-biphasic calcium phosphate), Mg-containing calcium silicate-based bioceramics (such as Mg2SiO4, Ca2MgSi2O7and Mg-doped bioglass), Mg-based biocements, Mg-containing metal/polymer-bioceramic composites were systematacially summarised. Additionally, the fabrication technologies and their materiobiological effects were deeply discussed, Clinical applications and perspectives of magnesium-containing bioceramics for bone repair are highlighted. Overall, Mg-containing bioceramics are regarded as regenerative therapy with their optimised performance. Furthermore, more in-depth two-way researches on their performance and structure are essential to satisfy their clinical needs. © 2024, Chinese Medical Multimedia Press Co Ltd. All rights reserved.

关键词： bioactive ions bioceramics bone repair magnesium osteoconductivity

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Superexchange and charge transfer in the nickelate superconductor La_(3)Ni_(2)O_(7) under pressure

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Science China(Physics,Mechanics & Astronomy) 2024年第11期67卷 1-8页

作者： wéi wú Zhihui Luo Dao-Xin Yao Meng wang Center for Neutron Science and Technology Guangdong Provincial Key Laboratory of Magnetoelectric Physics and DevicesSchool of PhysicsSun Yat-sen UniversityGuangzhou 510275China

Recently, a bulk nickelate superconductor La_(3)Ni_(2)O_(7) is discovered at pressures with a remarkable high transition temperature T_(c)~ 80 K. Here, we study a Hubbard model with tight-binding parameters derived from ab initio calculations of La_(3)Ni_(2)O_(7),by employing large scale determinant quantum Monte Carlo and cellular dynamical mean-field theory. Our result suggests that the superexchange couplings in this system are comparable to that of cuprates. The system is a charge transfer insulator as the hole concentration becomes four per site at large Hubbard U. Upon hole doping, two low-energy spin-singlet bands emerge in the system exhibiting distinct correlation properties: while the one composed of the out-of-plane Ni-d_(3z^(2)-r^(2)) and O-p_(z) orbitals demonstrates strong antiferromagnetic correlations and narrow effective bandwidth, the in-plane singlet band consisting of the Ni-d_(x^(2)-y^(2)) and O-p_(x)/p_(y) orbitals is in general more itinerant. Over a broad range of hole doping, the doped holes occupy primarily the d_(x^(2)-y^(2)) and p_(x)/p_(y) orbitals, whereas the d_(3z^(2)-r^(2)) and p_(z) orbitals retain underdoped. we propose an effective t-j.model to capture the relevant physics and discuss the implications of our result for comprehending the La_(3)Ni_(2)O_(7) superconductivity.

关键词： superconductivity nickelate superconductors Hubbard model superexchange charge transfer insulator quantum Monte Carlo

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Error field penetration in j.TEXT tokamak based on two-fluid drift-MHD model

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Chinese Physics B 2024年第4期33卷 545-551页

作者：王文徐涛张仪 the j.TEXT team State Key Laboratory of Advanced Electromagnetic Technology International Joint Research Laboratory of Magnetic Confinement Fusion and Plasma PhysicsSchool of Electrical and Electronic EngineeringHuazhong University of Science and TechnologyWuhan 430074China

An externally generated resonant magnetic perturbation can induce complex non-ideal MHD responses in their resonant *** have studied the plasma responses using Fitzpatrick's improved two-fluid model and program *** calculated the error field penetration threshold for *** addition,we find that the island width increases slightly as the error field amplitude increases when the error field amplitude is below the critical penetration ***,the island width suddenly j.mps to a large value because the shielding effect of the plasma against the error field disappears after the *** scanning the natural mode frequency,we find that the shielding effect of the plasma decreases as the natural mode frequency ***,we obtain the m/n=2/1 penetration threshold scaling on density and temperature.

关键词： plasma responses drift-MHD model error field penetration

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A hydrodynamic modelling of wave-activated bodies for wavelet marine regions

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CLEAN ENERGY 2024年第2期8卷 35-47页

作者： Peh, j. S. Muhieldeen, Mohammed w. Shuaib, N. H. UCSI Univ Fac Engn Technol & Built Environm Mech Engn Dept Kuala Lumpur 56000 Malaysia MTC Engineer Sdn Bhd Shah Alam Selangor Malaysia

Recently, most wave energy research has targeted sea areas with high power density under extreme wave conditions for deployment. This trend often leads to higher energy costs for wave power plants. However, many marine areas with low power density, characterized by wavelet conditions and less destructive forces than extreme wave conditions, remain underexplored. Therefore, this study aimed to propose a wave-activated body model for sea regions with wavelet conditions. The wave-activated body design process encompassed site selection, parameter determination, geometry design, comparison and performance evaluation using the ANSYS (R) AQwA model. The results indicated that the proposed device achieved the desired heave motion, with an amplitude range of 1.2 to -2.5 m, validating its potential for deployment in marine regions with wavelet conditions. Notably, while the proposed design is optimized for wavelet conditions, it was found to have potential limitations in extreme wave environments. This observation emphasizes the challenge of formulating a generalized design suitable for both conditions. Consequently, it is pivotal for wave-activated body designs to be customized based on the specific ocean conditions they target, underscoring the need for specialized designs of wave energy converters that consider the unique wave characteristics of their deployment sites. A wave-activated body is designed for energy generation in ocean regions with wavelet conditions. Hydrodynamic modelling is carried out for two locations with different wave characteristics to determine the optimum conditions for deployment. Graphical Abstract

关键词： wave energy converter South China sea renewable and sustainable energy wavelet ANSYS AQwA hydrodynamic wave-activated bodies ENERGY IMPACT

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Effects of different ratios of soluble to insoluble dietary fiber on growth performance and intestinal health of piglets

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ANIMAL NUTRITION 2024年第3期18卷 257-271页

作者： Feng, Luya Luo, Zhenfu wang, j.ng wu, Kunfu wang, wenliang Liu, Zhimou wen, j.ping wang, Zhenbin Duns, Gregory j. Ma, Xiaokang Tan, Bi'e Hunan Agr Univ Coll Anim Sci & Technol Hunan Prov Key Lab Prod Qual Regulat Livestock & P Changsha 410128 Peoples R China Yuelushan Lab Changsha 410128 Peoples R China Hunan Nuoze Biol Technol Co Ltd Yiyang 413001 Peoples R China

This study investigated the impact of different ratios of soluble to insoluble dietary fiber (SDF:IDF) formulations by sugar beet pulp (SBP) supplementation on piglet growth performance, nutrient digestibility, immune function, intestinal morphology, intestinal microbiota and intestinal health. A total of 60 crossbred piglets (Duroc x [Landrace x Yorkshire]) at 40 d old with body weight of 10.0 +/- 0.3 kg were randomly assigned to 5 treatments with 6 replicates per treatment and 2 piglets per replicate in a 21d trial. The dietary treatments included a corn-soybean meal diet (0% SBP supplementation; CON), and diets supplemented with 2%, 4%, 6%, and 8% SBP, representing different SDF:IDF ratios at 10.16%, 13.53%, 16.79%, 19.86%, and 24.81%, respectively. The results indicated that the 8% SBP treatment had a negative effect on feed-to-gain ratio (linear, P = 0.009) compared with the CON treatment (P = 0.021). The apparent total tract digestibility (ATTD) of crude protein was lower in treatments supplemented with SBP (P = 0.002) and showed a linear decrease (P = 0.001), while the ATTD of IDF showed a linear increase (P = 0.037) in four SBP treatments compared to the CON treatment. The 4% SBP treatment increased serum concentrations of triglyceride (quadratic, P = 0.019) and K (linear, P with the CON treatment. The concentrations of Cit, Cys, Ile, Leu, Orn, Arg, taurine, urea, 1-methylhistidine, a-aminoadipic acid, aaminobutyric acid and cystathionine in the 4% SBP treatment were highest among all treatments (P wth factor-b, and tumor necrosis factor-a in the 6% SBP treatment were higher than those in the CON treatment (P which also increased mucin-2 and G protein-coupled receptor 41 mRNA expression (P with the CON treatment and improved th

关键词： Soluble dietary fiber Insoluble dietary fiber Sugar beet pulp Growth performance Intestinal health Piglet SUGAR-BEET PULP CHAIN FATTY-ACIDS NUTRIENT DIGESTIBILITY MICROBIOTA COMPOSITION FECAL MICROBIOTA BARRIER FUNCTION wEANING STRESS AMINO-ACIDS wHEAT METABOLISM

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Rescattering of stimulated Raman side scattering in nonuniform plasmas

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Matter and Radiation at Extremes 2024年第5期9卷 68-76页

作者： S.Tan Q.wang Y.Chen w.B.Yao C.Z.Xiao j.F.Myatt School of Physics and Electronics Hunan UniversityChangsha 410082China Institute of Applied Physics and Computational Mathematics Beijing 100094China School of Electrical and Information Engineering Anhui University of Science and TechnologyHuainanAnhui 232001China Department of Electrical and Computer Engineering University of Alberta9211116 St.NWEdmontonAlberta T6G 1H9Canada

Rescattering of stimulated Raman side scattering(SRSS)is observed for the first time via two-dimensional(2D)particle-in-cell(PIC)*** construct a theoretical model for the rescattering process,which can predict the region of occurrence of mth-order SRSS and estimate its *** rescattering process is identified by the 2D PIC simulations under typical conditions of a direct-drive inertial confinement fusion *** electrons produced by second-order SRSS propagate nearly perpendicular to the density gradient and gain nearly the same energy as in first-order SRSS,but there is no cascade acceleration to produce superhot *** studies for a wide range of ignition conditions show that SRSS and associated rescatterings are robust and important processes in inertial confinement fusion.

关键词： nonuniform acceleration scattering

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