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Genotypes and Phenotypes of Uygur Children with x.roderma Pigmentosum: A Case Study in x.njiang, China

International Journal 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 x.njiang Clinical Research Center for Dermatologic Diseases Xinjiang Uygur Autonomous Region Urumqi 830001 China x.njiang Key Laboratory of Dermatology Research (x.YS1707) Xinjiang Uygur Autonomous Region Urumqi 830001 China

Objective: x.roderma pigmentosum (x.) 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 x. and their families and assess the patients' phenotypes, which may enrich the understanding of the genetic skin disorder spectrum in x.njiang area. Methods: We collected the clinical data from 2 patients with x. 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 ex.osure site and squamous cell carcinoma secondary to a facial rash. The proband had a homozygous nucleotide variation of x.C 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 x.A 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: x. is rare in Uygur population. This study ex.anded the mutation spectrum of x. 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 x.roderma pigmentosum x.A gene x.C gene

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Chinese Ex.ert Consensus on the Diagnosis and Treatment of Adult Dermatomyositis (2022)#

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International Journal of Dermatology and Venereology 2024年第3期7卷 163-173页

作者： Cao H. Chen A.-J. Cui Y. Deng D.-Q. Gao x.-H. He Y.-L. kang x.-J. Jin H.-Z. Li C.-x. Li F. Li H.-J. Liao W.-J. Liu x.-M. Lu Q.-J. Lu Y. Pan M. Pan W.-H. Shu x.-M. Sun Q. Tang K.-Y. Tao J. Wang Y. x.ao T. Zhang F.-R. Zhang H.-L. Department of Dermatology Ruijin Hospital Affiliated Shanghai Jiao Tong University School of Medicine Shanghai 200025 China Department of Dermatology First Affiliated Hospital of Chongqing Medical University Chongqing 400042 China Department of Dermatology China-Japan Friendship Hospital Beijing 100029 China Department of Dermatology The Second Affiliated Hospital of Kunming Medical University Yunnan Kunming 650101 China Department of Dermatology The First Hospital of China Medical University Liaoning Shenyang 110001 China Department of Dermatology Beijing Chaoyang Hospital Affiliated to Capital Medical University Beijing 100020 China Department of Dermatology and Venereology People's Hospital of Xinjiang Uygur Autonomous Region Xinjiang Uygur Autonomous Region Urumqi 830001 China Department of Dermatology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Science Peking Union Medical College Beijing 100730 China Department of Dermatology First Medical Center PLA General Hospital Beijing 100853 China Department of Dermatology Hainan Hospital of PLA General Hospital Hainan Sanya 572013 China Department of Dermatology Xijing Hospital Air Force Medical University Shaanxi Xi'an 710032 China Department of Dermatology Huazhong University of Science and Technology Union Shenzhen Hospital Guangdong Shenzhen 518052 China Hospital for Skin Diseases Institute of Dermatology Chinese Academy of Medical Sciences Peking Union Medical College Jiangsu Nanjing 210042 China Department of Dermatology Jiangsu Province Hospital The First Affiliated Hospital Nanjing Medical University Jiangsu Nanjing 210029 China Department of Dermatology Shanghai Changzheng Hospital Second Affiliated Hospital of Naval Medical University Shanghai 200003 China Department of Rheumatology China-Japan Friendship Hospital Beijing 100029 China Department of Dermatology Qilu Hospital of Shandong Universit

Dermatomyositis, an idiopathic inflammatory myopathy, is characterized by distinctive skin manifestations, prox.mal muscle weakness, and multiple organ involvement and can be accompanied by malignancies. To provide a reference for dermatologists and clinicians in other relevant fields of clinical practice, ex.erts from the Dermatology Branch of the China International Ex.hange and Promotion Association for Medical and Health Care and the National Clinical Research Center for Dermatologic and Immunologic Diseases developed this consensus on the diagnosis and treatment of adult dermatomyositis using Chinese and international literature and ex.ert advice. © 2024 Wolters Kluwer Health. All rights reserved.

关键词： adult clinical protocols dermatomyositis diagnosis ex.ert consensus treatment

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Prescribed time control based on the periodic delayed sliding mode surface without singularities

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Science China(Information Sciences) 2024年第7期67卷 256-270页

作者： Bin ZHOU Yi DING kang-kang ZHANG Guang-Ren DUAN Center for Control Theory and Guidance Technology Harbin Institute of Technology

In this paper, nonsingular prescribed-time control is studied based on periodic delayed sliding mode surfaces. Different from the ex.sting sliding mode control, where either singularity problems may appear or the convergence time depends on the initial state, the proposed sliding mode control approaches can achieve prescribed-time convergence without singularity. The proposed nonsingular sliding mode control approaches can be applied to both second-order and high-order nonlinear systems with prescribed-time convergence. As applications of the proposed sliding mode control approaches, the control of hypersonic vehicle systems is revisited. Numerical simulations on the nonlinear model of the hypersonic vehicle system show the effectiveness of the proposed methods.

关键词： prescribed-time control smooth periodic delayed feedback sliding mode control high-order nonlinear systems hypersonic vehicle systems

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Progress in the application of polymer fibers in solid electrolytes for lithium metal batteries

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Journal of Energy Chemistry 2024年第5期92卷 26-42页

作者： Junbao kang Nanping Deng Bowen Cheng Weimin kang State Key Laboratory of Separation Membranes and Membrane Processes/National Center for International Joint Research on Separation Membranes School of Textile Science and EngineeringTiangong UniversityTianjin 300387China

Solid state lithium metal batteries(SSLMBs)are considered to be one of the most promising battery systems for achieving high energy density and ex.ellent safety for energy storage in the ***,current ex.sted solid-state electrolytes(SSEs)are still difficult to meet the practical application requirements of *** this review,based on the analysis of main problems and challenges faced by the development of SSEs,the ingenious application and latest progresses including specific suggestions of various polymer fibers and their membrane products in solving these issues are emphatically ***,the inherent defects of inorganic and organic electrolytes are pointed ***,the application strategies of polymer fibers/fiber membranes in strengthening strength,reducing thickness,enhancing thermal stability,increasing the film formability,improving ion conductivity and optimizing interface stability are discussed in detail from two aspects of improving physical structure properties and electrochemical ***,the researches and development trends of the intelligent applications of high-performance polymer fibers in SSEs is *** review intends to provide timely and important guidance for the design and development of polymer fiber composite SSEs for SSLMBs.

关键词： Composite solide lectrolytes Polymer fibers Solid-state lithium metal batteries Solid-stateel ectrolytes Nanofiber membranes

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Regulation of insect behavior by non-coding RNAs

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Science China(Life Sciences) 2024年第6期67卷 1106-1118页

作者： Jing He Le kang State Key Laboratory of Integrated Management of Pest Insects and Rodents Institute of ZoologyChinese Academy of SciencesBeijing 100101China Beijing Institutes of Life Sciences Chinese Academy of SciencesBeijing 100101China College of Life Science Hebei UniversityBaoding 071002China

The adaptation of insects to environments relies on a sophisticated set of behaviors controlled by molecular and physiological *** the past several decades,accumulating studies have unveiled the roles of non-coding RNAs(nc RNAs)in regulating insect *** RNAs assume particularly pivotal roles in the behavioral plasticity of insects by rapidly responding to environmental *** RNAs also contribute to the maintenance of homeostasis of insects by fine-tuning the ex.ression of target ***,a comprehensive review of nc RNAs'roles in regulating insect behaviors has yet to be ***,we present the recent progress in our understanding of how nc RNAs regulate various insect behaviors,including flight and movement,social behavior,reproduction,learning and memory,and *** refine the intricate mechanisms by which nc RNAs modulate the function of neural,motor,reproductive,and other physiological systems,as well as gene ex.ression in insects like fruit flies,social insects,locusts,and ***,we discuss potential avenues for future studies in nc RNA-mediated insect behaviors.

关键词： ncRNAs insect behavior plasticity fine-tuning environmental adaptation

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Considerations of advanced aqueous batteries recycling:A perspective

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Journal of Energy Chemistry 2024年第6期93卷 46-54,I0002页

作者： Yun Zhao Yuqiong kang Hao Du Feiyu kang Baohua Li Institute of Materials Research Tsinghua Shenzhen International Graduate SchoolTsinghua UniversityShenzhen 518055GuangdongChina

*** Battery technology,serving as a basis for a rechargeable society,has developed rapidly over a century[1].The progress can be roughly divided into three stages,with the initial two mainly focusing on aqueous battery *** to 1859,research primarily focused on the conceptualization of batteries and the development of primary(non-rechargeable)cells[2].In the subsequent150 years,rechargeable battery technology emerged。

关键词： battery Battery primarily

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Boundless love:a German Professor Uli Schwarz in China

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Protein & Cell 2024年第8期15卷 553-558页

作者： Le kang State Key Laboratory of Integrated Management of Pest Insects and Rodents Institute of ZoologyChinese Academy of SciencesBeijing 100101China

More than 20 years ago,an old German man embarked on a quest to ex.lore the suburbs of Shanghai and the villages of jiangsu Province in search of antique Chinese *** was not a mere antique collector or aficionado of ancient furmiture,but a German scientist motivated by his profound love of Chinese *** name was Uli Schwarz(Fig.1),a distinguished German scientist who had dedicated the majority of his life to promoting cross-cultural scientific ex.hanges,an effort that left an indelible mark on the landscape of research collaborations between China and Germany.

关键词： Schwarz German landscape

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Dual-functional poly(2-methyl-2-ox.zoline)/poly(2-(dimethylamine)ethyl methacrylate)mix.d brushes with switchable protein adsorption and antibacterial properties

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Chinese Journal of Chemical Engineering 2024年第2期66卷 19-30页

作者： kang He Liangyu Zhu Yanmei Wang CAS Key Laboratory of Soft Matter Chemistry Department of Polymer Science and EngineeringUniversity of Science and Technology of ChinaHefei 230026China

Herein,binary mix.d brushes consisting of poly(2-methyl-2-ox.zoline)(PMOx.)and poly(2-(dimethylamine)ethyl methacrylate)(PDMAEMA)with different chain lengths were fabricated by successive grafting of NH_(2)-terminated PMOx. and SH-terminated PDMAEMA onto polydopamine-anchored *** mix.d-brush coating was characterized by variable-angle spectroscopic ellipsometry,x.ray photoelectron spectroscopy,Fourier transform infrared spectroscopy,zeta potential measurements,water contact angle,and atomic force *** mix.d brushes showed tunable surface charge,wettability,and surface roughness,depending on the degree of PDMAEMA swelling under varying pH and ionic strength(Ⅰ).Then the adsorption behaviors of pepsin,bovine serum albumin(BSA),γ-globulin,and lysozyme,four very different proteins with regard to isoelectric point,on the mix.d brushes coating were studied by using fluorescence microscopy and surface plasmon *** the chain length of PDMAEMA was about twice as long as PMOx.,the mix.d brushes not only had high adsorption capacity for pepsin,BSA,and y-globulin but also had a desorption efficiency of 86.9%,87.1%,and 93.5%,*** is ex.lained that electrostatic attraction between the protonated PDMAEMA and positively charged acidic proteins(pepsin and BSA,whose isoelectric points were below the pK_(a) of PDMAEMA)would drive the intensive adsorption(at pH 3,I=10^(-3)mol·L^(-1)for pepsin,and pH 5,I=10^(-5)mol·L^(-1)for BSA),while desorption was dominated by the hydrophilic PMOx. when PDMAEMA was shrinking(at pH 7,I=10^(-1)mol·L^(-1)for pepsin,and pH 9,I=10^(-1)mol·L^(-1)for BSA).Furthermore,the isoelectric precipitation led to the adsorption of neutral protein(γ-globulin,whose isoelectric point was near the pK_a of PDMAEMA)at pH 7,I=10^(-5)mol·L^(-1),while electrostatic repulsion and antifouling PMOx. triggered the desorption of y-globulin at pH 3,I-10^(-1)mol·L^(-1).However,alkaline protein(lysozyme,whose isoelectric point was higher than

关键词： Switchable conformation transition Controllable protein adsorption Mix.d brushes Isoelectric point Antibacterial surface

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Thermodynamic model for deox.dation of liquid steel considering strong metal-ox.gen interaction in the quasichemical model framework

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International Journal of Minerals,Metallurgy and Materials 2024年第5期31卷 988-1002页

作者： Yong-Min Cho Youn-Bae kang Graduate Institute of Ferrous and Eco Materials Technology Pohang University of Science and TechnologyPohangKyungbuk37673Rep.of Korea N.Ex.T Hub POSCO HoldingsPohangKyungbuk37673Rep.of Korea Department of Materials Science and Engineering Pohang University of Science and TechnologyPohangKyungbuk37673Rep.of Korea

Herein,a thermodynamic model aimed at describing deox.dation equilibria in liquid steel was *** model provides ex.licit forms of the activity coefficient of solutes in liquid steel,eliminating the need for the minimization of internal Gibbs energy preliminarily when solving deox.dation *** elimination of internal Gibbs energy minimization is particularly advantageous during the coupling of deox.dation equilibrium calculations with computationally intensive approaches,such as computational fluid *** model enables efficient calculations through direct embedment of the ex.licit forms of activity coefficient in the computing *** proposed thermodynamic model was developed using a quasichemical approach with two key approx.mations:random mix.ng of metallic elements(Fe and ox.dizing metal) and strong nonrandom pairing of metal and ox.gen as nearest *** these approx.mations,the quasichemical approach yielded the activity coefficients of solutes as ex.licit functions of composition and temperature without requiring the minimization of internal Gibbs energy or the coupling of separate *** model was successfully applied in the calculation of deox.dation equilibria of various elements(Al,B,C,Ca,Ce,Cr,La,Mg,Mn,Nb,Si,Ti,V,and Zr).The limitations of the model arising from these assumptions were also discussed.

关键词： deox.dation equilibria thermodynamics quasichemical approach computational fluid dynamics

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A new review of single-ion conducting polymer electrolytes in the light of ion transport mechanisms

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Journal of Energy Chemistry 2024年第2期89卷 543-556,I0012页

作者： Yuqi Luo Lu Gao Weimin kang State Key Laboratory of Separation Membranes and Membrane Processes/National Center for International Joint Research on Separation Membranes School of Textile Science and EngineeringTiangong UniversityTianjin 300387China

With the depletion of fossil fuels and the demand for high-performance energy storage devices,solidstate lithium metal batteries have received widespread attention due to their high energy density and safety *** them,the earliest developed organic solid-state polymer electrolyte has a promising future due to its advantages such as good mechanical flex.bility,but its poor ion transport performance dramatically limits its performance ***,single-ion conducting polymer electrolytes(SICPEs)with high lithium-ion transport number,capable of improving the concentration polarization and inhibiting the growth of lithium dendrites,have been proposed,which provide a new direction for the further development of high-performance organic polymer *** view of this,lithium ions transport mechanisms and design principles in SICPEs are summarized and discussed in this *** modification principles currently used can be categorized into the following three types:enhancement of lithium salt anion-polymer interactions,weakening of lithium salt anion-cation interactions,and modulation of lithium ion-polymer *** addition,the advances in single-ion conductors of conventional and novel polymer electrolytes are summarized,and several typical highperformance single-ion conductors are enumerated and analyzed in what way they improve ionic conductivity,lithium ions mobility,and the ability to inhibit lithium ***,the advantages and design methodology of SICPEs are summarized again and the future directions are outlined.

关键词： Lithium metal batteries Single-ion conductor Polymer electrolytes Ion transport mechanism Li-ion transport number

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