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A novel AMH variant at the prehelix loop impairs the binding to AMHR2 and causes persistent Mullerian duct syndrome

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Asian Journal of Andrology 2024年第2期26卷 222-224页

作者： qiang du Chuang Qiu Yuan-Yuan Zhang Xue-Jun Shang Xiao-Liang Liu Center of Reproductive Medicine Shengjing Hospital of China Medical UniversityShenyang 110004China Department of Orthopedics Shengjing Hospital of China Medical UniversityShenyang 110004China Department of Clinical Genetics Shengjing Hospital of China Medical UniversityShenyang 110004China Department of Urology Jinling HospitalSchool of MedicineNanjing UniversityNanjing 210012China

Dear Editor,Persistent Mullerian duct syndrome(PMDS;Online Mendelian Inheritance in Man[OMIM]:#261550)is a rare autosomal recessive inherent difference in sex development in males with Mullerian duct derivatives,including the uterus,fallopian tubes,and upper part of the vagina.'Approximately 88%of PMDS patients were found to have biallelic variants in the anti-Mullerian hormone(AMH;OMIM:*600957)or AMH type 2 receptor(AMHR2;OMIM:*600956)genes,and the rest were referred as idiopathic.I The serum AMH levels are usually very low or undetectable in PMDS patients with AMH gene mutations but normal or elevated in those with AMHR2 gene mutations.Here,an adult Chinese patient with PMDS carrying biallelic variants of the AMH gene was reported to have unexpectedly normal serum AMH level.Please refer to Supplementary Information of Patient and Methods for methodologies.

关键词： elevated AMH Muller

Current understanding of ELF4 deficiency:a novel inborn error of immunity

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World Journal of Pediatrics 2024年第5期20卷 444-450页

作者： Hong-qiang du Xiao-Dong Zhao Department of Rheumatology&Immunology National Clinical Research Center for Child Health and DisordersMinistry of Education Key Laboratory of Child Development and DisordersChongqing Key Laboratory of Child Rare Diseases in Infection and ImmunityChildren’s Hospital of Chongqing Medical UniversityZhongshan Er Road 136Yuzhong DistrictChongqingChina

Background ELF4 deficiency has been recently recognized as a novel disorder within the spectrum of inborn errors of immunity(IEIs),specifically categorized as a“disease of immune dysregulation.”Cases of this condition,reported by our team and others,are very limited worldwide.As such,our current knowledge of this new disease remains preliminary.This review aims to provide a brief overview of the clinical manifestations,pathogenesis,and treatment strategies for this novel IEI.Data sources A comprehensive review was conducted after an extensive literature search in the PubMed/Medline database and websites concerning transcriptional factor ELF4 and reports concerning patients with ELF4 deficiency.Our search strategy was“ELF4 OR ETS-related transcription factor Elf-4 OR EL4-like factor 4 OR myeloid Elf-1-like factor”as of the time of manuscript submission.Results The current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric males.In some cases,immunodeficiency and autoimmunity can also be prominent.Targeted Sanger sequencing or whole exome sequencing can be used to detect variation in ELF4 gene.Western blotting for ELF4 expression of the patient’s cells can confirm the pathogenic effect of the variant.To fully confirm the pathogenicity of the variant,further functional test is strongly advised.Glucocorticoid and biologics are the mainstream management of ELF4 deficiency disorder.Conclusions Pediatric males presenting with recurring ulcerations in digestive tract epithelium with or without recurrent fever should be suspected of DEX.When atypical presentations are prominent,variations in ELF4 gene should be carefully evaluated functionally due to the complex nature of ELF4 function.Experience of treating DEX includes use of glucocorticoid and biologics and more precise treatment needs more patients to identify and further mechanistic study.

关键词： ELF4 transcription factor Immune dysregulation Inborn errors of immunity Mechanism Recurrent infections

Influence of Vanes and Blades on Ingress in Axial Rim Seal for Turbine Stages

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Journal of Thermal Science 2024年第3期33卷 833-846页

作者： REN Ran du qiang LIU Guang ZENG Yanlian XIE Lei Institute of Engineering Thermophysics Chinese Academy of SciencesBeijing100190China National Key Laboratory of Science and Technology on Advanced Light-duty Gas-turbine Beijing100190China School of Aeronautics and Astronautics University of Chinese Academy of SciencesBeijing100190China

Hot gas ingestion refers to the phenomenon of mainstream hot gas flowing into the space cavity of a turbine wheel.Previous studies have found that mainstream annulus pressure distribution plays an important role in hot gas ingestion,but due to its complexity,the mechanism of the interaction between mainstream flow and hot gas ingestion remains unclear.This paper adopts the URANS method,and three sealing flow rates are considered,named C_(w)=0,C_(w)=500,and C_(w)=5000.The time-averaged annulus pressure distribution shows that an increase in the sealing flow decreases the pressure value,and the effects of the sealing flow on the pressure distribution of the leading edge of the blade are much more influential than that of the trailing edge of the vane.The unsteady pressure time-space distribution in the annulus indicates that a time-space tilted distribution of pressure at the rim exits when the sealing flow exists.This phenomenon is mainly due to the strong feedback mechanism of the sealing flow to the annulus pressure field.A comparison of the pressure and mean radial velocity distribution of the mainstream shows that the ingestion mainly occurs on the blade side,where the pressure is lower than on the vane side.The flow characteristics at the wheel rim are analyzed with a sealing flow rate C_(w)=5000,and under these conditions,both pressure-induced ingestion and ingestion caused by a passage vortex can be inferred.The three-dimensional and inertial effects of the mainstream at the wheel rim lead to the generation of separation vortices on the blade side,and the presence of separation vortices leads to ingestion along the blade side.At the same time,pressure on the blade side will cause the fluid to have a radial inward flow tendency,which will promote the formation of separation vortices,leading to more serious ingestion in the high-pressure region on the blade side.The blade pressure field can be more significant than the vane trailing pressure field in the rim seal ingestion,and it contributes some explanations

关键词： separation vortex unsteady rim seal the effect of blade on ingestion

Efficacy of interleukin-17 inhibitors on radiographic progression in psoriatic arthritis:A systematic review and meta-analysis

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Chinese Medical Journal 2024年第4期137卷 473-475页

作者： Han-Lei Jiang Yu du qiang Tong Sheng-Ming Dai Department of Rheumatology and Immunology Shanghai Sixth People’s HospitalShanghai Jiao Tong University School of MedicineShanghai 200233China

To the Editor:Psoriatic arthritis(PsA)is a highly heterogeneous inflammatory arthritis.It occurs in up to 30%of patients with psoriasis and can have severe impacts on peripheral joints,spine,tendon insertions,and fingers. ^([1])Radiographic progression is currently the preferred method for assessing structural damage of PsA. ^([2])Biologic treatment options for PsA include tumor necrosis factor-αinhibitors(TNFi),interleukin-12/23 inhibitors,and interleukin-17(IL-17)inhibitors.The therapeutic effects of IL-17 inhibitors on structural damage were not consistent over multiple trials.Therefore,a systematic review and meta-analysis of the available randomized controlled trials(RCTs)was conducted to quantify the overall effect of IL-17 inhibitors on structural damage in PsA.This study was registered with PROSPERO(No.CRD42022301774).

关键词： damage analysis inhibitors

A Nonlocal Stokes Systemwith Volume Constraints

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Numerical Mathematics(Theory,Methods and Applications) 2022年第4期15卷 903-937页

作者： qiang du Zuoqiang Shi Department of Applied Physics and Applied Mathematics and Data Science InstituteColumbia UniversityNew YorkNY 10027USA Yau Mathematical Sciences Center Tsinghua UniversityBeijing 100084China Yanqi Lake Beijing Institute of Mathematical Sciences and Applications Beijing 101408China

In this paper,we introduce a nonlocal model for linear steady Stokes system with physical no-slip boundary condition.We use the idea of volume constraint to enforce the no-slip boundary condition and prove that the nonlocal model is wellposed.We also show that and the solution of the nonlocal system converges to the solution of the original Stokes system as the nonlocality vanishes.

关键词： Nonlocal Stokes system nonlocal operators smoothed particle hydrodynamics incompressible flows well-posedness local limit

Dynamic regulation of the irrigation-nitrogen-biochar nexus for the synergy of yield,quality,carbon emission and resource use efficiency in tomato

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Journal of Integrative Agriculture 2024年第2期23卷 680-697页

作者： Ping’an Zhang Mo Li qiang Fu Vijay PSingh Changzheng du Dong Liu Tianxiao Li Aizheng Yang School of Water Conservancy&Civil Engineering Northeast Agricultural UniversityHarbin 150030China Key Laboratory of Effective Utilization of Agricultural Water Resources Ministry of Agriculture and Rural Affairs/Northeast Agricultural UniversityHarbin 150030China Department of Biological and Agricultural Engineering&Zachry Department of Civil&Environmental Engineering Texas A&M UniversityTX 77843-117USA National Water and Energy Center UAE UniversityAI Ain999041United Arab Emirates Heilongjiang Agricultural Engineering Vocational College Harbin 150030China

Integrated water and fertilizer management is important for promoting sustainable development of facility agriculture,and biochar plays an important role in guaranteeing food production,as well as alleviating water shortages and the overuse of fertilizers.The field experiment had twelve treatments and a control(CK)trial including two irrigation amounts(I1,100%ETm;I2,60%ETm;where ETm is the maximum evapotranspiration),two nitrogen applications(N1,360 kg ha^(−1);N2,120 kg ha^(−1))and three biochar application levels(B1,60 t ha^(−1);B_(2),30 t ha^(−1)and B3,0 t ha^(−1)).A multi-objective synergistic irrigation-nitrogen-biochar application system for improving tomato yield,quality,water and nitrogen use efficiency,and greenhouse emissions was developed by integrating the techniques of experimentation and optimization.First,a coupled irrigation-nitrogen-biochar plot experiment was arranged.Then,tomato yield and fruit quality parameters were determined experimentally to establish the response relationships between irrigation-nitrogen-biochar dosage and yield,comprehensive quality of tomatoes(TCQ),irrigation water use efficiency(IWUE),partial factor productivity of nitrogen(PFPN),and net greenhouse gas emissions(NGE).Finally,a multi-objective dynamic optimization regulation model of irrigation-nitrogen-biochar resource allocation at different growth stages of tomato was constructed which was solved by the fuzzy programming method.The results showed that the application of irrigation and nitrogen to biochar promoted increase in yield,IWUE and PFPN,while it had an inhibitory effect on NGE.In addition,the optimal allocation amounts of water and fertilizer were different under different scenarios.The yield of the S1 scenario increased by 8.31%compared to the B_(1)I_(1)N_(2) treatment;TCQ of the S2 scenario increased by 5.14%compared to the B_(2)I_(2)N_(1) treatment;IWUE of the S3 scenario increased by 10.01%compared to the B1I2N2 treatment;PFPN of the S4 scenario increased by 9.35%compared to the B_(1)I_(1)N_(2) treatment;a

关键词： irrigation-nitrogen-biochar dynamic regulation multi-dimensional target collaboration tomato sustainability

Numerical Analysis of PRISM-PY Calculations for Hard-and Soft-Core Generic Polymer Models

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Chinese Journal of Polymer Science 2024年第4期42卷 532-543页

作者： qiang Wang Department of Chemical and Biological Engineering Colorado State University1370 Campus DeliveryFort CollinsCO80523-1370USA

Generic polymer models capturing the chain connectivity and excluded-volume interactions between polymer segments can be classified, according to whether or not the 3D integral of the latter diverges, into hard- and soft-core models. Taking homogeneous systems of compressible homopolymer melts (or equivalently homopolymer solutions in an implicit, good solvent) in the continuum as an example, we recently compared the correlation effects on the structural and thermodynamic properties of the hard- and soft-core models given by the polymer reference interaction site model (PRISM) theory with the Percus-Yevick (PY) closure (Polymers 2023, 15, 1180). Here we analyzed in detail the numerical errors and behavior of the interchain pair correlation functions (PCFs) given by the PRISM-PY calculations of these models using an efficient numerical approach that we proposed. Our numerical approach has the least number of independent variables to be iteratively solved, analytically treats the discontinuities caused by the non-bonded pair potential (such as that of the hard spheres) and takes only the inverse Fourier transform of the interchain indirect PCF between polymer segments (which is continuous and decays towards 0 with increasing wavenumber much faster than both the interchain direct and total PCFs), and is essential for us to accurately solve the PRISM-PY theory for chain length N as large as 106. To capture the correlation-hole effect, the real-space cut-off in the PRISM calculations should be proportional to the square root of N.

关键词： Generic polymer models Soft potentials Polymer reference interaction site model theory

Constructing electron-blocking grain boundaries in garnet to suppress lithium dendrite growth

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Journal of Advanced Ceramics 2024年第2期13卷 166-175页

作者： Xing Xiang Zecheng Fang Congkun du Zhenzhen Zhao Jiajia Chen Yanhua Zhang Huihu Wang Chenhuinan Wei Fei Chen qiang Shen Hubei Provincial Key Laboratory of Green Materials for Light Industry Hubei University of TechnologyWuhan 430068China Hubei Longzhong Laboratory Wuhan University of Technology Xiangyang Demonstration ZoneXiangyang 441000China Collaborative Innovation Center of Green Light-weight Materials and Processing Hubei University of TechnologyWuhan 430068China New Materials and Green Manufacturing Talent Introduction and Innovation Demonstration Base Hubei University of TechnologyWuhan 430068China State Key Lab of Advanced Technology for Materials Synthesis and Processing Wuhan University of TechnologyWuhan 430070China

Li_(7)La_(3)Zr_(2)O_(12)(LLZO)is considered as a promising solid-state electrolyte due to its high ionic conductivity,wide electrochemical window,and excellent electrochemical stability.However,its application in solid-state lithium metal batteries(SSLMBs)is impeded by the growth of lithium dendrites in LLZO due to some reasons such as its high electronic conductivity.In this study,lithium fluoride(LiF)was introduced into Ta-doped LLZO(LLZTO)to modify its grain boundaries to enhance the performance of SSLMBs.A nanoscale LiF layer was uniformly coated on the LLZTO grains,creating a threedimensional continuous electron-blocking network at the grain boundaries.Benefiting from the electronic insulator LiF and the special structure of the modified LLZTO,the symmetric cells based on LLZO achieved a high critical current density(CCD)of 1.1 mA·cm^(-2)(in capacity-constant mode)and maintained stability over 2000 h at 0.3 mA·cm^(-2).Moreover,the full cells combined with a LiFePO_(4)(LFP)cathode,demonstrated excellent cycling performance,retaining 97.1% of capacity retention after 500 cycles at 0.5 C.Therefore,this work provides a facile and effective approach for preparing a modified electrolyte suitable for high-performance SSLMBs.

关键词： solid electrolyte garnet lithium dendrite microstructure solid-state batteries

From Liquid to Solid‑State Lithium Metal Batteries:Fundamental Issues and Recent Developments

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Nano-Micro Letters 2024年第2期16卷 68-125页

作者： Zhao Zhang Wei‑qiang Han School of Materials Science and Engineering Zhejiang UniversityHangzhou 310027People’s Republic of China

The widespread adoption of lithium-ion batteries has been driven by the proliferation of portable electronic devices and electric vehicles,which have increasingly stringent energy density requirements.Lithium metal batteries(LMBs),with their ultralow reduction potential and high theoretical capacity,are widely regarded as the most promising technical pathway for achieving high energy density batteries.In this review,we provide a comprehensive overview of fundamental issues related to high reactivity and migrated interfaces in LMBs.Furthermore,we propose improved strategies involving interface engineering,3D current collector design,electrolyte optimization,separator modification,application of alloyed anodes,and external field regulation to address these challenges.The utilization of solid-state electrolytes can significantly enhance the safety of LMBs and represents the only viable approach for advancing them.This review also encompasses the variation in fundamental issues and design strategies for the transition from liquid to solid electrolytes.Particularly noteworthy is that the introduction of SSEs will exacerbate differences in electrochemical and mechanical properties at the interface,leading to increased interface inhomogeneity—a critical factor contributing to failure in all-solidstate lithium metal batteries.Based on recent research works,this perspective highlights the current status of research on developing high-performance LMBs.

关键词： Lithium metal batteries All-solid-state lithium metal battery Li dendrite Solid electrolyte Interface