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Clinical and molecular characterization of 10 Chinese children with congenital adrenal hyperplasia due to 11beta‑hydroxylase deficiency

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World Journal of Pediatrics 2024年第4期20卷 422-433页

作者： Wen‑Li Lu Xiao‑Yu Ma jiao Zhang Jun‑Qi Wang Ting‑Ting Zhang Lei Ye Yuan Xiao Zhi‑Ya Dong Wei Wang Shou‑yue Sun Chuan‑Yin Li Rong‑Gui hu Guang Ning Li‑Dan Zhang Department of Pediatrics Ruijin HospitalShanghai Jiao Tong University School of MedicineNo.197Ruijin 2nd RdHuangpu DistrictShanghai 200025China Department of Pediatric Genetic and Metabolic Endocrinology West China Second University HospitalSichuan UniversityNo.20Section 3Renmin South RoadSichuan 610041China Department of Endocrine and Metabolic Diseases Shanghai Clinical Center for Endocrine and Metabolic DiseasesRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Cancer Center School of MedicineShanghai Tenth People’s HospitalTongji UniversityYanchang Zhong Lu 301St RdJing’an DistrictShanghai 200031China

Background The clinical manifestations of nonclassical 11beta-hydroxylase deficiency are very similar to those of nonclassical 21-hydroxylase *** this study,we investigated the relationship between the clinical and molecular features of congenital adrenal hyperplasia caused by 11beta-hydroxylase deficiency and reviewed the related literature,which are expected to provide assistance for the clinical diagnosis and analysis of congenital adrenal *** Clinical data for 10 Chinese patients diagnosed with congenital adrenal hyperplasia in our hospital from 2018 to 2022 were retrospectively *** examined the effects of gene mutations on protease activity and constructed threedimensional structure prediction models of *** We describe 10 patients with 11beta-hydroxylase gene mutations(n=5,46,XY;n=5,46,XX),with 10 novel mutations were *** patients received treatment at an early stage,with an average age of 2.08±1.66 years,whereas male patients received treatment significantly later,at an average age of 9.77±3.62 *** most common CYP11B1 pathogenic variant in the Chinese population was found to be c.1360C>*** mutations lead to spatial conformational changes that affect protein *** Our study found that there was no significant correlation between each specific mutation and the severity of clinical *** patients with the same gene pathogenic variant may have mild or severe clinical *** correlation between genotype and phenotype needs further ***-dimensional protein simulations may provide additional support for the physiopathological mechanism of genetic mutations.

关键词： 11β-hydroxylase deficiency CYP11B1 Enzymatic activity Phenotype-genotype correlation Threedimensional protein simulations

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An orchestrated ethylene-gibberellin signaling cascade contributes to mesocotyl elongation and emergence of rice direct seeding

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Journal of Integrative Plant Biology 2024年第7期66卷 1427-1439页

作者： Yusong Lyu Xinli Dong Shipeng Niu Ruijie Cao Gaoneng Shao Zhonghua Sheng Guiai jiao Lihong Xie Shikai hu Shaoqing Tang Xiangjin Wei Peisong hu State Key Laboratory of Rice Biology and Breeding China National Center for Rice ImprovementChina National Rice Research InstituteHangzhou 310006China College of Agriculture and Biotechnology Zhejiang UniversityHangzhou 310058China

A mechanized direct seeding of rice with less labor and water usage,has been widely ***,this approach requires varieties that exhibit uniform seedling *** elongation(ME)offers the main drive of fast emergence of rice seedlings from soils;nevertheless,its genetic basis remains ***,we identify a major rice quantitative trait locus Mesocotyl Elongation1(qME1),an allele of the Green Revolution gene Semi-Dwarf1(SD1),encoding GA20-oxidase for gibberellin(GA)***1 expression is strongly induced by soil depth and *** rice grains are direct-seeded in soils,the ethylene core signaling factor OsEIL1 directly promotes ME1 transcription,accelerating bioactive GA *** GAs further degrade the DELLA protein SLENDER RICE 1(SLR1),alleviating its inhibition of rice PHYTOCHROME-INTERACTING FACTOR-LIKE13(OsPIL13)to activate the downstream expansion gene OsEXPA4 and ultimately promote rice seedling ME and *** ancient traits of long mesocotyl and strong emergence ability in wild rice and landrace were gradually lost in company with the Green Revolution dwarf breeding process,and an elite ME1-R allele(D349H)is found in some modern Geng varieties(long mesocotyl lengths)in northern China,which can be used in the direct seeding and dwarf breeding of Geng ***,the ectopic and high expression of ME1 driven by mesocotyl-specific promoters resulted in rice plants that could be direct-seeded without obvious plant architecture or yield ***,we reveal the molecular mechanism of rice ME,and provide useful information for breeding new Green Revolution varieties with long mesocotyl suitable for direct-seeding practice.

关键词： rice(Oryza sativa L.) direct seeding Green Revolu-tion mesocotyl elongation ethylene gibberellin semi-dwarf1(SD1)

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One-step controlled electrodeposition nickel sulfides heterointerfaces favoring the desorption of hydroxyl groups for efficient hydrogen generation

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Rare Metals 2024年第9期43卷 4377-4386页

作者： Ru-Chun Li Xin-yue Zhang Ze-yue Qu Feng-Yi Liu Quan-Qing Xu Zhao-Xia hu Jing-Wei Li Mohamed-Nawfal Ghazzal Jin-Li Yu Faculty of Chemistry and Chemical Engineering Yunnan Normal UniversityKunming 650500China Foshan Gaoke Hechuang Technology Co. Ltd.Foshan 528200China School of Chemistry and Chemical Engineering Guangzhou UniversityGuangzhou 510006China Universite Paris-Saclay UMR 8000 CNRSInstitut de Chimie PhysiqueOrsay 91405France Department of Chemistry City University of Hong KongHong Kong 999077China

The heterointerface engineering involving different components or phases represents a desirable strategy for enhancing the sluggish kinetics of hydrogen evolution reaction(HER).However,constructing desired heterointerfaces and elucidating the reaction mechanisms on the interface remains a considerable *** this work,we propose a straightforward electrochemical synthesis strategy to prepare the nickel sulfide-based heterointerfaces for *** mechanism of electrochemical synthesis is revealed,wherein metal-thiourea species can be formed at the cathode potential and subsequently oxidized to nickel sulfides at the anode *** this mechanism,a range of nickel sulfides,including NiS,Ni_(3)S_(2)/NiS,Ni/Ni_(3)S_(2)and Ni_(3)S_(2),have been successfully synthesized by tuning the potential range of cyclic *** these,the obtained Ni_(3)S_(2)/NiS@CC(CC:carbon cloth)exhibits the smallest overpotential of84 mV at 10 mA·cm^(-2)and high *** calculations further reveal that the combination of NiS and Ni_(3)S_(2)induces electron redistribution at the interface,and thus the Volmer process is effectively promoted with faster water dissociation and OH desorption ***,the simplicity method coupled with a clear synthesis mechanism and outstanding HER performance highlights its promising potential for practical applications.

关键词： Hydrogen evolution reaction Heterointerfaces OH desorption kinetics Electrochemical synthesis

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Efficient wide-bandgap perovskite solar cells with open-circuit voltage deficit below 0.4 V via hole-selective interface engineering

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Science China Chemistry 2024年第6期67卷 2102-2110页

作者： Xiaoyu Ji Shuo Zhang Furong Yu huidong Zhang Liqing Zhan yue hu Wei-Hong Zhu Yongzhen Wu Key Laboratory for Advanced Materials and Joint International Research Laboratory of Precision Chemistry and Molecular Engineering Shanghai Key Laboratory of Functional Materials ChemistryFrontiers Science Center for Materiobiology and Dynamic ChemistryInstitute of Fine ChemicalsSchool of Chemistry and Molecular EngineeringEast China University of Science and TechnologyShanghai200237China School of Chemistry University of EdinburghKing’s BuildingsEdinburghEH93FJUK

Wide-bandgap mixed-halide perovskite solar cells(WBG-PSCs)are promising top cells for efficient tandem photovoltaics to achieve high power conversion efficiency(PCE)at low ***,the open-circuit voltage(V_(OC))of WBG-PSCs is still unsatisfactory as the V_(OC)-deficit is generally larger than 0.45 ***,we report a buried interface engineering strategy that substantially improves the V_(OC)of WBG-PSCs by inserting amphiphilic molecular hole-selective materials featuring with a cyanovinyl phosphonic acid(CPA)anchoring group between the perovskite and *** assembly and redistribution of CPA-based amphiphilic molecules at the perovskite-substrate buried interface not only promotes the growth of a low-defect crystalline perovskite thin film,but also suppresses the photo-induced halide phase *** energy level alignment between wide-bandgap perovskite and the hole-selective layer is further improved by modulating the substituents on the triphenylamine donor moiety(methoxyls for MPA-CPA,methyls for Me PA-CPA,and bare TPA-CPA).Using a 1.68 e V bandgap perovskite,the Me PA-CPA-based devices achieved an unprecedentedly high V_(OC)of 1.29 V and PCE of 22.3%under standard AM 1.5 *** V_(OC)-deficit(<0.40 V)is the lowest value reported for *** work not only provides an effective approach to decreasing the V_(OC)-deficit of WBG-PSCs,but also confirms the importance of energy level alignment at the charge-selective layers in PSCs.

关键词： cyanovinyl phosphonic acid energy level alignment hole-selective material open-circuit voltage wide-bandgap perovskite

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Allosterically activating SHP2 by oleanolic acid inhibits STAT3-Th17 axis for ameliorating colitis

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Acta Pharmaceutica Sinica B 2024年第6期14卷 2598-2612页

作者： Jinbo hu Wen Liu Yi Zou Chenyang jiao Jiazhen Zhu Qiang Xu Jianjun Zou Yang Sun Wenjie Guo State Key Laboratory of Pharmaceutical Biotechnology Nanjing Drum Tower HospitalSchool of Life SciencesNanjing UniversityNanjing 210093China Department of Clinical Pharmacology Nanjing First HospitalNanjing Medical UniversityNanjing 210006China Jiangsu Key Laboratory of New Drug Research and Clinical Pharmacy Xuzhou Medical UniversityXuzhou 221004China

Src homology 2 domain-containing tyrosine phosphatase 2(SHP2)is an essential tyrosine phosphatase that is pivotal in regulating various cellular signaling pathways such as cell growth,differentiation,and *** activation of SHP2 has been shown to have a therapeutic effect in colitis and Parkinson's ***,the identification of SHP2 activators and a complete understanding of their mechanism is *** used a two-step screening assay to determine a novel allosteric activator of SHP2 that stabilizes it in an open *** acid was identified as a suitable *** binding to R362,K364,and K366 in the active center of the PTP domain,oleanolic acid maintained the active open state of SHP2,which facilitated the binding between SHP2 and its *** oleanolic acid-activated SHP2 hindered Th17 differentiation by disturbing the interaction between STAT3 and IL-6Rαand inhibiting the activation of ***,via the activation of SHP2 and subsequent attenuation of the STAT3-Th17 axis,oleanolic acid effectively mitigated colitis in *** protective effect was abrogated by SHP2 knockout or administration of the SHP2 inhibitor *** findings underscore the potential of oleanolic acid as a promising therapeutic agent for treating inflammatory bowel diseases.

关键词： Oleanolic acid SHP2 STAT3 Th17 Colitis Allosteric activator

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Simplified liver imaging reporting and data system for the diagnosis of hepatocellular carcinoma on gadoxetic acid-enhanced magnetic resonance imaging

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World Journal of Gastrointestinal Oncology 2024年第6期16卷 2439-2448页

作者： Rong Lyu Wei-Juan hu Di Wang jiao Wang Yu-Bing Ye Ke-Feng Jia Department of Radiology Tianjin Third Central HospitalTianjin Key Laboratory of Extracorporeal Life Support for Critical DiseasesArtificial Cell Engineering Technology Research CenterTianjin 300170China

BACKGROUND The liver imaging reporting and data system(LI-RADS)diagnostic table has 15 cells and is too *** diagnostic performance of LI-RADS for hepatocellular carcinoma(HCC)is not satisfactory on gadoxetic acid-enhanced magnetic resonance imaging(EOB-MRI).AIM To evaluate the ability of the simplified LI-RADS(sLI-RADS)to diagnose HCC on *** A total of 331 patients with 356 hepatic observations were retrospectively *** diagnostic performance of sLI-RADS A-D using a single threshold was evaluated and compared with LI-RADS v2018 to determine the optimal *** algorithms of sLI-RADS A-D are as follows:The single threshold for sLI-RADS A and B was 10 mm,that is,classified observations≥10mm using an algorithm of 10-19 mm observations(sLI-RADS A)and≥20 mm observations(sLI-RADS B)in the diagnosis table of LI-RADS v2018,respectively,while the classification algorithm remained unchanged for observations0.999).With HBP hypointensity as an MF,the sensitivity of F-sLI-RADS was greater than that of LI-RADS v2018(93.0%vs 87.0%,P<0.001)and sLI-RADS D(93.0%vs 89.8%,P=0.016),without a lower specificity(86.5%vs 90.1%,P=0.062;86.5%vs 89.4%,P=0.125).Compared with that of LI-RADS v2018,the time to classif

关键词： Hepatocellular carcinoma Magnetic resonance imaging Liver Diagnosis Contrast agent

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Artificial selection of the Green Revolution gene Semidwarf 1 is implicated in upland rice breeding

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Journal of Integrative Agriculture 2024年第3期23卷 769-780页

作者： Shuliang jiao Qinyan Li Fan Zhang Yonghong Tao Yingzhen Yu Fan Yao Qingmao Li Fengyi hu Liyu huang Key Laboratory of Biology and Germplasm Innovation of Perennial Rice Ministry of Agriculture and Rural Affairs/School of AgricultureYunnan UniversityKunming 650091China State Key Laboratory of Crop Gene Resources and Breeding Institute of Crop SciencesChinese Academy of Agricultural SciencesBeijing 100081China Wenshan Academy of Agricultural Sciences Wenshan 663000China

Semidwarf breeding has boosted crop production and is a well-known outcome from the first Green Revolution. The Green Revolution gene Semidwarf 1(SD1), which modulates gibberellic acid(GA) biosynthesis, plays a principal role in determining rice plant height. Mutations in SD1 reduce rice plant height and promote lodging resistance and fertilizer tolerance to increase grain production. The plant height mediated by SD1 also favors grain yield under certain conditions. However, it is not yet known whether the function of SD1 in upland rice promotes adaptation and grain production. In this study, the plant height and grain yield of irrigated and upland rice were comparatively analyzed under paddy and dryland conditions. In response to dryland environments, rice requires a reduction in plant height to cope with water deficits. Upland rice accessions had greater plant heights than their irrigated counterparts under both paddy and dryland conditions, and appropriately reducing plant height could improve adaptability to dryland environments and maintain high grain yield formation. Moreover, upland rice cultivars with thicker stem diameters had stronger lodging resistance, which addresses the lodging problem. Knockout of SD1 in the upland rice cultivar IRAT104 reduced the plant height and grain yield, demonstrating that the adjustment of plant height mediated by SD1 could increase grain production in dryland fields. In addition, an SD1 genetic diversity analysis verified that haplotype variation causes phenotypic variation in plant height. During the breeding history of rice, SD1 allelic mutations were selected from landraces to improve the grain yield of irrigated rice cultivars, and this selection was accompanied by a reduction in plant height. Thus, five known mutant alleles were analyzed to verify that functional SD1 is required for upland rice production. All these results suggest that SD1 might have undergone artificial positive selection in upland rice, which provides

关键词： Green Revolution Semidwarf 1 high-yield breeding upland rice plant height

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3D Printed Polyimide/Silica Composite Aerogels for Customizable Thermal Insulation from-50℃ to 1300℃

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Chinese Journal of Polymer Science 2024年第7期42卷 936-945,I0007页

作者： Dingyi Yu Tiantian Xue Zhuocheng Ma Zaiyin hu Lijuan Long yue-E Miao Wei Fan Tianxi Liu State Key Laboratory for Modification of Chemical Fibers and Polymer Materials College of Materials Science and EngineeringDonghua UniversityShanghai201620China Key Laboratory of Synthetic and Biological Colloids Ministry of EducationSchool of Chemical and Material EngineeringJiangnan UniversityWuxi214122China Guizhou Aerospace Wujiang Electro-Mechanical Equipment Co. Ltd.Zunyi563000China School of Materials and Architectural Engineering Guizhou Normal UniversityGuiyang550025China

Aerogels are widely used as thermal insulation materials because of their high porosity and low bulk ***,the insulation performance of aerogels is limited to a narrow temperature ***,the preparation of aerogel materials with precisely controlled and complex architectures is still ***,we report 3D printed polyimide/silica aerogel particle(PI/SAP)composite aerogels for thermal insulation in a wide range of temperature with customized *** printability and shape fidelity of 3D printed composite aerogels is improved by adding hydrophilic SAP as a rheology *** resulting PI/SAP composite aerogel exhibits excellent flame-retardant properties and thermal insulation from-50℃ to 1300℃.Moreover,the PI/SAP composite aerogel with customized shape can be applied for battery insulation at subzero temperatures,promising to be used as customizable and stable insulating materials in a variety of complex and extreme applications.

关键词： 3D printing Polyimide Aerogel Silica Thermal insulation

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Operando measurement of lattice deformation profiles of synchrotron radiation monochromator

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Nuclear Science and Techniques 2024年第7期35卷 8-19页

作者： yue Zhang Zhong-Liang Li Shang-Yu Si Lian Xue Hong-Xin Luo Xiao-Wei Zhang Jun hu Shanghai Synchrotron Radiation Facility Shanghai Advanced Research InstituteChinese Academy of SciencesShanghai201204China ShanghaiTech University Shanghai201210China University of the Chinese Academy of Sciences Beijing100049China Beijing Synchrotron Radiation Facility Institute of High Energy PhysicsChinese Academy of ScienceBeijing100049China

This study presents a new method for characterizing the thermal lattice deformation of a monochromator with high precision under service conditions and first reports the operando measurements of nanoscale thermal lattice deformation on a double-crystal monochromator at different incident *** nanoscale thermal lattice deformation of the monochromator first crystal was obtained by analyzing the intensity of the distorted DuMond *** diagrams of the 333 diffraction index,sensitive to lattice deformation,were obtained directly using a 2D detector and an analyzer crystal orthogonal to the *** increasing incident power and power density,the maximum height of the lattice deformation increased from 3.2 to 18.5 nm,and the deformation coefficient of the maximum height increased from 1.1 to 3.2 nm/*** maximum relative standard deviation was 4.2%,and the maximum standard deviation was 0.1 *** on the measured thermal deformations,the flux saturation phenomenon and critical point for the linear operation of the monochromator were predicted with increasing incident *** study provides a simple solution to the problem of the lower precision of synchrotron radiation monochromator characterizations compared to simulations.

关键词： Synchrotron radiation Monochromator Lattice deformation X-ray dynamic diffraction

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Thermal conductivity of iron under the Earth’s inner core pressure

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Chinese Physics B 2024年第10期33卷 343-348页

作者： Cui-E hu Mu-Xin jiao Xue-Nan Yang Zhao-Yi Zeng Jun Chen College of Physics and Electronic Engineering Chongqing Normal UniversityChongqing 400047China Laboratory of Computational Physics Institute of Applied Physics and Computational MathematicsBeijing 100088China Center for Applied Physics and Technology Peking UniversityBeijing 100071China

The thermal conductivity ofε-iron at high pressure and high temperature is a key parameter to constrain the dynamics and thermal evolution of the Earth’s *** this work,we use first-principles calculations to study the hugoniot sound velocity and the thermal transport properties ofε-*** total thermal conductivity considering lattice vibration is 200 W/mK at the Earth’s inner core *** suppressed anharmonic interactions can significantly enhance the lattice thermal conductivity under high pressure,and the contribution of the lattice thermal conductivity should not be ignored under the Earth’s core conditions.

关键词： thermal conductivity first-principles high pressure and high temperature

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