检索结果-南通市图书馆

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

引用

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

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Visible-Light Photoredox-Catalyzed Hydrodecarboxylation and Deuterodecarboxylation of Fatty Acids

引用

Chinese Journal of Chemistry 2022年第16期40卷 1903-1908页

作者： Yuan-Li Sun Fang-Fang Tan rong-gui hu Chun-Hong hu Yang Li Center for Organic Chemistry Frontier Institute of Science and TechnologyXi'an Jiaotong UniversityXi'anShaanxi710054 China

We develop an efficient visible-light photoreodox-catalyzed hydrodecarboxylation and deuterodecarboxylation of fatty acids for alkanes and deuterium *** key to the efficient transformation should attribute to the co-catalysis of the suitable methoxy substituted acridine photocatalyst(Mes-1,3,6,8-tetramethoxy-Acr-3″,5″-dimethoxy-Ph)and the hydrogen atom transfer catalyst 4′,4′-dimethyl diphenyldisulfane,possibly via facilitating of quenching the active alkyl radicals to overcome the competitive homocoupling products,olefins via the disproportionation,cracking products via the C—C bondβ-scission.

关键词： Photocatalysis Fatty acids Hydrodecarboxylation Deuterodecarboxylation Alkanes

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

TRIM72‑mediated degradation of the short form of p62/SQSTM1 rheostatically controls selective autophagy in human cells

引用

军事医学研究:英文版 2023年第4期10卷 559-562页

作者： Cheng‑Cheng Wang Hong Peng Zi Wang Jiao Yang rong‑gui hu Chuan‑Yin Li Wu‑Jun Geng Cancer Center Shanghai Tenth People’s HospitalSchool of MedicineTongji UniversityShanghai 200040China guizhou University School of Medicine Guiyang 550025China MOE Key Laboratory of Tropical Disease Control Centre for Infection and Immunity Study(CIIS)School of MedicineSun Yat-Sen UniversityShenzhen 510275GuangdongChina Institute for FrontierLife and Medical Sciences Kyoto UniversityKyoto 606‑8501Japan State Key Laboratory of Molecular Biology Shanghai Institute of Biochemistry and Cell BiologyCenter for Excellence in Molecular Cell ScienceChinese Academy of SciencesShanghai 200031China Department of Anesthesiology Wenzhou Key Laboratory of Perioperative Medicinethe First Affiliated Hospital of Wenzhou Medical UniversityWenzhou 325000ZhejiangChina

Dear Editor,Autophagy is an evolutionarily conserved catabolic process that involves the sequestration and transport of organelles,macromolecules,or invading microorganisms to lysosomes for degradation[1].Sequestosome 1(p62/SQSTM1)was the first protein shown to bind target-associated ubiquitin(Ub)and LC3 conjugated to the phagophore membrane,thus,acting as an important autophagy receptor for ubiquitinated targets[2].

关键词： p62S Alternative splicing Ubiquitination TRIM72 Autophagy

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Study on the green extraction of corncob xylan by deep eutectic solvent

引用

Grain & Oil Science and Technology 2024年第1期7卷 50-59页

作者： Bingyu Jiao Le Wang Haitao gui Zifu Ni rong Du Yuansen hu School of Biological Engineering National Engineering Research Center of Wheat and Corn Further ProcessingZhengzhou 450001China

Corn as one of the world's major food crops,its by-product corn cob is also rich in ***,the unreasonable utilization of corn cob often causes the environmental pollution,waste of resources and other *** one of the most abundant polymers in nature,xylan is widely used in food,medicine,materials and other *** cob is rich in xylan,which is an ideal raw material for extracting ***,the intractable lignin is covalently linked to xylan,which increases the difficulty of xylan *** has been reported that the deep eutectic solvent(DES)could preferentially dissolve lignin in biomass,thereby dissolving the ***,the xylan in the extract was separated by ethanol precipitation *** xylan precipitate was obtained after centrifugation,while the supernatant was *** components of the supernatant after ethanol precipitation were separated by the rotary *** ethanol,water and DES were collected for the subsequent extraction of corn cob *** this study,a novel way was provided for the green production of corn cob *** DES was used to extract xylan from corn cob which was used as the raw *** effects of solid-liquid ratio,reaction time,reaction temperature and water content of DES on the extraction rate of corn cob xylan were investigated by the single factor ***,the orthogonal test was designed to optimize the xylan extraction *** structure of corn cob xylan was analyzed and *** results showed that the optimum extraction conditions of corn cob xylan were as follows:the ratio of corn cob to DES was 1:15(g:mL),the extraction time was 3 h,the extraction temperature was 60℃,and the water content of DES was 70%.Under these conditions,the extraction rate of xylan was 16.46%.The extracted corn cob xylan was distinctive triple helix of polysaccharide,which was similar to the structure of commercially available *** was effectively and workably extracted from corn

关键词： Corncob Deep eutectic solvent Xylan Process optimization Extraction

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Coupled conditional nonlinear optimal perturbations and their application to ENSO ensemble forecasts

引用

Science China Earth Sciences 2024年第3期67卷 826-842页

作者： Wansuo DUAN Lei hu rong FENG LASG Institute of Atmospheric PhysicsChinese Academy of SciencesBeijing 100029China University of Chinese Academy of Sciences Beijing 100049China

Limitations are existed in current ensemble forecasting initial perturbation methods for describing the interactions among various spheres of the Earth system. In this study, a new method is proposed, namely, the coupled conditional nonlinear optimal perturbation(C-CNOP) method, which incorporates multisphere interactions much appropriately. The El Nino-Southern Oscillation(ENSO) is a typical ocean-atmosphere “coupling”(or “interaction”) phenomenon. The C-CNOP method is applied to ensemble forecasting of ENSO. It is demonstrated that the C-CNOP method can generate coupled initial perturbations(CPs) that appropriately consider initial ocean-atmosphere coupling uncertainty for ENSO ensemble forecasts. Results reveal that the CPs effectively improve the ability of ENSO ensemble-mean forecasts in both temporal variability of Nio3.4 sea surface temperature anomalies(SSTAs) and spatial variability of ENSO mature-phase SSTAs. Notably, despite the weakest ocean-atmosphere coupling strength in the tropical Pacific occurring during the boreal spring and summer, CPs still capture the uncertainties of this weak coupling when ENSO predictions are initialized at these seasons. This performance of CPs significantly suppresses the rapid increase of ENSO prediction errors due to the high ocean-atmosphere coupling instability during these seasons, and thus effectively extends the lead time of skillful ENSO forecasting. Hence, the C-CNOP method is a suitable initial perturbation approach for ENSO ensemble forecast that can describe initial ocean-atmosphere coupling uncertainty. It is expected that the CCNOP method plays a significant role in predictions of other high-impact climate phenomena, and even future Earth system predictions.

关键词： Ensemble forecast Multisphere interaction Initial perturbations ENSO

来源：

维普期刊数据库

同方期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Capillary Property of Entangled Porous Metallic Wire materials and Its Application in Fluid Buffers:Theoretical Analysis and Experimental Study

引用

Defence Technology（防务技术） 2024年第1期31卷 400-416页

作者： Yu Tang Yiwan Wu hu Cheng rong Liu Engineering Research Center for Metal Rubber School of Mechanical Engineering and AutomationFuzhou UniversityFuzhou 350156China AECC guizhou Liyang Aviation Power Co. LTDGuiyang 550014China College of Computer and Information Sciences Fujian Agriculture and Forestry UniversityFuzhou 350156China

Strong impact does serious harm to the military industries so it is necessary to choose reasonable cushioning material and design effective buffers to prevent the impact of *** on the capillary property entangled porous metallic wire materials(EPMWM),this paper designed a composite buffer which uses EPMWM and viscous fluid as cushioning materials under the low-speed impact of the recoil force device of weapon equipment(such as artillery,mortar,etc.).Combined with the capillary model,porosity,hydraulic diameter,maximum pore diameter and pore distribution were used to characterize the pore structure characteristics of *** calculation model of the damping force of the composite buffer was *** low-speed impact test of the composite buffer was *** parameters of the buffer under low-speed impact were identified according to the model,and the nonlinear model of damping force was *** test results show that the composite buffer with EPMWM and viscous fluid can absorb the impact energy from the recoil movement effectively,and provide a new method for the buffer design of weapon equipment(such as artillery,mortar,etc.).

关键词： Entangled porous metallic wire materials Capillary property Viscousfluid Low-speed impact Damping force

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Adaptive observer-based integral event-triggered antivibration control of a full aircraft active landing gear system with irregular runway excitations

引用

Science China(Information Sciences) 2024年第6期67卷 248-261页

作者： Wenxiao hu Chenglong DU Fanbiao LI Xinmin CHEN Chunhua YANG Weihua gui School of Automation Central South University Research Center in System Engineering for Special Aircrafts Ningbo Institute of Materials Technology and EngineeringChinese Academy of Sciences

This study addresses the antivibration issue of a full aircraft active landing gear system(LGS)under landing impact and runway excitations via a novel adaptive observer-based integral event-triggered control method. First, a full aircraft active LGS comprising a front gear and two synchronized main left and right gears with active suspensions is established, where the information exchange among them is not implemented directly. Second, two adaptive observers are designed separately for the front and main LGSs to identify the varying damping parameters and estimate the system states simultaneously. Third, estimated states are transmitted to a shared network through a novel integral event-triggered mechanism(I-ETM);thus, the front and main LGSs are interconnected, and a networked control scheme for the aircraft LGS is constructed. Additionally, the integrated active control law is designed with the aid of the transmitted states of the front and main LGSs, enhancing the robustness against the pitch vibration of the ***, the L2-stability of the hybrid system is guaranteed to depress the vertical vibration effect of the landing impact and irregular runway excitations, thus improving passenger safety and comfort. Moreover,a codesign method is provided to obtain feasible solutions to the gain matrices of the observers and controllers simultaneously. Simulation and comparison results are presented to illustrate the effectiveness and superiority of the proposed control scheme in antivibration and saving communication resources.

关键词： aircraft active landing gear antivibration control integral event-triggered mechanism adaptive observer ${\mathcal{L}}_2$-stability

来源：

同方期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Synergistic Interdecadal Evolution of Precipitation over Eastern China and the Pacific Decadal Oscillation during 1951-2015

引用

Advances in Atmospheric Sciences 2024年第1期41卷 53-72页

作者： Minmin WU rong-hua ZHANG Junya hu Hai ZHI Key Laboratory of Ocean Circulation and Waves Institute of OceanologyChinese Academy of SciencesQingdao 266071China School of Marine Sciences Nanjing University of Information Science and TechnologyNanjing 210044China Laosan Laboratory Qingdao 266237China School of Atmospheric Sciences Nanjing University of Information Science and TechnologyNanjing 210044China University of Chinese Academy of Sciences Beijing 100029China

By using the multi-taper method(MTM)of singular value decomposition(SVD),this study investigates the interdecadal evolution(10-to 30-year cycle)of precipitation over eastern China from 1951 to 2015 and its relationship with the North Pacific sea surface temperature(SST).Two significant interdecadal signals,one with an 11-year cycle and the other with a 23-year cycle,are identified in both the precipitation and SST *** show that the North Pacific SST forcing modulates the precipitation distribution over China through the effects of the Pacific Decadal Oscillation(PDO)-related anomalous Aleutian low on the western Pacific subtropical high(WPSH)and Mongolia high(MH).During the development stage of the PDO cold phase associated with the 11-year cycle,a weakened WPSH and MH increased the precipitation over the Yangtze River Basin,whereas an intensified WPSH and MH caused the enhanced rain band to move northward to North China during the decay *** the development stage of the PDO cold phase associated with the 23-year cycle,a weakened WPSH and MH increased the precipitation over North China,whereas an intensified WPSH and the weakened MH increased the precipitation over South China during the decay *** 11-year and 23-year variabilities contribute differently to the precipitation variations in the different regions of China,as seen in the 1998flooding *** 11-year cycle mainly accounts for precipitation increases over the Yangtze River Basin,while the 23-year cycle is responsible for the precipitation increase over Northeast *** results have important implications for understanding how the PDO modulates the precipitation distribution over China,helping to improve interdecadal climate prediction.

关键词： MTM-SVD PDO SST anomalies interdecadal variability precipitation over China

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Shock-induced chemical reaction characteristics of PTFE-Al-Bi_(2)O_(3)reactive materials

引用

Defence Technology（防务技术） 2024年第6期36卷 1-12页

作者： Chunlan Jiang rong hu Jingbo Zhang Zaicheng Wang Liang Mao State Key Laboratory of Explosion Science and Technology Beijing Institute of TechnologyBeijing 100081China

A ternary system of PTFE/Al/Bi_(2)O_(3)is constructed by incorporating PTFE-based reactive material and thermite for enhancing the energy release of the PTFE-based reactive *** effects of Bi_(2)O_(3)in the PTFE/Al/Bi_(2)O_(3)on both mechanical properties and the energy release were investigated through various tests such as thermogravimetry-differential scanning calorimetry,adiabatic oxygen bomb test and split Hopkinson pressure bar *** microstructure observed through scanning electron microscope and Xray diffraction results are used to analyze the ignition and reaction mechanism of PTFE/Al/Bi_(2)O_(3).The results indicate that the PTFE/Al/Bi_(2)O_(3)are capable of triggering the exothermic reaction of molten PTFE/Bi_(2)O_(3)and Al/Bi_(2)O_(3)over the PTFE/Al reactive materials,thereby promoting *** excessive aluminum in the ternary system is beneficial for increasing energy *** ignition of shock-induced chemical reactions in PTFE/Al/Bi_(2)O_(3)is closely related to the material *** dominant mechanism for hot-spot generation under Split Hopkinson Pressure Bar test is the frictional temperature rise at the microcrack after failure.

关键词： PTFE/Al/Bi_(2)O_(3) Shock-induced chemical reaction Energy release

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Different El Niño Flavors and Associated Atmospheric Teleconnections as Simulated in a Hybrid Coupled Model

引用

Advances in Atmospheric Sciences 2024年第5期41卷 864-880页

作者： Junya hu Hongna WANG Chuan GAO rong-hua ZHANG Key Laboratory of Ocean Circulation and Waves Institute of Oceanologyand Center for Ocean Mega-ScienceChinese Academy of SciencesQingdao 266071China School of Marine Sciences Nanjing University of Information Science and TechnologyNanjing 210044China Laoshan Laboratory Qingdao 266237China

A previously developed hybrid coupled model(HCM)is composed of an intermediate tropical Pacific Ocean model and a global atmospheric general circulation model(AGCM),denoted as *** this study,different El Niño flavors,namely the Eastern-Pacific(EP)and Central-Pacific(CP)types,and the associated global atmospheric teleconnections are examined in a 1000-yr control simulation of the *** HCMAGCM indicates profoundly different characteristics among EP and CP El Niño events in terms of related oceanic and atmospheric variables in the tropical Pacific,including the amplitude and spatial patterns of sea surface temperature(SST),zonal wind stress,and precipitation *** SST budget analysis indicates that the thermocline feedback and zonal advective feedback dominantly contribute to the growth of EP and CP El Niño events,*** to the shifts in the tropical rainfall and deep convection during EP and CP El Niño events,the model also reproduces the differences in the extratropical atmospheric responses during the boreal *** particular,the EP El Niño tends to be dominant in exciting a poleward wave train pattern to the Northern Hemisphere,while the CP El Niño tends to preferably produce a wave train similar to the Pacific North American(PNA)*** a result,different climatic impacts exist in North American regions,with a warm-north and cold-south pattern during an EP El Niño and a warm-northeast and cold-southwest pattern during a CP El Niño,*** modeling result highlights the importance of internal natural processes within the tropical Pacific as they relate to the genesis of ENSO diversity because the active ocean–atmosphere coupling is allowed only in the tropical Pacific within the framework of the HCMAGCM.

关键词： hybrid coupled model tropical Pacific Ocean global atmosphere Eastern/Central-Pacific El Niño atmospheric teleconnections

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：