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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 deficiency.For 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 hyperplasia.Methods Clinical data for 10 Chinese patients diagnosed with congenital adrenal hyperplasia in our hospital from 2018 to 2022 were retrospectively analyzed.We examined the effects of gene mutations on protease activity and constructed threedimensional structure prediction models of proteins.Results We describe 10 patients with 11beta-hydroxylase gene mutations(n=5,46,XY;n=5,46,XX),with 10 novel mutations were reported.Female 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 years.The most common CYP11B1 pathogenic variant in the Chinese population was found to be c.1360C>T.All mutations lead to spatial conformational changes that affect protein stability.Conclusions Our study found that there was no significant correlation between each specific mutation and the severity of clinical manifestations.Different patients with the same gene pathogenic variant may have mild or severe clinical manifestations.The correlation between genotype and phenotype needs further study.Three-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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The ubiquitin codes in cellular stress responses

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Protein & Cell 2024年第3期15卷 157-190页

作者： Xiangpeng Sheng Zhixiong Xia Hanting Yang ronggui hu Key Laboratory of Systems Health Science of Zhejiang Province School of Life ScienceHangzhou Institute for Advanced StudyUniversity of Chinese Academy of SciencesHangzhou 310024China State Key Laboratory of Molecular Biology Shanghai Institute of Biochemistry and Cell BiologyCenter for Excellence in Molecular Cell ScienceChinese Academy of SciencesShanghai 200031China State Key Laboratory of Animal Disease Control Harbin Veterinary Research InstituteChinese Academy of Agricultural SciencesHarbin 150069China Department of Neurology State Key Laboratory of Medical NeurobiologyInstitute for Translational Brain ResearchMOE Frontiers Center for Brain ScienceZhongshan HospitalFudan UniversityShanghai 200032China

Ubiquitination/ubiquitylation,one of the most fundamental post-translational modifications,regulates almost every critical cellular process in eukaryotes.Emerging evidence has shown that essential components of numerous biological processes undergo ubiquitination in mammalian cells upon exposure to diverse stresses,from exogenous factors to cellular reactions,causing a dazzling variety of functional consequences.Various forms of ubiquitin sig-nals generated by ubiquitylation events in specific milieus,known as ubiquitin codes,constitute an intrinsic part of myriad cellular stress responses.These ubiquitination events,leading to proteolytic turnover of the substrates or just switch in functionality,initiate,regulate,or supervise multiple cellular stress-associated responses,supporting adaptation,homeostasis recovery,and survival of the stressed cells.In this review,we attempted to summarize the crucial roles of ubiquitination in response to different environmental and intracellular stresses,while discussing how stresses modulate the ubiquitin system.This review also updates the most recent advances in understanding ubiquitination machinery as well as different stress responses and discusses some important questions that may warrant future investigation.

关键词： ubiquitin E3 ligase environmental stresses intercellular stresses stress response homeostasis

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

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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 resources.However,the unreasonable utilization of corn cob often causes the environmental pollution,waste of resources and other problems.As one of the most abundant polymers in nature,xylan is widely used in food,medicine,materials and other fields.Corn cob is rich in xylan,which is an ideal raw material for extracting xylan.However,the intractable lignin is covalently linked to xylan,which increases the difficulty of xylan extraction.It has been reported that the deep eutectic solvent(DES)could preferentially dissolve lignin in biomass,thereby dissolving the xylan.Then,the xylan in the extract was separated by ethanol precipitation method.The xylan precipitate was obtained after centrifugation,while the supernatant was retained.The components of the supernatant after ethanol precipitation were separated by the rotary evaporator.The ethanol,water and DES were collected for the subsequent extraction of corn cob xylan.In this study,a novel way was provided for the green production of corn cob xylan.The DES was used to extract xylan from corn cob which was used as the raw material.The 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 test.Furthermore,the orthogonal test was designed to optimize the xylan extraction process.The structure of corn cob xylan was analyzed and verified.The 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 xylan.Xylan was effectively and workably extracted from corn cob by the DES method.This study provided a new

关键词： Corncob Deep eutectic solvent Xylan Process optimization Extraction

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

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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

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

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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 alkanes.The 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

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

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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 equipment.Based 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 EPMWM.The calculation model of the damping force of the composite buffer was established.The low-speed impact test of the composite buffer was conducted.The parameters of the buffer under low-speed impact were identified according to the model,and the nonlinear model of damping force was obtained.The 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

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Runx2 regulates peripheral nerve regeneration to promote Schwann cell migration and re-myelination

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Neural Regeneration Research 2024年第7期19卷 1575-1583页

作者： rong hu Xinpeng Dun Lolita Singh Matthew C.Banton School of Traditional Chinese Medicine Department of Traditional Chinese MedicineNanfang HospitalSouthern Medical UniversityGuangzhouGuangdong ProvinceChina The Co-innovation Center of Neuroregeneration Nantong UniversityNantongJiangsu ProvinceChina Faculty of Health University of PlymouthPlymouthUK

Runx2 is a major regulator of osteoblast differentiation and function;however,the role of Runx2 in peripheral nerve repair is unclea r.Here,we analyzed Runx2expression following injury and found that it was specifically up-regulated in Schwann cells.Furthermore,using Schwann cell-specific Runx2 knocko ut mice,we studied peripheral nerve development and regeneration and found that multiple steps in the regeneration process following sciatic nerve injury were Runx2-dependent.Changes observed in Runx2 knoc kout mice include increased prolife ration of Schwann cells,impaired Schwann cell migration and axonal regrowth,reduced re-myelination of axo ns,and a block in macrophage clearance in the late stage of regeneration.Taken together,our findings indicate that Runx2 is a key regulator of Schwann cell plasticity,and therefore peripheral nerve repair.Thus,our study shows that Runx2 plays a major role in Schwann cell migration,re-myelination,and peripheral nerve functional recovery following injury.

关键词： macrophage clearance migration peripheral nerve injury regeneration re-myelination Runx2 Schwann cells

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

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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 aircraft.Furthermore, 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

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

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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 fields.Results 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 stage.During 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 stage.The 11-year and 23-year variabilities contribute differently to the precipitation variations in the different regions of China,as seen in the 1998flooding case.The 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 China.These 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

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

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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 HCMAGCM.In 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.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 anomalies.An SST budget analysis indicates that the thermocline feedback and zonal advective feedback dominantly contribute to the growth of EP and CP El Niño events,respectively.Corresponding 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 winter.In 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)pattern.As 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,respectively.This 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

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