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Novel MIP gene mutation causes autosomal-dominant congenital cataract

International Journal of Ophthalmology(English edition) 2024年第3期17卷 454-465页

作者： Jing-Lan Ni hua-ming wen Xiao-Sheng huang Qian-wen Li Jia-Min Cai Bao-Jian Fan Jun Zhao The Second Clinical Medical College Jinan UniversityShenzhen 518020Guangdong ProvinceChina Department of Ophthalmology Dongguan Chang’an HospitalDongguan 523843Guangdong ProvinceChina Shenzhen Eye Hospital Affiliated to Jinan University Shenzhen Eye InstituteShenzhen 518040Guangdong ProvinceChina Department of Oral&Maxillofacial Surgery Shenzhen Stomatology Hospital Affiliated to Shenzhen UniversityShenzhen 518040Guangdong ProvinceChina Department of Neurosurgery Massachusetts General Hospital and Harvard Medical SchoolBoston 02114MassachusettsUSA Department of Ophthalmology Shenzhen People’s Hospital(the Second Clinical Medical CollegeJinan Universitythe First Affiliated HospitalSouthern University of Science and Technology)Shenzhen 518020Guangdong ProvinceChina

●AIM:To identify disease-causative mutations in families with congenital cataract.●METHODS:Two Chinese families with autosomaldominant congenital cataract(ADCC)were recruited and underwent comprehensive eye *** panel next-generation sequencing of common pathogenic genes of congenital cataract was performed in the proband of each *** sequencing was used to valid the candidate gene mutations and sequence the other family members for co-segregation *** effect of sequence changes on protein structure and function was predicted through bioinformatics *** intrinsic protein(MIP)-wildtype and MIP-G29R plasmids were constructed and microinjected into zebrafish single-cell stage *** embryonic lens phenotypes were screened using confocal microscopy.●RESULTS:A novel heterozygous mutation(c.85G>A;p.G29R)in the MIP gene was identified in the proband of one family.A known heterozygous mutation(c.97C>T;p.R33C;rs864309693)in MIP was found in the proband of another ***-silico prediction indicated that the novel mutation might affect the MIP protein *** embryonic lens was uniformly transparent in both wild-type PCS2+MIP and mutant PCS2+MIP.●CONCLUSION:Two missense mutations in the MIP gene in Chinese cataract families are identified,and one of which is *** findings expand the genetic spectrum of MIP mutations associated with *** functional studies suggest that the novel MIP mutation might not be a gain-of-function but a loss-of-function mutation.

关键词： congenital cataract major intrinsic protein missense mutation zebrafish model

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Weakly Approximate Diagonalization of Homomorphisms into Finite von Neumann Algebras

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Acta Mathematica Sinica,English Series 2024年第9期40卷 2187-2194页

作者： wen hua QIAN Jun Hao SHEN wen ming WU School of Mathematical Sciences Chongqing Normal UniversityChongqing 401331P.R.China Department of Mathematics and Statistics University of New HampshireDurhamNH 03824USA

Let A be a unital C^(∗)-algebra and B a unital C^(∗)-algebra with a faithful traceτ.Let n be a positive *** give the definition of weakly approximate diagonalization(with respect toτ)of a unital homomorphismφ:A→Mn(B).We give an equivalent characterization of McDuff Ⅱ_(1) *** show that,if A is a unital nuclear C^(∗)-algebra and B is a type Ⅱ_(1) factor with faithful traceτ,then every unital^(∗)-homomorphism φ:A→M_(n)(B)is weakly approximately *** B is a unital simple infinite dimensional separable nuclear C^(∗)-algebra,then any finitely many elements in Mn(B)can be simultaneously weakly approximately diagonalized while the elements in the diagonals can be required to be the same.

关键词： Weakly approximately diagonalizable McDuff factors nuclear hyperfinite

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Goal-Oriented Control Systems(GOCS):From HOW to WHAT

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IEEE/CAA Journal of Automatica Sinica 2024年第4期11卷 816-819页

作者： wen-hua Chen the Department of Aeronautical and Automotive Engineering Loughborough UniversityLeicestershireLE113TUU.K.

Brief:New control theory is required to underpin safe design and deployment of future highly automated systems to deal with uncertain environments and complicated tasks,enabled by AI and other advanced ***-Oriented Control Systems offer potential to transform the control system design from currently instructing a control system how to perform a task to specifying what is to be achieved.

关键词： automated instru system

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Synergistic catalysis of the N-hydroxyphthalimide on flower-like bimetallic metal-organic frameworks for boosting oxidative desulfurization

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Petroleum Science 2024年第1期21卷 674-682页

作者： Jing He Kun Zhu Wei Jiang Dong-Ao Zhu Lin-hua Zhu Hai-Yan huang wen-Shuai Zhu hua-ming Li Institute for Energy Research Jiangsu UniversityZhenjiang212013JiangsuChina College of Chemistry and Chemical Engineering Hainan Normal UniversityKey Laboratory of Water Pollution Treatment and Resource Reuse of Hainan ProvinceHaikou571158HainanChina

Synergic catalytic effect between active sites and supports greatly determines the catalytic activity for the aerobic oxidative desulfurization of fuel *** this work,Ni-doped Co-based bimetallic metal-organic framework(CoNi-MOF)is fabricated to disperse N-hydroxyphthalimide(NHPI),in which the whole catalyst provides plentiful synergic catalytic effect to improve the performance of oxidative desulfurization(ODS).As a bimetallic MOF,the second metal Ni doping results in the flower-like morphology and the modification of electronic properties,which ensure the exposure of NHPI and strengthen the synergistic effect of the overall *** with the monometallic Co-MOF and naked NHPI,the NHPI@CoNi-MOF triggers the efficient activation of molecular oxygen and improves the ODS performance without an *** sulfur removal of dibenzothiophene-based model oil reaches 96.4%over the NHPI@CoNi-MOF catalyst in 8 h of ***,the catalytic product of this aerobic ODS reaction is sulfone,which is adsorbed on the catalyst surface due to the difference in *** work provides new insight and strategy for the design of a strong synergic catalytic effect between NHPI and bimetallic supports toward high-activity aerobic ODS materials.

关键词： Metal-organic frameworks Doped Bimetallic N-hydroxyphthalimide Aerobic processes Oxidative desulfurization

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Design of dual-functional protic porous ionic liquids for boosting selective extractive desulfurization

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Petroleum Science 2024年第4期21卷 2817-2829页

作者： Jin-Rui Zhang Jie Yin Jing He Hong-Shun Ran Wei Jiang Hong-Ping Li wen-Shuai Zhu hua-ming Li ming Zhang School of the Environment and Safety Engineering & Institute for Energy Research Jiangsu University School of Chemistry and Chemical Engineering Jiangsu University

Porous ionic liquids have demonstrated excellent performance in the field of separation, attributed to their high specific surface area and efficient mass transfer. Herein, task-specific protic porous ionic liquids(PPILs) were prepared by employing a novel one-step coupling neutralization reaction strategy for extractive desulfurization. The single-extraction efficiency of PPILs reached 75.0% for ***, adding aromatic hydrocarbon interferents resulted in a slight decrease in the extraction efficiency of PPILs(from 45.2% to 37.3%, 37.9%, and 33.5%), indicating the excellent extraction selectivity of PPILs. The experimental measurements and density functional theory calculations reveal that the surface channels of porous structures can selectively capture dibenzothiophene by the stronger electrophilicity(Eint(HS surface channel/DBT)=-39.8 kcal mol-1), and the multiple extraction sites of ion pairs can effectively enrich and transport dibenzothiophene from the oil phase into PPILs through π...π, C-H...π and hydrogen bonds interactions. Furthermore, this straightforward synthetic strategy can be employed in preparing porous liquids, offering new possibilities for synthesizing PPILs with tailored functionalities.

关键词： Protic porous ionic liquids Extractive desulfurization Selectivity Density functional theory

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Nano polystyrene microplastics could accumulate in Nile tilapia(Oreochromis niloticus):Negatively impacts on the intestinal and liver health through water exposure

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Journal of Environmental Sciences 2024年第3期137卷 604-614页

作者： Di Wu Xing Lu Li-Xue Dong Juan Tian Jin Deng Lei Wei hua wen Shan Zhong ming Jiang School of Basic Medical Sciences Wuhan UniversityWuhan 430071China Key Laboratory of Freshwater Biodiversity Conservation Ministry of Agriculture and Rural AffairsYangtze River Fisheries Research InstituteChinese Academy of Fishery SciencesWuhan 430223China Hubei Province Key Laboratory of Allergy and Immunology Wuhan 430071China

Microplastics(MPs)have become a significant concern for their potential ***,the correlation between the size of plastic particles and their toxicity remains ***,we investigate the toxic effects of different sizes(80 nm,800 nm,8μm and 80μm)polystyrene MPs(PS-MPs)on the model organism Nile tilapia(Oreochromis niloticus).The results of bioluminescent imaging indicate that the 80 nm PS-MPs are more likely to invade the body.H&E staining shows severe damage on the intestinal villi and distinct hepatic steatosis in the 80 nm *** U labeling shows that the proliferation activity of intestinal and liver cells reduces significantly in the 80 nm *** gut microbiome analysis shows a severe imbalance of gut microbiota homeostasis in the 80 nm *** analysis of liver transcriptomics and metabolomics shows that the liver lipid metabolism is disordered in the 80 nm *** conclusion,this study confirms that the 80 nm PS-MPs are more likely to induce intestinal and liver *** the above lay the foundation for further study on the pathological damage of MPs to other organisms.

关键词： Microplastics(MPs) Intestinal toxicity Liver toxicity Gut microbiome Lipid metabolism

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Using Copula functions to predict climatic change impacts on floods in river source regions

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Advances in Climate Change Research 2024年第3期15卷 406-418页

作者： Ting-Xing CHEN Hai-Shen LYU Robert HORTON Yong-hua ZHU Ren-Sheng CHEN ming-Yue SUN ming-wen LIU Yu LIN The National Key Laboratory of Water Disaster Prevention College of Hydrology and Water ResourcesHohai UniversityNanjing 2098China College of Hydrology and Water Resources Hohai UniversityNanjing 210098China Departmentof Agronomy lowa State UniversityAmes IA5oollUSA Qilian Alpine Eology and Hydrology Research Station Northwest Instiuteof Eco-Environmentand ReourcesChinese Academy of SciencesLanzhou0000 China

Flood frequency in river source regions is significantly affected by rainfall and snowmelt as part of climatic changes.A traditional univariate flood frequency analysis cannot reflect the complexity of floods,and when used in isolation,it can only underestimate flood *** effective flood prevention and mitigation,it is essential to consider the combined effects of precipitation and *** functions can effectively quantify the joint distribution relationship between floods and their associated variables without restrictions on their distribution *** study uses copula functions to consider a multivariate probability distribution model of flood peak flow(Q)with cumulative snowmelt(CSm)and cumulative precipitation(CPr)for the Hutubi River basin located in northern Xinjiang,*** joint frequencies of rainfall and snowmelt floods are predicted using copula models based on the Coupled Model Intercomparison Project Phase 6 *** results show that Q has a significant positive correlation with 24-d CSm(r=0.559,p=0.002)and 23-d CPr(r=0.965,p<0.05).Flood frequency will increase in the future,and mid-(2050e2074)and long-term(2075e2099)floods will be more severe than those in the near-term(2025e2049).The probability of flood occurrence is higher under the SSP2-4.5 and SSP1-2.6 scenarios than under *** during the historical period(1990e2014)led to extreme floods,and increasing future precipitation trends are found to be *** increases with rising temperatures and occurs earlier than estimated,leading to an earlier flood period in the basin and more frequent snowmelt *** Q under the joint return period is larger than that during the same univariate return *** difference indicates that neglecting the interaction between precipitation and snowmelt for floods leads to an underestimation of the flood risk(with underestimations ranging from 0.3%to 22%).The underestimations decrease with an increase

关键词： Climate change Flood frequency Copula CMIP6 River source regions

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Fluoro-functionalized plant biomass adsorbent:Preparation and application in extraction of trace perfluorinated compounds from environmental water samples

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Journal of Environmental Sciences 2024年第3期137卷 703-715页

作者： Xi Tan Yan Shi Chun-Feng Ma Quan Chi Yu-Hang Yang wen-Xiang Zhang hua-ming Xiao Xian Wang Key Laboratory of Analytical Chemistry of the State Ethnic Affairs Commission School of Chemistry and Materials ScienceSouth-Central Minzu UniversityWuhan 430074China School of Pharmaceutical Sciences South-Central Minzu UniversityWuhan 430074China Key Laboratory of Oilseeds Processing of Ministry of Agriculture Hubei Key Laboratory of Lipid Chemistry and NutritionOil Crops Research Institute of the Chinese Academy of Agricultural SciencesWuhan 430062China

Perfuorinated compounds(PFCs)are toxic and widely present in the environment,and therefore effective adsorbents are required to remove PFCs from environmental *** the present study,a new type of fuorinated biomass materials was synthesized via an ingenious fuorosilanization *** adsorbents were applied for the adsorption of 13typical PFCs,including perfuorocarboxylic acids(PFCAs)and perfuorosulfonic acids(PFSAs).By comparing their adsorption performance,Fluorinated cedar slag(FCS)was discovered to have the best absorption efficiency and enabled highly efficient enrichment of *** adsorption recovery of FCS with the investigated PFCs is greater than 90%under the optimal adsorption *** to the high affinity of F-F sorbent-sorbate interaction,FCS had good adsorption capacities of PFCs from aqueous solution,with the maximum adsorption capacity of 15.80 mg/g for PFOS and 10.71 mg/g for PFOA,***,the adsorption time could be achieved in a short time(8 min).Using the FCS absorbent,an innovative FCS-solid phase extraction assisted with high performance liquid chromatography-electrospray-tandem mass spectrometry(FCS-SPE-HPLC-ESI-MS/MS)method was first developed to sensitively detect PFCs in the environmental water *** intra-day and inter-day recovery rates of the 13 compounds ranged from 90.7%-104.3%,with the RSD of 2.1%-4.7%(intra-day)and 2.5%-8.5%(inter-day),*** research demonstrates the potential of the newly fuoro-functionalized plant biomass to adsorb PFCs from environmental water,with the advantages of high adsorption efficiencies,high antiinterference,easy operation and low economic cost.

关键词： Fluoro-functionalized biomass Perfuorinated compounds(PFCs) Adsorption efficiency Mass spectrometry(MS) Fluorinated cedar slag(FCS)

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Influence of laser parameters on the microstructures and surface properties in laser surface modification of biomedical magnesium alloys

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Journal of Magnesium and Alloys 2024年第1期12卷 72-97页

作者： Chee Ying Tan Cuie wen hua Qian Ang School of Engineering RMIT UniversityMelbourneVictoria 3001Australia

Biodegradable implants from magnesium(Mg)alloys have emerged in the biomedical field especially in the orthopedic and cardiovascular stent applications owing to their low density,high specific strength,excellent machinability,good biocompatibility,and *** primary shortcoming of Mg-based implants is their low corrosion resistance in the physiological environment,which results in premature mechanical integrity loss before adequate healing and the production of excessive hydrogen gas,which is harmful to the body tissues and negatively affects the biocompatibility of the *** surface modification has recently received attention because it can improve the surface properties such as surface chemistry,roughness,topography,corrosion resistance,wear resistance,hydrophilicity,and thus cell response to the surface of the *** composition and microstructures including textures and phases of laser-treated surfaces depend largely on the laser processing parameters(input laser power,laser scan velocity,frequency,pulse duration,pressure,gas circulation,working time,spot size,beam focal position,and laser track overlap)and the thermophysical properties of the substrate(solubility,melting point,and boiling point).This review investigates the impacts of various laser surface modification techniques including laser surface melting,laser surface alloying,laser cladding,laser surface texturing,and laser shock peening,and highlights their significance in improving the surface properties of biodegradable Mg alloys for implant ***,we explore how different laser process parameters affect its composition,microstructure,and surface properties in each laser surface modification technique.

关键词： Biocompatibility Biodegradability Corrosion Implant applications Laser surface modification Magnesium alloys

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Clinical Outcomes and Risks of Single-stage Bilateral Unicompartmental Knee Arthroplasty via Oxford Phase III

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Chinese Medical Journal 2015年第21期128卷 2861-2865页

作者： Tong Ma Yi-Hui Tu hua-ming Xue Tao wen Min-Wei Cai Department of Orthopedics YangPu District Central Hospital Affiliated to Tongji University Shanghai 200090 China

Background： Osteoarthritis often affects the joint bilaterally, and the single-stage （SS） unicompartmental knee arthroplasty （UKA） is advantageous in terms of a single anesthesia administration, a short hospital stay, lower medical costs, and enhanced patient convenience. However, the complication risk of SS UKA continues to be debated. The aim of this article was to evaluate the clinical effectiveness, complications, and functional recovery of SS and two-stage （TS） UKA. Methods： From January 2008 to December 2013, we compared a series of 36 SS UKA with 45 TS UKA for osteoarthritis. The mean age was 65.4 years （range： 55-75 years）. The mean body mass index was 25.2 kg/m^2 （range： 22-29 kg/m^2）. The preand post-operative Oxford Knee Scores （OKSs）, complications, operative times, tourniquet times, the amount of drainage, and hemoglobin （Hb） were evaluated. The Chi-square test, Fisher＇s exact test, and paired and grouped t-tests were used in this study. Results： The mean tbllow-up was 50 months. No complications of death, fat embolism, deep vein thrombosis, and prosthetic infection were reported. Patients who underwent SS U KA had a shorter cumulative anesthesia time （ 113.5 vs. 133.0 rain, P 〈 0.01 ）. There were no significant variations between the values of the mean tourniquet time, the amount of drainage, pre- and post-operative Hb in the different groups. No patient required a blood transfusion. No statistical differences were found in the complications between two groups （P 〉 0.05）. At the final follow-up, the mean OKS improved from 39.48 ±5.69 to 18.83 ± 3.82 （P 〈 0.01 ）, with no statistical differences between the two groups （P 〉 0.05）. Patients who underwent SS UKA had a faster recovery. Conclusions： The single-staged UKA offers the benefits of a single anesthesia administration, redticed total anesthetic time, decreased overall rehabilitation time, and absence of an increase in perioperative mortality or complications compared with the TS bilateral UKA.

关键词： Arthroplasty Bilateral: Osteoarthritis: Single-stage: Unicompartmental

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