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A novel mutation of RPGR in a Chinese family with X-linked retinitis pigmentosa

International Journal of Ophthalmology(English edition) 2022年第9期15卷 1423-1430页

作者： Hui-Hui Sun Jing-Cong Zhao Su-Ling Yang Jin-Dou Shi yun-shuo wei Jian-Cang Wang Feng Gu Lu Chen Children's Hospital of Hebei Province Shijiazhuang 0050000Hebei ProvinceChina Hebei General Hospital Shijiazhuang 050000Hebei ProvinceChina School of Ophthalmology and Optometry Eye HospitalWenzhou Medical UniversityState Key Laboratory Cultivation Base and Key Laboratory of Vision ScienceMinistry of Health and Zhejiang Provincial Key Laboratory of Ophthalmology and OptometryWenzhou 325027Zhejiang ProvinceChina

·AIM:To identify potential mutations and elucidate the clinical findings of male patients and female carriers of X-Iinked retinitis pigmentosa(XLRP)in a Chinese family.·METHODS:A four generation pedigree was collected that consisted of 20 *** DNA was extracted from peripheral blood,and then the target fragments were amplified by PCR and sequenced *** addition,all affected patients and female carriers underwent comprehensively ophthalmic evaluation.·RESULTS:A novel mutation c.2865 G>A p.W955 X in RPGR gene was identified of this family,including four affected individuals and eight *** male patients,aging from 7 to 31 y,tended to have more various,even potentially deleterious clinical features of *** the same time,individuals with heterozygous mutations(carriers)manifested a wide spectrum of clinical ***,only two male patients and three female carriers manifested pathological myopia(PM).Among the female carriers,half of subjects who harbor poor visual acuity suffered esotropia or ***,16.7%and 66.7%of carriers had abnormal electroretinogram(ERG)and fundus,respectively.·CONCLUSION:In this study,a novel mutation of the RPGR gene is identified,which broadens the spectrum of RPGR mutations,and elaborates the relationship between genotype and phenotype.

关键词： X-linked retinitis pigmentosa RPGR nonsense mutation phenotype

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Simulation of nanofluid natural convection based on single-particle hydrodynamics in energy-conserving dissipative particle dynamics(eDPD)

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Applied Mathematics and Mechanics(English Edition) 2024年第8期45卷 1429-1446页

作者： wei LU shuo CHEN Zhiyuan YU Jiayi ZHAO School of Aerospace Engineering and Applied Mechanics Tongji University Shanghai Key Laboratory of Vehicle Aerodynamics and Vehicle Thermal Management Systems Tongji University School of Energy and Power Engineering University of Shanghai for Science and Technology

In the present study, the nanofliud natural convection is investigated by the energy-conserving dissipative particle dynamics(eDPD) method, where the nanoparticles are considered at the single-particle level. The thermal expansion coefficient β and the viscosity μ of the simulated system containing nanoparticles are calculated and found to be in close alignment with the previous simulation results. The single-particle hydrodynamics in e DPD enables simulations of nanofluid natural convection with higher Rayleigh numbers and greater nanoparticle volume fractions. Additionally, this approach is utilized to simulate the nanoparticle distribution during the enhanced heat transfer process in the nanofluid natural convection. The localized aggregation of nanoparticles enhances the heat transfer performance of the nanofluid under specific Rayleigh numbers and nanoparticles volume fractions.

关键词： 68U01 energy-conserving dissipative particle dynamics (eDPD) heat transfer nanofluid nanoparticle O351.2 single-particle hydrodynamics

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Genetically predicted fatty liver disease and risk of psychiatric disorders: A mendelian randomization study

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World Journal of Clinical Cases 2024年第14期12卷 2359-2369页

作者： wei-Ming Xu Hai-Fu Zhang Yong-Hang Feng shuo-Jun Li Bi-yun Xie Department of Medicine The First People's Hospital of FuyangHangzhou 311400Zhejiang ProvinceChina Department of Internal Medicine The First People's Hospital of FuyangHangzhou 311400Zhejiang ProvinceChina

BACKGROUND Non-alcoholic fatty liver disease(NAFLD)and alcohol-related liver disease(Ar-LD)constitute the primary forms of chronic liver disease,and their incidence is progressively increasing with changes in lifestyle *** studies have do-cumented a correlation between the occurrence and development of prevalent mental disorders and fatty *** To investigate the correlation between fatty liver and mental disorders,thus ne-cessitating the implementation of a mendelian randomization(MR)study to elu-cidate this *** Data on NAFLD and ArLD were retrieved from the genome-wide association studies catalog,while information on mental disorders,including Alzheimer's disease,schizophrenia,anxiety disorder,attention deficit hyperactivity disorder(ADHD),bipolar disorder,major depressive disorder,multiple personality dis-order,obsessive-compulsive disorder(OCD),post-traumatic stress disorder(PTSD),and schizophrenia was acquired from the psychiatric genomics consor-tium.A two-sample MR method was applied to investigate mediators in signifi-cant *** After excluding weak instrumental variables,a causal relationship was identified between fatty liver disease and the occurrence and development of some psychia-tric ***,the findings indicated that ArLD was associated with a significantly elevated risk of developing ADHD(OR:5.81,95%CI:5.59-6.03,P<0.01),bipolar disorder(OR:5.73,95%CI:5.42-6.05,P=0.03),OCD(OR:6.42,95%CI:5.60-7.36,P<0.01),and PTSD(OR:5.66,95%CI:5.33-6.01,P<0.01).Meanwhile,NAFLD significantly increased the risk of developing bipolar disorder(OR:55.08,95%CI:3.59-845.51,P<0.01),OCD(OR:61.50,95%CI:6.69-565.45,P<0.01),and PTSD(OR:52.09,95%CI:4.24-639.32,P<0.01).CONCLUSION Associations were found between genetic predisposition to fatty liver disease and an increased risk of a broad range of psychiatric disorders,namely bipolar disorder,OCD,and PTSD,highlighting the significance of preven-tive measures against psy

关键词： Non-alcoholic fatty liver disease Alcohol-related liver disease Psychiatric disorders Mendelian randomization Single nucleotide polymorphisms

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mTORC2:The“Ace in the Hole”for a Broader Control of Epileptic Seizures?

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Neuroscience Bulletin 2024年第5期40卷 677-679页

作者： shuo Zhang Xiongfeng Guo wei Huang Cenglin Xu Department of Pharmacy The First Affiliated Hospital of Zhejiang Chinese Medical University(Zhejiang Provincial Hospital of Chinese Medicine)Hangzhou310006China The Second Affiliated Hospital of Zhejiang Chinese Medical University(Zhejiang Xinhua Hospital) Key Laboratory of Neuropharmacology and Translational Medicine of Zhejiang ProvinceSchool of Pharmaceutical SciencesZhejiang Chinese Medical UniversityHangzhou310053China

Over 70 million people worldwide suffer from epilepsy-one of the most prevalent chronic neurological disorders[1].Despite the availability of over thirty anti-seizure drugs(ASDs)that mainly act on voltage-gated ion channels or inhibitory GABAergic receptors,the effectiveness of these drugs in achieving seizure freedom remains unsatisfactory due to pharmacoresistance and the inevitable occurrence of side-effects.

关键词： drugs spite epilepsy

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Chain Dynamics Heterogeneity in Plasticized Poly(vinyl butyral)(PVB) as Elucidated by Solid-State NMR

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Chinese Journal of Polymer Science 2024年第1期42卷 113-124,I0010页

作者： Jing Lv Xu Chen Zi-shuo Wu Ya-Hui Li wei Chen National Synchrotron Radiation Laboratory Anhui Provincial Engineering Laboratory of Advanced Functional Polymer FilmCAS Key Laboratory of Soft Matter ChemistryUniversity of Scienceand Technology of ChinaHefei 230026China

The chain dynamics heterogeneity of the poly(vinyl butyral)(PVB) plasticized by triethylene glycol bis(2-ethylhexa noate)(TEG-EH) was investigated by various solid-state NMR *** plasticized PVB shows two domains in distinct molecular dynamics differences,namely,rigid and soft domains,where the latter is the plasticizer-rich *** time domain low field NMR was first used to investigate the dynamics heterogeneity of the plasticized PVB,and the results show the decreasing activated energy of components in the soft domain of plasticized PVB(E_a=20.2 kJ/mol) as compared with that of the pristine one(E_a=24.3 kJ/mol).Detailed dynamics heterogeneity was obtained by high-field NMR with site-specific *** quadrupole-echo ~2H-NMR was adopted to elucidate the dynamics heterogeneity of the vinyl alcohol(VA) units,where only the hydroxyl group of VA is *** ~1H-^(13)C WISE NMR spectra show that there is not much difference in the mobility of the VB unit in PVB with and without plasticizer,whereas the glass transition temperature differed by approximately 53℃.This is further supported by Torchia's T_1 relaxation *** origin of such an unusual phenomenon is attributed to the critical role of the remaining VA(~22%) in the soft domain,where the VA units locally aggregate through hydrogen ***,the existence of a mobility gradient in the VB unit has been ***,the mobility difference for VB with different stereo-geometry(meso or racemic conformation) is observed for the first *** indicates the importance of modulating the ratio of meso over racemic VB for controlling the macroscopic perfo rmance of PVB.

关键词： Poly(vinyl butyral) Dynamics heterogeneity Plasticizer Solid state NMR

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Conductive and stable polyphenylene/CNT composite membrane for electrically enhanced membrane fouling mitigation

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Frontiers of Environmental Science & Engineering 2024年第1期18卷 35-47页

作者： Huijuan Xie Haiguang Zhang Xu Wang Gaoliang wei shuo Chen Xie Quan Key Laboratory of Industrial Ecology and Environmental Engineering(Ministry of Education) School of Environmental Science and TechnologyDalian University of TechnologyDalian 116024China

Nanocarbon-based conductive membranes, especially carbon nanotube (CNT)-based membranes, have tremendous potential for wastewater treatment and water purification because of their excellent water permeability and selectivity, as well as their electrochemically enhanced performance (e.g., improved antifouling ability). However, it remains challenging to prepare CNT membranes with high structural stability and high electrical conductivity. In this study, a highly electroconductive and structurally stable polyphenylene/CNT (PP/CNT) composite membrane was prepared by electropolymerizing biphenyl on a CNT hollow fiber membrane. The PP/CNT membrane showed 3.4 and 5.0 times higher electrical conductivity than pure CNT and poly(vinyl alcohol)/CNT (PVA/CNT) membranes, respectively. The structural stability of the membrane was superior to that of the pure CNT membrane and comparable to that of the PVA/CNT membrane. The membrane fouling was significantly alleviated under an electrical assistance of −2.0 V, with a flux loss of only 11.7% after 5 h filtration of humic acid, which is significantly lower than those of PP/CNT membranes without electro-assistance (56.8%) and commercial polyvinylidene fluoride (PVDF) membranes (64.1%). Additionally, the rejection of negatively charged pollutants (humic acid and sodium alginate) was improved by the enhanced electrostatic repulsion. After four consecutive filtration-cleaning cycle tests, the flux recovery rate after backwashing reached 97.2%, which was much higher than those of electricity-free PP/CNT membranes (67.0%) and commercial PVDF membranes (61.1%). This study offers insights into the preparation of stable conductive membranes for membrane fouling control in potential water treatment applications.

关键词： Polyphenylene CNTs Membrane Electro-assistance Membrane fouling mitigation

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Uptrend of the Western North Pacific Tropical Cyclone Outflow Height during 1959-2021

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Journal of Meteorological Research 2024年第2期38卷 339-350页

作者： Yuan SUN Zhihao FENG wei ZHONG Panmao ZHAI Yanluan LIN shuo LYU College of Advanced Interdisciplinary Studies National University of Defense TechnologyNanjing 211100 College of Meteorology and Oceanography National University of Defense TechnologyChangsha 410073 State Key Laboratory of Severe Weather Chinese Academy of Meteorological SciencesChina Meteorological AdministrationBeijing 100081 Ministry of Education Key Laboratory for Earth System Modeling Department of Earth System ScienceTsinghua UniversityBeijing 100084

Relatively little is known about the impact of global warming on the tropical cyclone(TC)outflow,despite its large contribution to TC *** this study,based on the International Best Track Archive for Climate Stewardship(IBTrACS)dataset and ERA5 reanalysis data,we show that the TC outflow height has risen significantly(48.20±22.18 m decades-1)in the past decades(1959-2021)over the western North Pacific,and the rising trend tends to be sharper for stronger TCs(the uptrend of severe typhoon is 61.09±40.92 m decades-1).This rising trend of the outflow height explains the contradiction between the decrease trend of the TC outflow temperature and the increase trend of the atmospheric troposphere ***,possible contribution of the TC outflow height uptrend to TC intensity has also been *** results show that the rise of outflow height leads to the decrease of outflow temperature,and thus an increased difference between underlying sea surface temperature(SST)and TC outflow temperature,which eventually favors the increase of TC intensity.

关键词： tropical cyclone outflow height global warming western North Pacific

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Repeated inoculation with rumen fluid accelerates the rumen bacterial transition with no benefit on production performance in postpartum Holstein dairy cows

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Journal of Animal Science and Biotechnology 2024年第2期15卷 795-811页

作者： Fanlin Kong Feiran Wang Yijia Zhang shuo Wang wei Wang Shengli Li Beijing Engineering Technology Research Center of Raw Milk Quality and Safety Control State Key Laboratory of Animal NutritionDepartment of Animal Nutrition and Feed ScienceCollege of Animal Science and TechnologyChina Agricultural UniversityBeijing 100193PR China College of Veterinary Medicine Nanjing Agricultural UniversityNanjingJiangsu 210095PR China

Background The dairy cow’s postpartum period is characterized by dramatic physiological changes,therefore imposing severe challenges on the animal for maintaining health and milk *** dynamics of the ruminal microbiota are also tremendous and may play a crucial role in lactation *** aim to investigate the potential benefits of early microbial intervention by fresh rumen microbiota transplantation(RMT)and sterile RMT in postpartum dairy *** fistulated peak-lactation dairy cows were selected to be the donors for rumen fluid *** postpartum cows were divided into 3 groups as the transplantation receptors respectively receiving 10 L fresh rumen fluid(FR),10 L sterile rumen fluid(SR),or 10 L saline(CON)during 3 d after *** Production performance,plasma indices,plasma lipidome,ruminal microbiome,and liver transcriptome were *** fresh and sterile RMT,we found that the molar proportion of propionic acid was increased on d 7 in the FR and SR groups and the bacterial composition was also significantly changed when compared with the CON group.A similarity analysis showed that the similarities between the CON group and FR or SR group on d 7 were 48.40%or 47.85%,whereas the similarities between microbiota on d 7 and 21 in the FR and SR groups were 68.34%or 66.85%.Dry matter intake and feed efficiency were not affected by ***β-hydroxybutyrate concentration in the FR group was decreased and significantly different lipids mainly included phosphatidylcholine and lysophosphatidylcholine containing polyunsaturated fatty *** transcriptomics analysis indicated acutephase response pathways were upregulated in the SR *** Our study suggests that RMT can shorten the transition process of the ruminal microbiota of postpartum dairy cows with no benefit on dry matter intake or feed *** with rumen fluid may not be a useful approach to promote the recovery of postpartum dairy cows.

关键词： Dairy cow Lipidomics Liver Rumen microbiota transplantation Transition period

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Dual-additives enable stable electrode-electrolyte interfaces for long life Li-SPAN batteries

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Chinese Chemical Letters 2024年第5期35卷 459-465页

作者： Zhenqiang Guo Huicong Yang Qian wei Shengjun Xu Guangjian Hu shuo Bai Feng Li School of Materials Science and Engineering University of Science and Technology of ChinaShenyang 110016China Shenyang National Laboratory for Materials Science Institute of Metal ResearchChinese Academy of SciencesShenyang 110016China Department of Biochemistry and Molecular Biology College of Life ScienceChina Medical UniversityShenyang 110122China Dalian National Laboratory for Clean Energy Dalian 116023China Shenyang King Power Technology Co. Ltd.Shenyang 110016China

Sulfurized polyacrylonitrile(SPAN)is proposed as a promising cathode material for lithium sulfur ***,the continuous side reactions at the electrolyte-electrode interfaces as well as the slow redox kinetics of SPAN cathode deteriorate the electrochemical *** this study,an electrolyte with dual-additives comprising 2-fluoropyridine(2-FP)and lithium difluorobis(oxalato)phosphate(LiDFBOP)was used to improve the performance of Li||SPAN cells.2-FP has a lower lowest occupied molecular orbital energy than that of the solvents in the electrolyte,leading to its prior reduction.A LiF-rich film can be formed on the electrode,effectively improving the stability of the electrolyte-electrode interfaces and prolonging the ***,LiDFBOP could form an electrolyte-electrode interface film containing a large amount of Li_(x)PO_(y)F_(z) species,compensating for the kinetic deterioration caused by the lower ionic conductive of LiF formed at the electrolyte-electrode ***,an electrode-interface film with good chemical stability and high Li^(+) transport was established by LiF and Li_(x)PO_(y)F_(z)-rich *** Li||SPAN cell with the electrolyte containing dual-additives demonstrates an excellent capacity retention of 97.5%after 200 cycles at 1.0 C,25℃,comparing to 56.2%capacity retention without ***,the rate capacities of cells with dual-additives can reach 1128.1 mAh/g at 5 C,comparing to only 813.5 mAh/g using electrolyte without *** results shown that the dual-additives in electrolyte provide a promising strategy for practical application of lithium sulfur batteries with SPAN cathodes.

关键词： Sulfurized polyacrylonitrile Lithium metal Interface film 2-Fluoropyridine Difluorobis(oxalato)phosphate

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The coordinated evolution of ecological environment,public service,and tourism economy along the Silk Road Economic Belt,using the Dual-Carbon Targets

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Chinese Journal of Population,Resources and Environment 2024年第1期22卷 34-47页

作者： shuo Yang wei Guo Tianjun Xu Tongtong Liu School of Economic and Management Yanshan UniversityQinhuangdao 066004China Regional Economic Development Center Yanshan UniversityQinhuangdao 066004China

Carbon peak and carbon neutrality(dual-carbon)are important targets for the international response to climate *** Silk Road Economic Belt is a strategic resource region and is important for future ecological environment and tourism *** on the“dual-carbon”targets,the Single index quantification,Multiple index synthesis,and Poly-criteria integration evaluation model were used in this study to measure the coordinated development index of the ecological environment,public service,and tourism economy along the Silk Road Economic Belt and to analyze its spatial and temporal ***,it explores the dynamic evolution and development trend of the three systems using the Kernel Density and Grey Markov Prediction *** results show that the coordinated development index along this region needs to be improved during the study ***,the coordinated development index of the Southwest region is relatively higher than that of the Northwest *** the development trend of the three systems,all of them develop in a stable manner;however,the tourism economy system is easily affected by external *** coordinated development index of the three systems changes dynamically and tends to be in a good state of *** is a certain spatial and temporal *** gravity center of the coordinated development index has been in the Southwest *** the forecast period,the coordinated development index along this region will improve significantly,while insufficient and unbalanced development will continue.

关键词： Dual-carbon Ecological environment Public services Tourist economy Silk Road Economic Belt

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