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Identification of a novel p.R1443W mutation in RP1 gene associated with retinitis pigmentosa sine pigmento

International Journal of Ophthalmology(English edition) 2013年第4期6卷 430-435页

作者： Li Ma Xun-Lun Sheng Hui-Ping Li Fang-Xia Zhang ya-ni liu Wei-ning Rong Jian-Ling Zhang ningxia Eye Hospital People's Hospital of ningxia Hui Autonomous Region

AIM:To screen mutations in the retinitis pigmentosa 1(RP1) gene and the rhodopsin(RHO) gene in Chinese patients with retinitis pigmentosa sine pigmento(RPSP)and describe the genotype-phenotype relationship of the mutations.·METHODS:Twenty affected,unrelated Chinese individuals with RPSP(4 autosomal dominant RPSP,12autosomal recessive RPSP and 4 unknown inheritance pattern) were recruited between 2009 and *** clinical features were determined by complete ophthalmologic *** chain reaction(PCR) and direct DNA sequencing were used to screen the entire coding region and splice junctions of the RP1gene and the RHO *** cosegregation analysis and population frequency studies were performed for patients with identified mutations.·RESULTS:Five variants in the RP1 gene and one in the RHO gene were detected in 20 *** missense changes(rs444772,rs446227,rs414352,rs441800) and one non-coding variant(rs56340615) were common SNPs and none of them showed a significant relationship with RPSP.A missense mutation p.R1443W was identified in the RP1 gene in three affected individuals from a family with autosomal dominant RPSP and was found to cosegregate with the phenotype in this family,suggestive of *** addition,population frequency analysis showed the p.R1443W mutation was absent in 300 healthy controls.·CONCLUSION:The identification of p.R1443W mutationcosegregating in a family with autosomal dominant RPSP highlights an atypical phenotype of the RP1 gene mutation,while RHO gene is not associated with the pathogenesis of RPSP in this *** our knowledge,this is the fist mutation identified to associate with RPSP.

关键词： retinitis pigmentosa sine pigmento RP1 and RHO gene gene mutation

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Selective photosynthesis of Z-olefins through crystalline metal-organic cage-initiated expeditious cascade reactions

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Carbon Energy 2024年第3期6卷 118-131页

作者： Jia-ni Lu Yunze Huang Yuan-Sheng Xia Long-Zhang Dong Lei Zhang Jing-Jing liu Lan-Gui Xie Jiang liu ya-Qian Lan Jiangsu Key Laboratory of New Power Batteries School of Chemistry and Materials ScienceJiangsu Collaborative Innovation Centre of Biomedical Functional MaterialsNanjing Normal UniversityNanjingChina School of Chemistry South China Normal UniversityGuangzhouChina

The semi-hydrogenation of alkyne to form Z-olefins with high conversion and high selectivity is still a huge challenge in the chemical ***,flammable and explosive hydrogen as the common hydrogen source of this reaction increases the cost and danger of industrial ***,we connect the photocatalytic hydrogen evolution reaction and the semihydrogenation reaction of alkynes in series and successfully realize the high selective production of Z-alkenes using low-cost,safe,and green water as the proton *** the cascade reaction,a series of isomorphic metal–organic cage catalysts(Co_(x)Zn_(8−x)L_(6),x=0,3,4,5,and 8)are designed and synthesized to improve the yield of the photocatalytic hydrogen *** them,Co_(5)Zn_(3)L_(6) shows the highest photocatalytic activity,with a H_(2) generation rate of 8.81 mmol g^(−1) h^(−1).Then,Co_(5)Zn_(3)L_(6) is further applied in the above tandem reaction to efficiently reduce alkynes to Z-alkenes under ambient conditions,which can reach high conversion of>98%and high selectivity of>99%,and maintain original catalytic activity after multiple ***“one-pot”tandem reaction can achieve a highly selective and safe stepwise conversion from water into hydrogen into Z-olefins under mild reaction conditions.

关键词： alkyne semi-hydrogenation hydrogen evolution reaction metal-organic cages photocatalysis Z-olefins

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Bulging Distortion of Austenitic Stainless Steel Sheet on the Partially Penetrated Side of Non-Penetration Lap Laser Welding Joint

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Chinese Journal of Mechanical Engineering 2024年第1期37卷 286-295页

作者： Chengwu yao Enze liu Jiaming ni Binying nie Shanghai Key Laboratory of Materials Laser Processing and Modification School of Material Science and EngineeringShanghai Jiao Tong UniversityShanghai 200240China Shanghai Aerospace Precision Machinery Institute Shanghai 201600China Chemical and Biological Engineering College Yichun UniversityYichun 336000China

Non-penetration laser welding of lap joints in austenitic stainless steel sheets is commonly preferred in fields where the surface quality is of utmost ***,the application of non-penetration welded austenitic stainless steel parts is limited owing to the micro bulging distortion that occurs on the back surface of the partial penetration *** this paper,non-penetration lap laser welding experiments,were conducted on galvanized and SUS304 austenitic stainless steel plates using a fiber laser,to investigate the mechanism of bulging distortion.A comparative experiment of DC01 galvanized steel-Q235 carbon steel lap laser welding was carried out,and the deflection and distortion profile of partially penetrated side of the sheets were measured using a noncontact laser *** addition,the cold-rolled SUS304 was subjected to heat holding at different temperatures and water quenching after bending to characterize its microstructure under tensile and compressive *** results show that,during the heating stage of the thermal cycle of laser lap welding,the partial penetration side of the SUS304 steel sheet generates compressive stress,which extrudes the material in the heat-affected zone to the outside of the back of the SUS304 steel sheet,thereby forming a *** findings of these experiments can be of great value for controlling the distortion of the partial penetrated side of austenitic stainless steel sheet during laser non-penetration lap welding.

关键词： Non-penetration lap laser welding Bulging distortion Austenitic stainless steel Compressive stress Tension stress

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Therapeutic Effect of Rhodopseudomonas palustris-Bioconverted Extract of Lycium barbarum on Rats with Lead Exposure

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Biomedical and Environmental Sciences 2024年第4期37卷 440-444页

作者： yaNG Hong SUN Yi CHAI Shu Tong LIANG yan Hui liu ya ni yaNG Guan E School of Pharmacy Shanxi Medical UniversityTaiyuan 030001ShanxiChina

Lead is a ubiquitous pollutant and Pb pollution is a global public health *** has been reported to induce multiple adverse effects,including reproductive toxicity,neurotoxicity,carcinogenicity,nephrotoxicity,immunotoxicity,and hypertension[1].The traditional medical treatment available for Pb poisoning is chelation,which can save lives in individuals with very high blood Pb *** commonly used chelating agents include CaNa2EDTA and meso-2,3-dimercaptosuccinic ***,chelation therapy has strong short-term effects on the overall long-term management of Pb exposure.

关键词： converted chelation chelating

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FGFR2 mutation in a Chinese family with unusual Crouzon syndrome

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International Journal of Ophthalmology(English edition) 2016年第10期9卷 1403-1408页

作者： Zi-Li Li Xue Chen Wen-Juan Zhuang Wei Zhao ya-ni liu Fang-Xia Zhang Ruo-Shui Ha Jin-Hua Wu Chen Zhao Xun-Lun Sheng ningxia Eye Hospital People Hospital of Ningxia Hui Autonomous Region (First Affiliated Hospital of Northwest University for Nationalities) Yinchuan 750001 Ningxia Hui Autonomous Region China Department of Ophthalmology the First Affiliated Hospital of Nanjing Medical University State Key Laboratory of Reproductive Medicine Nanjing 210029 Jiangsu ProvinceChina Central Laboratory of ningxia Medical University Yinchuan 750000 Ningxia Hui Autonomous Region China Department of Radiology People Hospital of Ningxia Hui Autonomous Region Yinchuan 750000 Ningxia Hui Autonomous Region China

AIM：To describe the clinical characteristics with genetic lesions in a Chinese family with Crouzon syndrome. METHODS： All five patients from this family were included and received comprehensive ophthalmic and systemic *** sequencing of the FGFR2 gene was employed for mutation *** structure analysis was applied to analyze the structural changes associated with the substitution. RESULTS： All patients presented typical Crouzon features,including short stature,craniosynostosis,mandibular prognathism,shallow orbits with proptosis,and *** phenotypic diversities were *** optic nerves were exclusively detected in the proband and her *** magnetic resonance imaging implied a cystic lesion in her sellar and third ventricular regions.A missense mutation,FGFR2 ***342 Trp,was found as disease *** substitution would generate conformational changes in the extracellular Ig-III domain of the FGFR-2 protein,thus altering its physical and biological ***： We describe the clinical presentations and genotypic lesions in a Chinese family with Crouzon *** intrafamilial phenotypic varieties in this family suggest that other genetic modifiers may also play a role in the pathogenesis of Crouzon syndrome.

关键词： Crouzon syndrome familial cases phenotypic diversity FGFR2 mutation

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Fixed-Time Anti-Disturbance Average-Tracking for Multi-Agent Systems Without Velocity Measurements

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Journal of Systems Science & Complexity 2024年第3期37卷 985-1002页

作者： LI Yuling liu Chenglin ZHANG ya CHEN yangyang Key Laboratory of Advanced Process Control for Light Industry(Ministry of Education) Institute of AutomationJiangnan UniversityWuci 214122China School of Automation Southeast UniversityNanjing 210096China

In this paper,the authors tackle a fixed-time average-tracking problem for multi-agent systems which do not require velocity measurements and are affected by external mismatched and matched *** objective requires the considered agents with second-order dynamics to follow the average value of several time-varying reference signals,each of which is acquired by only one *** of all,the average value is estimated by using a novel distributed average estimator in a fixed ***,an original observer is created to provide estimated values in a fixed time for external mismatched and matched disturbances as well as unmeasured velocities of ***,the authors propose a novel fixed-time anti-disturbance average-tracking protocol with the aid of the observer and a sliding mode *** the end,the feasibility of theoretical analysis is illustrated by some simulation experiments.

关键词： Average-tracking control fixed-time algorithm fixed-time observer mismatched distur-bances multi-agent systems velocity measurements

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Ecological Network Resilience of Shiyang River Basin: An Arid Inland Watershed of Northwest China

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Chinese Geographical Science 2024年第5期34卷 951-966页

作者： HE yangxi liu Chunfang ni Bowen LIAN Hugang School of Management Northwest Normal UniversityLanzhou 730070China Research Center of Land Use and Comprehensive Improvement Engineering of Gansu Province Lanzhou 730070China

Due to the uncertainties posed by climate change,resilience has become an increasingly important variable for evaluating regional ecosystem *** assessment of Ecological Network Resilience(ENR)is crucial for establishing mitigation strategies and sustainable socioeconomic development in arid *** River Basin is an arid watershed in Northwest China with complex characteristics,its ENR and spatial differentiation characteristics in 2020 were investigated in this work based on the Complex Adaptive System(CAS)*** results indicated that the mean Ecological Network Resilience Index(ENRI)value for the Shiyang River Basin was 0.390 in 2020,and the mean values in the southern mountainous,middle oasis,and northern desert regions of the basin were 0.598,0.461,and 0.237,respectively,demonstrating evident spatial ***,the ENR of the basin exhibited distinct distribution characteristics across different dimension,whereas the trend of the integrated ENR of the basin was consistent with that of elemental,structural,and functional resilience,indicating the constructed three-region ENR model based on the logical relationship of element-structure-function was suitable for evaluation of the ENR in arid inland river ***,strategies for enhancing regional ENR were proposed from the perspective of adapting to climate change.

关键词： Ecological Network Resilience(ENR) Complex Adaptive System(CAS) element-structure-function Shiyang River Basin Northwest China

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Solution-processed rare-earth thulium oxide with high permittivity for low-voltage transistor and inverters applications

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Journal of Rare Earths 2024年第8期42卷 1604-1609,I0007页

作者： Daiming liu Fei Wang Yongtao Zhang ya'nan Ding Shandong Engineering Laboratory for Preparation and Application of High-performance Carbon-Materials College of Electromechanical EngineeringQingdao University of Science&TechnologyQingdao266061China College of Electronic and Information Engineering Shandong University of Science and TechnologyQingdao266590China

The use of high-permittivity(high-k) thin films as gate dielectrics is essential in the development of lowpower electronics,In this work,rare-earth thulium oxide(Tm_(2)O_(3)) thin films were prepared by a facile solution process and annealed at various temperatures from 400 to 700℃.The evolution of the physical and dielectric properties of Tm_(2)O_(3) thin film with annealing temperature was *** is demonstrated that the Tm_(2)O_(3) thin film annealed at 600℃ exhibits the optimal performance,including a low leakage current of 3×10^(-10) A/cm^(2),a large areal capacitance of 250 nF/cm^(2) at 100 Hz,and a high permittivity value of *** Tm_(2)O_(3) thin film as a gate insulator was integrated into the thin film transistor(TFT) employing In_(2)O_(3)-based semiconducting *** In_(2)O_(3) TFT with 600℃-annealed Tm_(2)O_(3) dielectric exhibits the superior perfo rmance,with a high I_(on)/I_(off) of 1.65×10^(7),a small subthre shold swing(SS) value of 0.2 V/dec,a V_(TH) of+1.8 V,and a mobility of 1.68 cm^(2)/(V·s).Furthermore,an inverter constructed by connecting the TFT with a resistor exhibits full-swing *** work provides a facile and appealing method for preparing the high-k Tm_(2)O_(3) thin films as alternative gate dielectrics with the potential for use in low-power electronics and logic circuit applications.

关键词： Rare earths Tm_(2)O_(3) Dielectric Transistor Inverter

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Development and validation of a novel non-invasive test for diagnosing nonalcoholic fatty liver disease in Chinese children

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World Journal of Pediatrics 2024年第4期20卷 413-421页

作者： Zhe-Wen Qin Qian-Nan Ren Hong-Xi Zhang ya-Ru liu Ke Huang Wei Wu Guan-Ping Dong yan ni Jun-Fen Fu Division of Endocrinology National Clinical Research Center for Child HealthNational Children’s Regional Medical CenterThe Children’s HospitalDepartment of EndocrinologyNational Clinical Research Center for Child HealthNational Children’s Regional Medical CenterThe Children’s HospitalZhejiang University School of MedicineHangzhou310052China

With the exploding prevalence of obesity,many children are at risk of developing nonalcoholic fatty liver *** anthropometric and laboratory parameters,our study aimed to develop a model to quantitatively evaluate liver fat content(LFC)in children with *** A well-characterized cohort of 181 children between 5 and 16 years of age were recruited to the study in the Endocrinology Department as the derivation *** external validation cohort comprised 77 *** assessment of liver fat content was performed using proton magnetic resonance *** and laboratory metrics were measured in all subjects.B-ultrasound examination was carried out in the external validation *** Kruskal-Wallis test,Spearman bivariate correlation analyses,univariable linear regressions and multivariable linear regression were used to build the optimal predictive *** The model was based on indicators including alanine aminotransferase,homeostasis model assessment of insulin resistance,triglycerides,waist circumference and Tanner *** adjusted R^(2)of the model was 0.589,which presented high sensitivity and specificity both in internal[sensitivity of 0.824,specificity of 0.900,area under curve(AUC)of 0.900 with a 95%confidence interval:0.783–1.000]and external validation(sensitivity of 0.918 and specificity of 0.821,AUC of 0.901 with a 95%confidence interval:0.818–0.984).Conclusions Our model based on five clinical indicators was simple,non-invasive,and inexpensive;it had high sensitivity and specificity in predicting LFC in ***,it may be useful for identifying children with obesity who are at risk for developing nonalcoholic fatty liver disease.

关键词： Biochemical markers Children Magnetic resonance spectroscopy Nonalcoholic fatty liver disease Pediatric obesity

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On a Flexible Loan Repayment Method Depending on Borrower's Asset with an Early Termination Clause

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Acta Mathematicae Applicatae Sinica 2024年第2期40卷 557-567页

作者： Jin LIANG Jia-qi MAO Zhao-ya liu School of Mathematical Sciences Tongji UniversityShanghai 200092China

Aiming at the problem that the asset’s fluctuation influences the borrower’s repayment ability,a loan with a new and flexible repayment method is designed,which depends on the asset value of the *** repayment method can reduce the loan default probability,but it causes the uncertainty of the pay off *** the repayment term is related to the regular repayment amount in this method,a boundary for the regular repayment amount is set up in order to avoid too long repayment *** will balance the benefit of borrowers and lenders and improve the applicability of this *** establishing a mathematical model of the residual value of the loan,this model can be transformed into an initial-boundary problem of a partial differential *** analytic solution and the expected time to pay off the loan are ***,numerical analysis are shown.

关键词： flexible repayment method repayment ratio early termination clause expected pay off time

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