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Application of isoxanthopterin as a new pterin marker in the differential diagnosis of hyperphenylalaninemia

World Journal of Pediatrics 2019年第1期15卷 66-71页

作者： pei-zhong bao Jun Ye Lian-Shu Han Wen-Juan Qiu Hui-Wen Zhang Yong-Guo Yu Jian-Guo Wang Xue-Fan Gu Department of Endocrinology and Genetics Shanghai Institute of PediatricsXinhua HospitalShanghai Jiaotong University School of Medicine1665 Kong Jiang RoadShanghai 200092China

Background This study aimed to explore the value of applying a new pterin marker (isoxanthopterin) to the traditional urine pterin analysis to reduce the rate of mis-diagnosis of 6-pyruvoyltetrahydropterin synthase deficiency (PTPSD) and improve the accuracy of *** We compared the urine neopterin (N),biopterin (B),isoxanthopterin (Iso),B% and Iso% levels between patients with phenylalanine hydroxylase deficiency and those with PTPSD,and found the most specific pterin biomarkers by ROC analysis.A positive cut-off value of urine pterins was *** effect of combined Iso% + B + B% in reducing PTPSD mis-diagnosis was evaluated,and the different urine pterin levels in PTPSD and false PTPSD (FPTPSD) were *** concordance of PTPSD diagnosis by the new pterin scheme and gene mutation analysis was *** (1) Urinary B,B%,Iso and Iso% were significantly lower in PTPSD than those in phenylalanine hydroxylase-deficiency group (P < 0.01);(2) Iso%,B%,and B were the most specific markers;(3) The positive cut-off values of B,B%,Iso% for PTPSD were < 0.17 mmoL/moLCr,< 5.0%,and < 9.5%,respectively;(4) urinary B +B% + Iso% scheme significantly reduced the false-positive rate of PTPSD compared to traditional *** Iso% levels in FPTPSD group were higher than the ones in PTPSD group;(5) an accuracy of diagnosis for PTPSD was increased by 9-19% when Iso% was introduced to urinary pterin *** Iso% is helpful to reduce the rate of misdiagnosis of PTPSD in the diagnosis by urinary pterin analysis for hyperphenylalaninemias and improve the accuracy of *** approach is worthy of further development and increased utilization.

关键词： 6-Pyruvoyltetrahydropterin synthase Hyperphenylalaninemia Isoxanthopterin Phenylalanine hydroxylase Pterin

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Study on 6-DOF active vibration-isolation system of the ultra-precision turning lathe based on GA-BP-PID control for dynamic loads

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Advances in Manufacturing 2024年第1期12卷 33-60页

作者： Bo Wang zhong Jiang pei-Da Hu Department of Precision Instrument Tsinghua UniversityBeijing100084People’s Republic of China Institute of Machinery Manufacturing Technology China Academy of Engineering PhysicsMianyang621900SichuanPeople’s Republic of China

The vibration disturbance from an external environment affects the machining accuracy of ultra-precision machining *** active vibration-isolation systems(AVIS)have been developed based on static *** a vibration-isolation load changes dynamically during ultra-precision turning lathe machining,the system parameters change,and the efficiency of the active vibration-isolation system based on the traditional control strategy *** solve this problem,this paper proposes a vibration-isolation control strategy based on a genetic algorithm-back propagation neural network-PID control(GA-BP-PID),which can automatically adjust the control parameters according to the machining ***-isolation simulations and experiments based on passive vibration isolation,a PID algorithm,and the GA-BP-PID algorithm under dynamic load machining conditions were *** experimental results demonstrated that the active vibration-isolation control strategy designed in this study could effectively attenuate vibration disturbances in the external environment under dynamic load *** design is reasonable and feasible.

关键词： Ultra-precision diamond turning lathe Active vibration isolation Six degrees of freedom Dynamic load Genetic algorithm-back propagation neural network-PID(GA-BP-PID)control

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Long-term Evolution of Solar Activity and Prediction of the Following Solar Cycles

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Research in Astronomy and Astrophysics 2024年第3期24卷 194-204页

作者： pei-Xin Luo bao-Lin Tan National Astronomical Observatories Chinese Academy of SciencesBeijing 100101China School of Astronomy and Space Science University of Chinese Academy of SciencesBeijing 100049China

Solar activities have a great impact on modern high-tech systems,such as human aerospace activities,satellite communication and navigation,deep space exploration,and related scientific ***,studying the long-term evolution trend of solar activity and accurately predicting the future solar cycles are highly *** on the wavelet transform and empirical function fitting of the longest recorded data of the annual average relative sunspot number(ASN)series of 323 yr to date,this work decisively verifies the existence of the solar century cycles and confirms that its length is about 104.0 yr,and the magnitude has a slightly increasing trend on the timescale of several hundred *** on this long-term evolutionary trend,we predict solar cycles 25 and26 by using phase similar prediction *** for solar cycle 25,its maximum ASN will be about146.7±33.40,obviously stronger than solar cycle *** peak year will occur approximately in 2024,and the period will be about 11±1 *** for solar cycle 26,it will start around 2030,and reach its maximum between2035 and 2036,with maximum ASN of about 133.0±3.200,and period of about 10 yr.

关键词： Sun activity-Sun evolution-(Sun) sunspots-methods data analysis

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QDPR gene mutation and clinical follow-up in Chinese patients with dihydropteridine reductase deficiency

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World Journal of Pediatrics 2014年第3期10卷 219-226页

作者： De-Yun Lu Jun Ye Lian-Shu Han Wen-Juan Qiu Hui-Wen Zhang Jian-De Zhou pei-zhong bao Ya-Fen Zhang Xue-Fan Gu Department of Pediatric Endocrinology and Genetic Metabolism and Shanghai Institute for Pediatric Research Xinhua HospitalShanghai Jiaotong University School of MedicineShanghai 200092China

Background:This study aimed to investigate the mutation spectrum of the QDPR gene,to determine the effect of mutations on dihydropteridine reductase(DHPR)structure/function,to discuss the potential genotype-phenotype correlation,and to evaluate the clinical outcome of Chinese patients after ***:Nine DHPR-deficient patients were enrolled in this study and seven of them underwent neonatal *** gene mutations were analyzed and confi rmed by routine *** potential pathogenicity of missense variants was analyzed using Clustal X,PolyPhen program and Swiss-PDB Viewer 4.04_OSX software,*** clinical outcomes of the patients were evaluated after long-term ***:In 10 mutations of the 9 patients,4 were novel mutations(G20V,V86D,G130S and A175R),4 were reported by us previously,and 2 known mutations were identified.R221X was a hotspot mutation(27.7%)in our *** missense mutations probably had damage to *** patients in this series were treated with a good control of phenylalanine *** height and weight of the patients were normal at the age of 4 months to 7.5 *** patients,who underwent a neonatal screening and were treated early,showed a normal mental *** 2 patients diagnosed late,neurological symptoms were signifi cantly ***:The mutation spectrum of the QDPR gene is different in the Chinese *** mutations are related to severe *** determination of DHPR activity should be performed in patients with ***-defi cient patients who were treated below the age of 2 months may have a near normal mental development.

关键词： dihydropteridine reductase hyperphenylalaninaemia tetrahydrobiopterin

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Modelling and Temperature Control of Liquid Cooling Process for Lithium-Ion Battery

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Journal of Thermal Science 2024年第5期33卷 1794-1808页

作者： LI Guanru zhong Yangfan bao Chujin CHEN Yongping ZHANG Chengbin School of Energy and Environment Southeast UniversityNanjing 210096China Key Laboratory of Efficient Low-Carbon Energy Conversion and Utilization of Jiangsu Provincial Higher Education Institutions Suzhou University of Science and TechnologySuzhou 215009China Alibaba Cloud Computing Co.Ltd. Hangzhou 311121China Zhejiang Immertech Co.Ltd. Hangzhou 310000China

Efficient thermal management of lithium-ion battery,working under extremely rapid charging-discharging,is of widespread interest to avoid the battery degradation due to temperature rise,resulting in the enhanced ***,thermal management of lithium-ion battery has been performed via a liquid cooling theoretical model integrated with thermoelectric model of battery packs and single-phase heat *** to alleviate the battery temperature fluctuation by automatically manipulating the flow rate of working fluid,a nominal model-free controller,i.e.,fuzzy logic controller is *** optimized on-off controller based on pump speed optimization is introduced to serve as the comparative *** control simulations are conducted under regular operating and extreme operating conditions,and two controllers are applied to control battery temperature with proper intervals which is conducive to enhance the battery charge-discharge *** results indicate that,for any operating condition,the fuzzy logic controller shows excellence in terms of the tracking accuracy of set-point of battery *** to the establishment of fuzzy set and fuzzy behavioral rules,the battery temperature has been throughout maintained near the set point,and the temperature fluctuation amplitude is highly reduced,with better temperature control accuracy of~0.2℃(regular condition)and~0.5℃(extreme condition)compared with~1.1℃(regular condition)and~1.6℃(extreme condition)of optimized on-off *** in the case of extreme operating condition,the proposed optimized on-off controller manifests the hysteresis in temperature fluctuation,which is ascribed to the set of dead-band for the feedback *** simulation results cast new light on the utilization and development of model-free temperature controller for the thermal management of lithium-ion battery.

关键词： temperature control battery thermal management model-free controller fuzzy logic control

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Crystal structure,phase transitions,and thermodynamic properties of magnesium metavanadate(MgV_(2)O_(6))

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Journal of Magnesium and Alloys 2024年第4期12卷 1449-1460页

作者： Guishang pei Cheng Pan Dapeng zhong Junyi Xiang Xuewei Lv College of Materials Science and Engineering Chongqing UniversityChongqing 400044China Chongqing Key Laboratory of Vanadium-Titanium Metallurgy and Advanced Materials Chongqing UniversityChongqing 400044China Pangang Group Research Institute Co. Ltd.Panzhihua 617000China

As a promising anode material for magnesium ion rechargeable batteries,magnesium metavanadate(MgV_(2)O_(6))has attracted considerable research interest in recent years.A MgV_(2)O_(6)sample was synthesized via a facile solid-state reaction by multistep-firing stoichiometric mixtures of MgO and V2O5 powder under an air *** solid-state phase transition fromα-MgV_(2)O_(6)toβ-MgV_(2)O_(6)occurred at 841 K and the enthalpy change was 4.37±0.04 kJ/*** endothermic effect at 1014 K and the enthalpy change was 26.54±0.26 kJ/mol,which is related to the incongruent melting ofβ-MgV_(2)O_(6).In situ XRD was performed to investigate phase transition of the as-prepared MgV_(2)O_(6)at high *** cell parameters obtained by Rietveld refinement indicated that it crystallizes in a monoclinic system with the C2/m space group,and the lattice parameters of a=9.280 A°,b=3.501 A°,c=6.731 A°,β=111.76°.The solid-state phase transition fromα-MgV_(2)O_(6)toβ-MgV_(2)O_(6)was further studied by thermal kinetics,indicating that this process is controlled first by a fibril-like mechanism and then by a spherulitic-type mechanism with an increasing heating ***,the enthalpy change of MgV_(2)O_(6)at high temperatures was measured utilizing the drop calorimetry,heat capacity was calculated and given as:Cp=208.3+0.03583T-4809000T^(−2)(298-923 K)(J mol^(−1)K^(−1)),the high-temperature heat capacity can be used to calculate Gibbs free energy of MgV_(2)O_(6)at high temperatures.

关键词： MgV_(2)O_(6) Crystal structure Phase transitions Thermodynamic functions

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Permanent lining castable with low bulk density and thermal conductivity:bauxite castable for tundish obtained by adding pearlescent sand

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Journal of Iron and Steel Research International 2024年第3期31卷 634-646页

作者： Yi Wang Jian Yang bao Wang Hai-jun Zhang Juan Han pei-yan pei Wen-long Lv Jian-an Zhou The State Key Laboratory of Refractories and Metallurgy Wuhan University of Science and TechnologyWuhan 430081HubeiChina Key Laboratory for Ferrous Metallurgy and Resources Utilization of Ministry of Education Wuhan University of Science and TechnologyWuhan 430081HubeiChina Wuhan Research Institute of Metallurgical Construction MCCWuhan 430081HubeiChina

The low thermal conductivity and light mass of castables for tundish permanent linings are crucial for minimizing the heat loss of molten *** consideration of the low bulk density and thermal conductivity of pearlescent sand,the thermal insulation performance of castables was attempted to be improved by adding pearlescent *** sand was modified to prevent the strength of its porous structure from *** modification mechanism of pearlescent sand and the effect of pearlescent sand on the performance of bauxite castables were *** results suggested that the addition of the modified pearlescent sand significantly raised the apparent porosity and decreased the bulk density of bauxite *** 1000℃,the bulk density of more than 60%of the modified pearlescent sand-bauxite castable was only 2.03 g/cm^(3).The mechanical properties and thermal shock resistance of the modified pearlescent sand-bauxite castable were inferior to those of conventional bauxite castable but were adequate to meet the use conditions of casta-bles for tundish permanent *** high temperatures of 200-800℃,the thermal conductivity of more than 60%of the modified pearlescent sand-bauxite castable was smaller than that of conventional bauxite *** addition of the modified pearlescent sand can greatly reduce the thermal conductivity and bulk density of bauxite castable.

关键词： Pearlescent sand-Hydrophobic modification Bauxite castable Thermal insulation performance Mechanical property

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Effect of Mo and cold forging deformation on strength and ductility of cobalt-based alloy L605

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Journal of Iron and Steel Research International 2024年第7期31卷 1811-1823页

作者： zhong-lin Wang Zhi-hua Gong Quan Li Han-sheng bao School of Materials and Metallurgy Inner Mongolia University of Science and TechnologyBaotou 014017Inner MongoliaChina Institute of Special Steels Central Iron and Steel Research Institute Co.Ltd.Beijing 100081China

Mo element was added to cobalt-based alloy L605,and cold forging deformation was *** effects of the addition and cold forging deformation on the microstructure and mechanical properties of the alloy were studied by thermodynamic calculation,electron backscatter diffraction,transmission electron microscopy,and X-ray *** stacking fault energy(SFE)of the alloy decreased after the addition,and the formation of stacking faults and intersections were promoted to improve the strength and *** tensile strength of the alloy with Mo increased from 1190 to 1702 MPa after 24%cold deformation,producing significant work *** strengthening mechanism is strain-induced martensitic transformation(SIMT)and deformation *** alloy,combined with Mo and after 24%deformation,had both high strength and ductility in comparison with the original cobalt-based alloy *** is attributed to the lower SFE which caused the increase in stacking fault *** the tensile process,theε-hcp phase was easily generated at the stacking fault to reduce the stress concentration and increase the *** SIMT by adjusting the density of stacking faults can improve the mechanical properties of cobalt-based ***ε-hcp phase,the interaction between deformation twins and dislocations,and the interaction between e-hcp phases during cold forging deformation caused local stress concentration,lowering ductility and toughness.

关键词： Cobalt-based alloy-Stacking fault energy Strain-induced martensitic transformation Deformation twin Cold forging deformation

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mRNA vaccine technology for infectious diseases and beyond

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Science China(Life Sciences) 2024年第10期67卷 2267-2270页

作者： pei Hao Xiao Li Xuan Li Wu zhong Key Laboratory of Molecular Virology and Immunology Shanghai Institute of Immunity and InfectionChinese Academy of SciencesShanghai 200031China Changchun Veterinary Research Institute Chinese Academy of Agricultural SciencesChangchun 130122China Key Laboratory of Synthetic Biology CAS Center for Excellence in Molecular Plant SciencesInstitute of Plant Physiology and EcologyChinese Academy of SciencesShanghai 200032China National Engineering Research Center for the Emergency Drug Beijing Institute of Pharmacology and ToxicologyBeijing 100085China University of Chinese Academy of Sciences Beijing 100049China

The rise of mRNA vaccine technology Vaccines stand as one of the most significant medical advancements,playing a crucial role in the prevention of infectious diseases and the improvement of global *** triumph of smallpox vaccination stands as a towering achievement in the annals of healthcare,leading to the complete elimination of a oncedevastating disease and showcasing the efficacy of vaccination methods.

关键词： methods vaccination prevention

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Comparison of corrosion mechanisms of 510L low alloy steel treated by ACS and EPS techniques under various service environments

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Journal of Iron and Steel Research International 2024年第2期31卷 501-518页

作者： bao-sheng Liu Peng-fei Xu Shao-hua Zhang Yan-rui Li Li-ming Mou Jia-li Li Yue-zhong Zhang pei-mao Fu Fa-cai Shi College of Materials Science and Engineering Taiyuan University of Science and TechnologyTaiyuan030024ShanxiChina College of Chemical and Biological Engineering Taiyuan University of Science and TechnologyTaiyuan030024ShanxiChina Technical Center Taiyuan Iron and Steel(Group)Co.Ltd.Taiyuan030003ShanxiChina

The corrosion mechanism of 510L low alloy steel treated by acid-cleaned surface(ACS)and eco-pickled surface(EPS)techniques in three simulated solutions(S0:atmospheric environment;S1:soil environment;S2:industrial environment)and the influence of interaction between different corrosive anions on corrosion were *** results show that the total corrosion rates of samples in three simulated solutions were in order of S2>S0>S1,which is simultaneously correlated with initial corrosion dissolution processes as well as after the formation of corrosion ***3−will inhibit the initial corrosion owing to the formation of films,whereas HSO3−will accelerate the dissolutions of the matrix based on the synergistic action of HSO3−and Cl−.On the other hand,there is no significant difference in corrosion rates between the samples treated by ACS and EPS *** EPS technique that is safe,reusable and environmentally friendly can be further widely used in future work.

关键词： Corrosion mechanism Low alloy steel Simulated solution Surface technique Immersion time

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