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CGG repeat expansion in LOC642361/NUTM2B-AS1 typically presents as oculopharyngodistal myopathy

journal of Genetics and Genomics 2024年第2期51卷 184-196页

作者： Yan Shi Chunyan Cao Yiheng Zeng Yuanliang Ding Long chen Fuze Zheng Xuejiao chen Fanggui Zhou Xiefeng Yang jinjing Li Liuqing Xu Guorong Xu Minting Lin Hiroyuki Ishiura Shoji Tsuji Ning Wang Zhiqiang Wang Wan-jin chen Kang Yang Department of Neurology and Institute of Neurology of First Affiliated Hospital Institute of NeuroscienceFujian Key Laboratory of Molecular NeurologyFujian Medical UniversityFuzhouFujian 350005China Department of Neurology National Regional Medical CenterBinhai Campus of the First Affiliated HospitalFujian Medical UniversityFuzhouFujian 350212China The First Affiliated Hospital College of Clinical Medicine of Henan University of Science and TechnologyLuoyangHenan 471000China Department of Neurology Zhangzhou Municipal Hospital of Fujian Province and Zhangzhou Affiliated Hospital of Fujian Medical UniversityZhangzhouFujian 363000China Department of Neurology Jian'ou Municipal Hospital of Fujian ProvinceJian'ouFujian 353100China Department of Radiology First Affiliated Hospital of Fujian Medical UniversityFuzhouFujian 350005China Department of Neurology Graduate School of MedicineThe University of TokyoTokyo 113-8655Japan Department of Neurology Okayama University Graduate School of MedicineDentistry and Pharmaceutical SciencesOkayama 700-8558Japan Institute of Medical Genomics International University of Health and WelfareChiba 286-0048Japan

CGG repeat expansions in LOC642361/NUTM2B-AS1 have recently been identified as a cause of oculopharyngeal myopathy with ***,since only three patients from a single family were reported,it remains unknown whether their clinicopathological features are typical for CGG repeat expansions in LOC642361/***,using repeat-primed-polymerase chain reaction and long-read sequencing,we identify 12 individuals from 3 unrelated families with CGG repeat expansions in LOC642361/NUTM2B-AS1,typically presenting with oculopharyngodistal *** CGG repeat expansions range from 161 to 669 repeat *** of the patients present with ptosis,restricted eye movements,dysphagia,dysarthria,and diffuse limb muscle *** one patient shows T2-weighted hyperintensity in the cerebellar white matter surrounding the deep cerebellar nuclei on brain magnetic resonance *** biopsies from three patients show a myopathic pattern and rimmed *** of muscle biopsies suggest that CGG repeat expansions in LOC642361/NUTM2B-AS1 may deleteriously affect aggrephagic capacity,suggesting that RNA toxicity and mitochondrial dysfunction may contribute to *** study thus expands the phenotypic spectrum for the CGG repeat expansion of LOC642361/NUTM2B-AS1 and indicates that this genetic variant typically manifests as oculopharyngodistal myopathy with chronic myopathic changes with rimmed vacuoles and filamentous intranuclear inclusions in muscle fibers.

关键词： Oculopharyngeal myopathy with leukoencephalopathy Oculopharyngodistal myopathy CGG repeat expansion Rimmed vacuoles

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Clinical and genetic heterogeneity of adult polyglucosan body disease caused by GBE1 biallelic mutations in China

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Genes & Diseases 2024年第5期11卷 17-20页

作者： Yikun chen Yan Shi Yuan Gao Yan Hu Linying Zhou jingmei Hong Shirui Gan Xiang Lin Wanjin chen Guorong Xu jin He Department of Neurology and Institute of Neurology of First Affiliated Hospital Institute of Neuroscienceand Fujian Key Laboratory of Molecular NeurologyFujian Medical UniversityFuzhouFujian 350005China Fujian Key Laboratory of Molecular Neurology Institute of NeuroscienceFujian Medical UniversityFuzhouFujian 350005China Electron Microscopy Lab of Public Technology Service Center Fujian Medical UniversityFuzhouFujian 350005China

Adult polyglucosan body disease(APBD)is a rare and highly heterogeneous glycogen storage disorder due to biallelic variants in GBE1.1 Typical APBD presentations include gait abnormalities with polyneuropathy,leukodystrophy,neurogenic bladder,and mild cognitive *** diagnosis of APBD encompasses a large spectrum of conditions including axonal and demyelinating sensorimotor polyneuropathy,progressive spastic paraparesis,and leukodystrophies.

关键词： GBE bladder diagnosis

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Characterization and quantification of multi-field coupling in lithium-ion batteries under mechanical constraints

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journal of Energy Chemistry 2024年第8期95卷 364-379,I0009页

作者： Xue Cai Caiping Zhang Zeping chen Linjing Zhang Dirk Uwe Sauer Weihan Li National Active Distribution Network Technology Research Center Beijing Jiaotong UniversityBeijing 100044China Chair for Electrochemical Energy Conversion and Storage Systems Institute for Power Electronics and Electrical Drives(ISEA)RWTH Aachen UniversityCampus Boulevard 8952074 AachenGermany Center for Ageing Reliability and Lifetime Prediction of Electrochemical and Power Electronic Systems(CARL)Campus-Boulevard 8952074 AachenGermany juelich Aachen Research Alliance JARA-EnergyAachenGermany Helmholtz Institute Münster(HI MS) IEK-12Forschungszentrum JülichGermany

The safety and durability of lithium-ion batteries under mechanical constraints depend significantly on electrochemical,thermal,and mechanical fields in *** and quantifying the multi-field coupling behaviors requires interdisciplinary ***,we design experiments under mechanical constraints and introduce an in-situ analytical framework to clarify the complex interaction mechanisms and coupling degrees among multi-physics *** proposed analytical framework integrates the parameterization of equivalent models,in-situ mechanical analysis,and quantitative assessment of coupling *** results indicate that the significant impact of pressure on impedance at low temperatures results from the diffusion-controlled step,enhancing kinetics when external pressure,like 180 to 240 k Pa at 10℃,is *** diversity in control steps for the electrochemical reaction accounts for the varying impact of pressure on battery performance across different *** thermal expansion rate suggests that the swelling force varies by less than 1.60%per unit of elevated temperature during the lithiation *** introducing a composite metric,we quantify the coupling correlation and intensity between characteristic parameters and physical fields,uncovering the highest coupling degree in electrochemical-thermal *** results underscore the potential of analytical approaches in revealing the mechanisms of interaction among multi-fields,with the goal of enhancing battery performance and advancing battery management.

关键词： Lithium-ion battery Muti-field coupling Mechanical constraints Interaction mechanisms Quantitative analysis

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Adaptive neural network control of a 2-DOF helicopter system considering input constraints and global prescribed performance

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Science China(Information Sciences) 2024年第7期67卷 224-239页

作者： Zhijia ZHAO jiale WU Zhijie LIU We HE C.L.Philip chen School of Mechanical and Electrical Engineering Guangzhou University School of Intelligence Science and Technology and Key Laboratory of Intelligent Bionic Unmanned Systems of Ministry of Education University of Science and Technology Beijing School of Computer Science and Engineering South China University of Technology Pazhou Lab

In this study, an adaptive neural network(NN) control is proposed for nonlinear two-degree-offreedom(2-DOF) helicopter systems considering the input constraints and global prescribed ***, radial basis function NN(RBFNN) is employed to estimate the unknown dynamics of the helicopter system. Second, a smooth nonaffine function is exploited to approximate and address nonlinear constraint functions. Subsequently, a new prescribed function is proposed, and an original constrained error is transformed into an equivalent unconstrained error using the error transformation and barrier function transformation methods. The analysis of the established Lyapunov function proves that the controlled system is globally uniformly bounded. Finally, the simulation and experimental results on a constructed Quanser's test platform verify the rationality and feasibility of the proposed control.

关键词： adaptive NN control 2-DOF helicopter global prescribed performance input constraints

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Magnetic Co-doped 1D/2D structuredγ-Fe_(2)O_(3)/MoS_(2) effectively activated peroxymonosulfate for efficient abatement of bisphenol A via both radical and non-radical pathways

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Frontiers of Environmental Science & Engineering 2024年第3期18卷 143-159页

作者： junge Xu Dong Wang Die Hu Ziwei Zhang junhong chen Yingmu Wang Yifeng Zhang College of Civil Engineering Fuzhou UniversityFuzhou 350116China China Nuclear Power Engineering Co. Ltd.Shenzhen 518120China Department of Environmental&Resource Engineering Technical University of DenmarkLyngby DK-2800Denmark

Iron-based catalysts have been widely used to treat refractory organic pollutants in *** this paper,magnetic Co-γ-Fe_(2)O_(3)was synthesized by a facile tartaric acid-assisted hydrothermal method,and Co-γ-Fe_(2)O_(3)/MoS_(2) nanocomposite catalyst was obtained via in situ growth of MoS_(2) nanosheets on Co-γ-Fe_(2)O_(3)*** nanocomposite catalysts were used to decompose bisphenol A(BPA)by activating peroxymonosulfate(PMS).It was shown that only 0.15 g/L catalyst and 0.5 mmol/L PMS degraded 10 mg/L of BPA(99.3%within 10 min)in the pH range of 3–*** was activated due to redox cycling among the pairs Co(III)/Co(II),Fe(III)/Fe(II),and Mo(VI)/Mo(IV).Quenching experiments and electron paramagnetic resonance spectroscopy demonstrated that both radical and non-radical pathways were involved in BPA degradation,in which active radical sulfate radical and non-radical singlet oxygen were the main reactive oxygen *** intermediates were identified by liquid chromatography-coupled mass spectrometry,and three possible BPA degradation pathways were *** toxicity of several degradation intermediates was lower,and Co-γ-Fe_(2)O_(3)/MoS_(2) exhibited excellent reusability and could be magnetically recovered.

关键词： Magnetic Co-γ-Fe_(2)O_(3) /MoS_(2) Hydrothermal method Bisphenol A Degradation pathways Toxicity analysis

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j-family genes redundantly regulate flowering time and increase yield in soybean

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The Crop journal 2024年第3期12卷 944-949页

作者： Haiyang Li Zheng chen Fan Wang Hongli Xiang Shuangrong Liu Chuanjie Gou Chao Fang Liyu chen Tiantian Bu Fanjiang Kong Xiaohui Zhao Baohui Liu Xiaoya Lin Guangdong Key Laboratory of Plant Adaptation and Molecular Design/Guangzhou Key Laboratory of Crop Gene Editing/Innovative Center of Molecular Genetics and Evolution School of Life SciencesGuangzhou UniversityGuangzhou 510006GuangdongChina

Soybean(Glycine max)is a short-day crop whose flowering time is regulated by *** longjuvenile trait extends its vegetative phase and increases yield under short-day *** variation in j,the major locus controlling this trait,modulates flowering *** report that the three j-family genes influence soybean flowering time,with the triple mutant Guangzhou Mammoth-2 flowering late under short days by inhibiting transcription of E1-family genes.j-family genes offer promising allelic combinations for breeding.

关键词： Soybean Flowering time Yield j-family genes

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Construct a 3D microsphere of HMX/B/Al/PTFE to obtain the high energy and combustion reactivity

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Defence Technology（防务技术） 2024年第2期32卷 45-54页

作者： jian Wang jie chen Yaofeng Mao Yongjun Deng Wei Cao Fude Nie jun Wang Institute of Chemical Materials China Academy of Engineering PhysicsMianyang 621999PR China Shock and Vibration of Engineering Materials and Structures Key Lab of Sichuan Province Mianyang 621010PR China

Metal(aluminum and boron)based energetic materials have been wildly applied in various fields including aerospace,explosives and micro-devices due to their high energy ***,the low combustion efficiency and reactivity of metal fuels,especially boron(B),severely limit their practical ***,multi-component 3D microspheres of HMX/B/Al/PTFE(HBA)have been designed and successfully prepared by emulsion and solvent evaporation method to achieve superior energy and combustion *** reactivity and energy output of HBA are systematically measured by ignitionburning test,constant-volume explosion vessel system and bomb *** to the increased interfacial contact and reaction area,HBA shows higher flame propagation rate,faster pressurization rate and larger combustion heat of 29.95 cm/s,1077 kPa/s,and 6164.43 j/g,which is 1.5 times,3.5 times,and 1.03 times of the physical mixed counterpart(HBA-P).Meanwhile,HBA also shows enhanced energy output and reactivity than 3D microspheres of HMX/B/PTFE(HB)resulting from the high reactivity of *** reaction mechanism of 3D microspheres is comprehensively investigated through combustion emission spectral and thermal analysis(TG-DSC-MS).The superior reactivity and energy of HBA originate from the surface etching of fluorine to the inert shell(Al_(2)O_(3) and B_(2)O_(3))and the initiation effect of Al to *** work offers a promising approach to design and prepare high-performance energetic materials for the practical applications.

关键词： HMX/B/Al/PTFE 3D microspheres Surface etching Reaction mechanism

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Crosslinked solubilizer enables nitrate-enriched carbonate polymer electrolytes for stable,high-voltage lithium metal batteries

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Science Bulletin 2024年第2期69卷 209-217页

作者： Chuyang jing Kuan Dai Dong Liu Wenran Wang Libao chen Chunxiao Zhang Weifeng Wei State Key Laboratory of Powder Metallurgy Central South UniversityChangsha 410083China College of Materials and Chemical Engineering Hunan Institute of EngineeringXiangtan 411104China

High-voltage lithium metal batteries(LMBs)have been considered promising next-generation highenergy-density ***,commercial carbonate electrolytes can scarcely be employed in LMBs owing to their poor compatibility with metallic lithium.N,N-dimethylacrylamide(DMAA)-a crosslinkable solubilizer with a high Gutmann donor number-is employed to facilitate the dissolution of insoluble lithium nitrate(LiNO3)in carbonate-based electrolytes and to form gel polymer electrolytes(GPEs)through in situ *** Lit solvation structure of the GPEs is regulated using LiNO3 and DMAA,which suppresses the decomposition of LiPFe and facilitates the formation of an inorganic-rich solid electrolyte ***,the Coulombic efficiency(CE)of the LillCu cell assembled with a GPE increases to 98.5%at room temperature,and the high-voltage LillNCM622 cell achieves a capacity retention of 80.1%with a high CE of 99.5%after 400 *** bifunctional polymer electrolytes are anticipated to pave the way for next-generation high-voltage LMBs.

关键词： Crosslinked solubilizer Lithium nitrate Carbonate electrolytes Gel polymer electrolytes Lithium metal batteries

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Enhancement of low-temperature toughness of Fe-Mn-C-Al alloy by rare earth Ce-modified inclusions

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journal of Iron and Steel Research International 2024年第1期31卷 157-173页

作者： Guang-kai Yang Chang-ling Zhuang Yi-zhuang Li chen Hu Shao-bo Li College of Materials and Metallurgy Guizhou UniversityGuiyang550025GuizhouChina Department of Mechanical Engineering University of Hong KongHong Kong999077China The State Key Laboratory of Rolling and Automation Northeastern UniversityShenyang110819LiaoningChina State Key Laboratory of Public Big Data Guizhou UniversityGuiyang550025GuizhouChina

Fe-Mn-C-Al alloys have been recognized as promising materials for certain low-temperature applications due to their exceptional mechanical properties and ***,their limited low-temperature toughness restricts their large-scale applications in specific *** influence of trace amounts of rare earth cerium(Ce)on the low-temperature toughness of Fe-18Mn-0.6C-1.8Al alloys was *** addition of Ce effectively alters the inclu-sions in the alloy,transforming large-sized irregular inclusions into fine ellipsoidal rare earth *** leads to a significant reduction in both the proportion and average size of the inclusions,resulting in their effective dispersion throughout the matrix and improved cryogenic *** presence of Ce-containing inclusions within the matrix reduces stress concentration,thereby inhibiting microcrack formation and improving impact absorption ***-cally,the addition of rare earth Ce alters the fracture behavior of the material at room temperature and low temperature,changing from brittle cleavage fracture to a more ductile failure *** impact toughness of the Fe-Mn-C-Al alloy is significantly improved by the addition of 0.0048 wt.%Ce,particularly at-196℃where the impact toughness reaches 103.6 j/cm^(2),representing an impressive improvement of 87.3%.

关键词： Fe-Mn-C-Al alloy Rare earth Low-temperature toughness Inclusion Fracture

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A novel(Sm0.2Eu0.2Gd0.2Ho0.2Yb0.2)CrO3 high-entropy ceramic nanofiber as a negative temperature coefficient thermistor

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journal of Rare Earths 2024年第10期42卷 1937-1942页

作者： Weijun Zhao Meng Zhang Liyan Xue Kaixian Wang Fan Yang jiuping Zhong Heng chen School of Materials Sun Yat-sen University Xiamen Institute of Rare Earth Materials Haixi InstituteChinese Academy of Sciences Xiamen Key Laboratory of Rare Earth Photoelectric Functional Materials Xiamen Institute of Rare Earth MaterialsHaixi InstituteChinese Academy of Sciences Fujian Science & Technology Innovation Laboratory for Optoelectronic Information of China Advanced Energy Science and Technology Guangdong Laboratory Sichuan jcc Rare Earth Metals New Material Co. Ltd.

The electrical properties of high-entropy ceramics（HECs） have been extensively studied in recent years due to their unique structural characteristics and fascinating functional properties induced by entropy *** high-entropy(Sm0.2Eu0.2Gd0.2Ho0.2Yb0.2)CrO3（HE-RECrO3） nanofibers were prepared by electro *** work demonstrates that HE-RECrO3nanofibe rs were successfully synthesized at a low temperature（800℃）,which is approximately 400 ℃ lower than the temperatures at which chromate ceramics were synthesized via the sol-gel method and the solid-state reaction *** resistivity of HE-RECrO3nanofibers decreases exponentially with increasing temperature from 25 to600℃.The logarithm of the resistivity is linearly related to the inverse of the temperature,confirming the negative temperature coefficient property of *** B25/50value of the HERECrO3nanofibers reaches 4072 *** conclusion,HE-RECrO3nanofibers are expected to be potential candidates for negative-temperature-coefficient（NTC） thermistors.

关键词： Ceramic fiber materials High-entropy ceramics Negative temperature coefficient thermistors Rare earths

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