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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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Natural vibration and critical velocity of translating Timoshenko beam with non-homogeneous boundaries

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Applied Mathematics and Mechanics(English Edition) 2024年第9期45卷 1523-1538页

作者： Yanan LI Jieyu ding Hu ding Liqun CHEN Shanghai Institute of Applied Mathematics and Mechanics Shanghai Key Laboratory of Mechanics in Energy EngineeringShanghai Frontier Science Center of MechanoinformaticsSchool of Mechanics and Engineering ScienceShanghai UniversityShanghai 200072China School of Mathematics and Statistics Qingdao UniversityQingdao 266071Shandong ProvinceChina

In most practical engineering applications,the translating belt wraps around two fixed *** boundary conditions of the dynamic model are typically specified as simply supported or fixed *** this paper,non-homogeneous boundaries are introduced by the support *** the translating belt as the mechanical prototype,the vibration characteristics of translating Timoshenko beam models with nonhomogeneous boundaries are investigated for the first *** governing equations of Timoshenko beam are deduced by employing the generalized Hamilton's *** effects of parameters such as the radius of wheel and the length of belt on vibration characteristics including the equilibrium deformations,critical velocities,natural frequencies,and modes,are numerically calculated and *** numerical results indicate that the beam experiences deformation characterized by varying curvatures near the *** radii of the wheels play a pivotal role in determining the change in trend of the relative difference between two beam *** the results unearths that the relative difference in equilibrium deformations between the two beam models is more pronounced with smaller-sized *** the two wheels are of equal size,the critical velocities of both beam models reach their respective *** addition,the relative difference in natural frequencies between the two beam models exhibits nonlinear variation and can easily exceed 50%.Furthermore,as the axial velocities increase,the impact of non-homogeneous boundaries on modal shape of translating beam becomes more *** dealing with non-homogeneous boundaries is challenging,beam models with non-homogeneous boundaries are more sensitive to parameters,and the differences between the two types of beams undergo some interesting variations under the influence of non-homogeneous boundaries.

关键词： translating beam Timoshenko beam non-homogeneous boundary natural frequency critical velocity

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Whittle’s Index Based Sensor Scheduling for Multiprocess Systems Under DoS Attacks

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Journal of Systems Science & Complexity 2024年第1期37卷 329-350页

作者： CAI Zai ding Kemi Shenzhen Key Laboratory of Control Theory and Intelligent Systems Southern University of Science and TechnologyShenzhen 518055China.

In this paper,the authors consider how to design defensive countermeasures against DoS attacks for remote state estimation of multiprocess *** each system,a sensor will measure its state and transmits the data packets through an unreliable channel which is vulnerable to be jammed by an *** limited communication bandwidth,only a subset of sensors are allowed for data transmission,and how to select the optimal one to maximize the accuracy of remote state estimation is the focus of the proposed *** authors first formulate this problem as a Markov decision process and investigate the existence of optimal ***,the authors demonstrate the piecewise monotonicity structure of optimal *** the difficulty of obtaining an optimal policy of large-scale problems,the authors develop a suboptimal heuristic policy based on the aforementioned policy structure and Whittle’s ***,a closed form of the indices is derived in order to reduce implementation complexity of proposed scheduling policy and numerical examples are provided to illustrate the proposed developed results.

关键词： DoS attacks Kalman filter remote state estimation Whittle’s index.

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Deep structure of the Southeast Asian curved subduction system and its dynamic process

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Journal of Oceanology and Limnology 2024年第3期42卷 701-704页

作者： Weiwei ding Key Laboratory of Submarine Geoscience&Second Institute of Oceanography Ministry of Natural ResourcesHangzhou 310012China School of Oceanography Shanghai Jiao Tong UniversityShanghai 200030China

The deep structure,material circulation,and dynamic processes in the Southeast Asia have long been an elusive scientific puzzle due to the lack of systematic scientific observations and recognized theoretical *** on the deep seismic tomography using long-period natural earthquake data,in this study,the deep structure and material circulation of the curved subduction system in Southeast Asia was studied,and the dynamic processes since 100 million years ago was *** is pointed out that challenges still exist in the precise reconstruction of deep mantle structures of the study area,the influence of multi-stage subduction on deep material exchange and shallow magma activity,as well as the spatiotemporal evolution and coupling mechanism of multi-plate *** work should focus on high-resolution land-sea joint 3-D seismic tomography imaging of the curved subduction system in the Southeast Asia,combined with geochemical analysis and geodynamic modelling works.

关键词： curved subduction system deep structure material recycling dynamic process Southeast Asia

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A vertical track nonlinear energy sink

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Applied Mathematics and Mechanics(English Edition) 2024年第6期45卷 931-946页

作者： Meng LI Hu ding Shanghai Key Laboratory of Mechanics in Energy Engineering School of Mechanics and Engineering ScienceShanghai Frontier Science Center of MechanoinformaticsShanghai UniversityShanghai 200444China

Eliminating the effects of gravity and designing nonlinear energy sinks(NESs)that suppress vibration in the vertical direction is a challenging task with numerous damping *** this paper,the dynamic design of a vertical track nonlinear energy sink(VTNES)with zero linear stiffness in the vertical direction is proposed and realized for the first *** motion differential equations of the VTNES coupled with a linear oscillator(LO)are *** the strong nonlinearity considered of the VTNES,the steady-state response of the system is analyzed with the harmonic balance method(HBM),and the accuracy of the HBM is verified *** this basis,the VTNES prototype is manufactured,and its nonlinear stiffness is *** damping effect and dynamic characteristics of the VTNES are studied theoretically and *** results show that the VTNES has better damping effects when strong modulation responses(SMRs)***,even for small-amplitude vibration,the VTNES also has a good vibration suppression *** sum up,in order to suppress the vertical vibration,an NES is designed and developed,which can suppress the vertical vibration within certain ranges of the resonance frequency and the vibration intensity.

关键词： track nonlinear energy sink(TNES) vertical direction gravity nonlinear stiffness harmonic balance method(HBM) vibration control

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半夏白术天麻汤、通窍活血汤联合西药治疗急性脑梗死的疗效

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中国社区医师 2020年第33期36卷 106-107页

作者：丁远良如皋康慈医院中西医结合科江苏南通226576

目的:分析急性脑梗死患者接受半夏白术天麻汤、通窍活血汤联合西药的疗效。方法:2019年1-12月收治急性脑梗死患者60例,随机分为两组,各30例。对照组采用常规西药治疗;试验组采用半夏白术天麻汤、通窍活血汤联合西药治疗。比较两组神经... 详细信息

目的:分析急性脑梗死患者接受半夏白术天麻汤、通窍活血汤联合西药的疗效。方法:2019年1-12月收治急性脑梗死患者60例,随机分为两组,各30例。对照组采用常规西药治疗;试验组采用半夏白术天麻汤、通窍活血汤联合西药治疗。比较两组神经功能缺损评分量表(NHISS)、日常生活活动能力评分表(MBI)评分、临床疗效与生活质量评分。结果:试验组治疗总有效率高于对照组,差异有统计学意义(P<0.05);试验组治疗后NIHSS评分低于对照组,MBI及生活质量评分均高于对照组,差异有统计学意义(P<0.05)。结论:采用半夏白术天麻汤、通窍活血汤联合西药治疗急性脑梗死患者可有效提升临床疗效,从而更好地改善患者的生理状态与生活状态。

关键词：半夏白术天麻汤通窍活血汤西药急性脑梗死疗效

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红龙夏海汤加减联合西药治疗老年高血压患者的临床效果

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中外医学研究 2020年第24期18卷 126-128页

作者：丁远良如皋康慈医院江苏如皋226576

目的:探究老年高血压患者应用红龙夏海汤加减联合西药治疗的临床效果。方法:选取笔者所在医院2018年5月-2019年5月收治的老年高血压患者70例作为此次研究对象,以治疗方案的不同进行分组,即单一应用西药治疗的35例患者设为对照组,而应用... 详细信息

目的:探究老年高血压患者应用红龙夏海汤加减联合西药治疗的临床效果。方法:选取笔者所在医院2018年5月-2019年5月收治的老年高血压患者70例作为此次研究对象,以治疗方案的不同进行分组,即单一应用西药治疗的35例患者设为对照组,而应用红龙夏海汤加减联合西药治疗的35例患者设为观察组。观察两组治疗前后血压水平、中医证候积分变化情况与临床疗效。结果:治疗前,两组患者血压水平及中医证候积分基本一致,差异均无统计学意义(P>0.05);治疗后,两组患者血压水平及中医证候积分均下降,且观察组均低于对照组,差异有统计学意义(P<0.05);对照组与观察组治疗后总有效率分别为68.57%和91.43%,观察组总有效率更高,对比差异有统计学意义(P<0.05)。结论:在老年高血压患者中应用红龙夏海汤加减联合西药治疗,可显著改善患者的血压水平与中医证候积分,且疗效确切。

关键词：红龙夏海汤加减西药高血压血压水平中医证候积分

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A newly unraveled 13.6-year oscillation from GPS displacements and its potential implications for the dynamic reference frame

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Science China Earth Sciences 2024年第10期67卷 3204-3212页

作者： Hao ding Weiping JIANG School of Geodesy and Geomatics Hubei Luojia LaboratoryWuhan UniversityWuhan 430079China GNSS Research Center Wuhan UniversityWuhan 430079China

Numerous practical geodetic and geophysical applications necessitate precise measurements of GNSS displacements at the millimeter or sub-millimeter level. To attain such precision, it is imperative to identify and analyze the unidentified decadal signals inherent in the GPS displacements. In this research, we employ the optimal sequence estimation method to effectively detect an about 13.6-year oscillational signal with an excited amplitude of 3.6±1.2 mm in the U-components of the GPS displacements. It is noteworthy that this signal demonstrates a consistent spatial pattern characterized by the spherical harmonic Y_(2,-2). We conduct a comparative analysis with the 13.6-year oscillation observed in length-of-day variations(and geomagnetic records), finding that they are in reverse phase. After eliminating the Earth's external excitation sources through the utilization of two distinct in-situ hydrological records, we suggest that the 13.6-year GPS signal may come from the internal motions within the Earth. However, the specific excitation source and the detailed physical mechanism remain uncertain. Additionally, we develop a mathematical displacement model to explain the 13.6-year signal. Our findings indicate that this signal can result in displacements of up to 1.37 mm and velocity effects of 0.63 mm/yr(for U-component) at maximum. These results underscore the necessity of incorporating this 13.6-year signal into the construction and maintenance of a dynamic reference frame at the millimeter level.

关键词： GPS displacements Decadal changes 13.6-year oscillation Displacement model Dynamic reference frame

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Towards human-leveled vision systems

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Science China(Technological Sciences) 2024年第8期67卷 2331-2349页

作者： ding JianHao HUANG TieJun State Key Laboratory of Multimedia Information Processing School of Computer SciencePeking UniversityBeijing 100871China

The human visual system is a complex and interconnected network comprising billions of neurons. It plays an essential role in translating environmental light stimuli into information that guides and shapes human perception and action. Research on the visual system aims to uncover the underlying neural structure principles of human visual perception and their possible ***, there are two main approaches: biological system analysis and simulation, artificial intelligence models based on deep learning. Here we aim to discuss the two approaches to human-level vision systems. Deep learning has significantly impacted the field of vision with achievements in representation, modeling, and hardware design. However, there is still a significant gap between deep learning models and the human visual system in terms of scalability, transferability, and sustainability. The progress of the biological visual system can help fill the gap by further understanding the properties and functions of different components of the system. We take the efforts of reconstructing the retina as an example to illustrate that even if we are unable to replicate the visual system on a computer right now, we can still learn a lot by combining existing research outcomes in neuroscience. At the end of the paper, we suggest tracing back to gradually build visual systems from the computational counterpart of biological structures to achieve a human-level vision system in the future.

关键词： human-leveled vision system deep learning biological visual system

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Capturing metal clusters at the strong-interaction region to reverse Ostwald ripening

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Science China Materials 2024年第4期67卷 1363-1364页

作者： Shuheng Tian ding Ma Beijing National Laboratory for Molecular Sciences New Cornerstone Science LaboratoryCollege of Chemistry and Molecular EngineeringPeking UniversityBeijing 100871China

High-temperature interactions induce sintering of metal nanoparticles(NPs) on heterogeneous catalyst surfaces, leading to reduced surface area and active site loss through Ostwald ripening [1]. This irreversible deactivation necessitates costly catalyst replacement, notably impacting metals like copper in hydrogenation and reforming reactions [2].

关键词： catalyst reforming irreversible

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