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A different spectrum of DMD gene mutations in local Chinese patients with Duchenne/Becker muscular dystrophy

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Chinese Medical Journal 2006年第13期119卷 1079-1087页

作者： Ivan Fai-man Lo Kent Keung-san Lai tony ming-for tong Stephen Tak-sum Lam Clinical Genetic Service Department of HealthHong KongChina

Background Duchenne muscular dystrophy （DMD） and Becker muscular dystrophy （BMD） are X-linked recessive, allelic disorders. This study was conducted to look into the spectrum of DMD gene mutations in Hong Kong Chi... 详细信息

关键词： Duchenne muscular dystrophy Becker muscular dystrophy Chinese DMD mutation deletion duplication

Molecular insights into oil detachment from hydrophobic quartz surfaces in clay-hosted nanopores during steam-surfactant co-injection

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Petroleum Science 2024年第4期21卷 2457-2468页

作者： Ben-Jie-ming Liu Xuan-tong Lei Mohammadali Ahmadi Zhangxin Chen Department of Chemical and Petroleum Engineering University of Calgary Eastern Institute of Technology

Thermal recovery techniques for producing oil sands have substantial environmental impacts. Surfactants can efficiently improve thermal bitumen recovery and reduce the required amount of steam. Such a technique requires solid knowledge about the interaction mechanism between surfactants, bitumen,water, and rock at the nanoscale level. In particular, oil sands ores have extremely complex mineralogy as they contain many clay minerals(montmorillonite, illite, kaolinite). In this study, molecular dynamics simulation is carried out to elucidate the unclear mechanisms of clay minerals contributing to the bitumen recovery under a steam-anionic surfactant co-injection process. We found that the clay content significantly influenced an oil detachment process from hydrophobic quartz surfaces. Results reveal that the presence of montmorillonite, illite, and the siloxane surface of kaolinite in nanopores can enhance the oil detachment process from the hydrophobic surfaces because surfactant molecules have a stronger tendency to interact with bitumen and quartz. Conversely, the gibbsite surfaces of kaolinite curb the oil detachment process. Through interaction energy analysis, the siloxane surfaces of kaolinite result in the most straightforward oil detachment process. In addition, we found that the clay type presented in nanopores affected the wettability of the quartz surfaces. The quartz surfaces associated with the gibbsite surfaces of kaolinite show the strongest hydrophilicity. By comparing previous experimental findings with the results of molecular dynamics(MD) simulations, we observed consistent wetting characteristics. This alignment serves to validate the reliability of the simulation outcomes. The outcome of this paper makes up for the lack of knowledge of a surfactant-assisted bitumen recovery process and provides insights for further in-situ bitumen production engineering designs.

关键词： Clay minerals Bitumen Contact angle Interaction energy Surfactant Molecular dynamics

Topology optimization of modular structures with multiple assemblies and applications to airborne shelves

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Chinese Journal of Aeronautics 2024年第4期37卷 321-332页

作者： Jie WANG tong GAO ming LI Jihong ZHU Longlong SONG Weihong ZHANG State IJR Center of Aerospace Design and Additive Manufacturing Northwestern Polytechnical UniversityXi’an 710072China Beijing Specialized Machinery Institute Beijing 100143China

This work is devoted to the aeronautical application of topology optimization for modular structures with multiple assemblies that consist of repeated standard modules and optional *** kinds of structures are widely used owing to their transportability,reconfigurability,low manufacturing and service *** this work,the design of airborne shelves with modular structures characterized by the standard module configuration is formulated for the first time as a topology optimization problem of multiple assemblies and multiple load cases subjected to the volume *** is shown that the weighted compliance design of multiple assemblies is a compromising solution compared to the optimization result of each individual assembly of standard ***,the performance of optimized airborne shelves with the modular structures can effectively be ameliorated with the help of reinforcements.

关键词： Topology optimization Modular structures Multiple assemblies Standard modules Airborne shelves

A novel multi-resolution network for the open-circuit faults diagnosis of automatic ramming drive system

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Defence Technology（防务技术） 2024年第4期34卷 225-237页

作者： Liuxuan Wei Linfang Qian Manyi Wang minghao tong Yilin Jiang ming Li School of Mechanical Engineering Nanjing University of Science and TechnologyNanjing 210094JiangsuChina Northwest Institute of Mechanical and Electrical Engineering Xianyang 712099China

The open-circuit fault is one of the most common faults of the automatic ramming drive system(ARDS),and it can be categorized into the open-phase faults of Permanent Magnet Synchronous Motor(PMSM)and the open-circuit faults of Voltage Source Inverter(VSI). The stator current serves as a common indicator for detecting open-circuit faults. Due to the identical changes of the stator current between the open-phase faults in the PMSM and failures of double switches within the same leg of the VSI, this paper utilizes the zero-sequence voltage component as an additional diagnostic criterion to differentiate *** the variable conditions and substantial noise of the ARDS, a novel Multi-resolution Network(Mr Net) is proposed, which can extract multi-resolution perceptual information and enhance robustness to the noise. Meanwhile, a feature weighted layer is introduced to allocate higher weights to characteristics situated near the feature frequency. Both simulation and experiment results validate that the proposed fault diagnosis method can diagnose 25 types of open-circuit faults and achieve more than98.28% diagnostic accuracy. In addition, the experiment results also demonstrate that Mr Net has the capability of diagnosing the fault types accurately under the interference of noise signals(Laplace noise and Gaussian noise).

关键词： Fault diagnosis Deep learning Multi-scale convolution Open-circuit Convolutional neural network

Toward a Direct Measurement of the Cosmic Acceleration:The Pilot Observation of HI 21 cm Absorption Line at FAST

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Research in Astronomy and Astrophysics 2024年第7期24卷 47-55页

作者： Jiangang Kang Chang-Zhi Lu tong-Jie Zhang ming Zhu Institute for Frontiers in Astronomy and Astrophysics Beijing Normal UniversityBeijing 102206China School of Physics and Astronomy Beijing Normal UniversityBeijing 100875China National Astronomical Observatories Chinese Academy of SciencesBeijing 100101China

This study presents results on detecting neutral atomic hydrogen(H I)21 cm absorption in the spectrum of PKS PKS1413+13 at redshift z=*** observation was conducted by FAST,with a spectral resolution of10 Hz,using 10 minutes of observing *** global spectral profile is examined by modeling the absorption line using a single Gaussian function with a resolution of 10 kHz within a 2 MHz *** goal is to determine the rate of the latest cosmic acceleration by directly measuring the redshift evolution of the H I 21 cm absorption line with Hubble flow toward a common background quasar over a decade or longer time *** will serve as a detectable signal generated by the accelerated expansion of the Universe at redshift z<1,referred to as redshift drift z(5)or the SL *** measured H I gas column density in this DLA system is approximately equivalent to the initial observation value,considering uncertainties of the spin temperature of a spiral host *** high signal-to-noise ratio of 57,obtained at a 10 kHz resolution,strongly supports the feasibility of using the H I 21 cm absorption line in DLA systems to accurately measure the redshift drift rate at a precision level of around 10~(-10)per decade.

关键词： (cosmology)cosmological parameters-cosmology observations-cosmology theory-(cosmology)dark energy-radio lines ISM-radio lines galaxies-ISM clouds

Research on simulation of gun muzzle flow field empowered by artificial intelligence

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Defence Technology（防务技术） 2024年第2期32卷 196-208页

作者： Mengdi Zhou Linfang Qian Congyong Cao Guangsong Chen Jin Kong ming-hao tong School of Mechanical Engineering Nanjing University of Science and TechnologyNanjing210094China Northwest Institute of Mechanical and Electrical Engineering Xianyang712099ShaanxiChina School of Automation Nanjing University of Science and TechnologyNanjing210094China

Artificial intelligence technology is introduced into the simulation of muzzle flow field to improve its simulation efficiency in this paper.A data-physical fusion driven framework is ***,the known flow field data is used to initialize the model parameters,so that the parameters to be trained are close to the optimal *** physical prior knowledge is introduced into the training process so that the prediction results not only meet the known flow field information but also meet the physical conservation *** two examples,it is proved that the model under the fusion driven framework can solve the strongly nonlinear flow field problems,and has stronger generalization and *** proposed model is used to solve a muzzle flow field,and the safety clearance behind the barrel side is *** is pointed out that the shape of the safety clearance under different launch speeds is roughly the same,and the pressure disturbance in the area within 9.2 m behind the muzzle section exceeds the safety threshold,which is a dangerous *** with the CFD results shows that the calculation efficiency of the proposed model is greatly improved under the condition of the same calculation *** proposed model can quickly and accurately simulate the muzzle flow field under various launch conditions.

关键词： Muzzle flow field Artificial intelligence Deep learning Data-physical fusion driven Shock wave

Slow magnetic relaxation and photoluminescence behaviors of dilanthanide complexes bearing N_(8)O_(2)donor macrocyclic ligand

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Journal of Rare Earths 2024年第7期42卷 1298-1303,I0004页

作者： Pei-Yu Liao Yu-Quan Qi Zhen Li Jian-Hua Jia ming-Liang tong Key Laboratory of Bioinorganic and Synthetic Chemistry of Ministry of Education School of ChemistrySun Yat-Sen UniversityGuangzhou510006China

On account of the complicated magnetic exchange interactions between lanthanide ions,binuclear lanthanide complexes have broad application prospect in the field of single-molecule ***,it is necessary to develop reasonable bridging ligands to manipulate the directional assembly of binuclear lanthanide ***,we selected the macrocyclic ligand L^(N_(8)O_(2))to build up two new dilanthanide complexes[Ln_(2)(LN_(8)O_(2))(OpyO)_(2)(H_(2)O)2](NO_(3))_(2)(1-Ln,Ln=Dy,Tb;LN_(8)O_(2)=hexamethyl-tetraaza-dioxe-dipyrazolacycloicosaphane-2,9,12,19-tetraene;OpyOH=2-pyridinol-1-oxide).Dynamic magnetic studies show that 1-Dy exhibits slow relaxation behavior under a 1 kOe applied *** fitting analysis of relaxation times gives the effective energy barrier of 38.2 cm^(−1),and reveals that the slow magnetic relaxation behavior is dominated by the Orbach and Raman ***-resolution luminescence emission spectrum indicates the energy gap of 36.8 cm^(−1)between the ground state and the first excited state,consistent with the magnetic measurement results.1-Tb exhibits brilliant characteristic green light emission under UV light *** absolute quantum yield of 1-Tb is 44.8%,and its first-order fitted decay lifetime is 779.21μs at room *** study provides the way for directional construction of high-performance molecular materials with magnetic and optical dual-function.

关键词： Dilanthanide complexes Single-molecule magnets Macrocyclic ligand Photoluminescence behavior Rare earths

PHOX2B mutations in three Chinese patients with congenital central hypoventilation syndrome

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Chinese Medical Journal 2006年第20期119卷 1749-1752页

作者： Siu-Fong June Or ming-for tony tong Fai-Man Ivan Lo Chi-Wai Law Ting-Yat Miu Delphine Trochet Tak-Sum Stephen Lam Clinical Genetic Service Department of Health Hong Kong SAR China Department of Paediatrics Queen Elizabeth Hospital Hong Kong SAR China Department de Genetique Hospital Necker-Enfants Malades Paris France

Congenital central hypoventilation syndrome （CCHS, OMIM #209880） is a rare autosomal dominant disorder of the autonomic nervous system （ANS） characterized by an abnormal autonomic ventilatory response to progressive hypercarbia and sustained hypoxemia. Patients typically present in the newborn period with hypoventilation or apnea asleep, awake, or both, without any associated cardiac, pulmonary, neuromuscular or brainstem lesions. Rarely, some patients may present at a later age and are diagnosed to have late onset central hypoventilation syndrome （LOCHS）.1 Other features of ANS dysfunction such as feeding difficulty due to oesophageal dysmotility, severe constipation in the absence of Hirschsprung disease, poor regulation of basal body temperature, episodes of profuse sweating, pupillary and ocular abnormalities, decreased beat-to-beat variability of heart rate, attenuated response of heart rate to exercise, abnormal fluctuation of blood pressure, decreased perception to pain, and decreased perception to anxiety may be variably present but not essential for diagnosis Furthermore, this central hypoventilation can occur as an isolated feature or in association with a number of neurocristopathies, notably Hirschsprung disease （Haddad syndrome, OMIM #209880） and tumours of the sympathetic nervous system particularly neuroblastoma, ganglioneuro- blastoma, and ganglioneuroma, which were found in 20% and 5%--10% of CCHS patients, respectively.2 Studies of genes pertinent to the early embryologic development of the neural crest cells, specifically the endothelin and the RET-GDNF signaling pathways, have recently led to the identification of PHOX2B as the major disease causing gene for CCHS.2-6 PHOX2B was mapped to chromosome 4p12 and consists of 3 exons. It encodes a highly conserved paired-like homeobox transcription factor of 314 amino acids linked to the RET-GDNF signaling

关键词： congenital central hypoventilation syndrome PHOX2B Chinese mutation

Immune functions of C-type lectins in medical arthropods

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Insect Science 2024年第3期31卷 652-662页

作者： Zhihao ming Zhiqiang Chen Hao tong Xia Zhou Tingting Feng Jianfeng Dai Institute of Biology and Medical Sciences Jiangsu Key Laboratory of Infection and ImmunitySoochow UniversitySuzhouJiangsu ProvinceChina School of Biology and Basic Medical Science Suzhou Medical College of Soochow UniversitySuzhouJiangsu ProvinceChina

C-type lectins(CTLs)are a family of proteins that contain 1 or more carbohydrate-recognition domains(CRDs)and bind to a broad repertoire of ligands in the presence of calcium *** play important roles in innate immune defenses against microorganisms by acting as pattern-recognition receptors(PRRs)for invading pathogens,such as bacteria,viruses,and *** binding to pathogen-associated ligands,CTLs mediate immune responses,such as agglutination,phagocytosis,and the activation of phenol oxidase progenitors,thereby clearing *** are an evolutionarily conserved family found in almost all vertebrates and *** arthropods can acquire and transmit a range pathogens through various approaches,such as bloodsucking,lancing,and parasitism,thus infecting humans and animals with related diseases,some of which can be *** studies have shown that lectins are important components of the arthropod immune system and are essential for the immune responses of arthropods to arthropod-borne *** article reviews the current understanding of the structure,function,and signaling pathways involved in CTLs derived from important medical arthropods.

关键词： arthropod C-type lectin innate immunity vector-borne disease