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missense mutations of the fibrillin-1 gene in two Chinese patients with severe marfan syndrome

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Chinese medical Journal 2001年第5期 25-28页

作者： Ivan f.m. LO, Rosanna m.S. WONG, fanny W.f. LAm, tony m.f. tong and Stephen T.S. LAm

Objective To describe two Chinese patients with severe forms of marfan syndrome and to report findings of mutational analysis of the fibrillin-1 (fBN1) *** Two Chinese patients were studied, one suffering from marfan syndrome of infantile onset and the other of neonatal onset. Their clinical features were described. mutational analysis of the fBN1 gene was performed using polymerase chain reaction (PCR) technique and direct sequencing of exons 23 - 32,where the mutational hotspots for severe forms of marfan syndrome are *** Two missense mutations were successfully identified, a G3037A transition and an A3083T transversion, the latter being an unreported *** Taking advantage of the clustering phenomenon of mutations in severe forms of marfan syndrome, one can identify fBN1 mutations in these patients by first screening the mutational hotspots,thus reducing the effort that would otherwise be much greater because of the size of the gene.

关键词： marfan syndrome fibrillin-1 mutation

Prader-Willi Syndrome:16-Year Experience in Hong Kong

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Journal of Genetics and Genomics 2012年第4期39卷 191-194页

作者： Ivan f.m. Lo Ho ming Luk luksite@*** tony m.f. tong Kent K.S. Lai Daniel H.C. Chan Albert C.f. Lam David K.H. Chan Edgar W.L. Hau Connie O.Y. fung Stephen T.S. Lam Clin Genet Serv Dept Hlth Kowloon Hong Kong Peoples R China

Prader-Willi syndrome （PWS） is an important, well-recognized syndromic form of neurodevelopmental disorder. The incidence is about 1 in 15,000-25,000 live births, and it affects both males and females （Vogels et al... 详细信息

关键词： PWS Prader-Willi Syndrome

Generating a nanoscale blade-like optical field in a coupled nanofiber pair

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Photonics Research 2024年第1期12卷 154-162页

作者： YUXIN YANG JIAXIN GAO HAO WU ZHANKE ZHOU LIU YANG XIN GUO PAN WANG LImIN tong Interdisciplinary Center for Quantum Information New Cornerstone Science LaboratoryState Key Laboratory of Extreme Photonics and InstrumentationCollege of Optical Science and EngineeringZhejiang UniversityHangzhou 310027China Jiaxing Key Laboratory of Photonic Sensing&Intelligent Imaging Intelligent Optics&Photonics Research CenterJiaxing Research Institute Zhejiang UniversityJiaxing 314000China Collaborative Innovation Center of Extreme Optics Shanxi UniversityTaiyuan 030006China

An optical field with sub-nm confinement is essential for exploring atomic-or molecular-level light-matter *** such fields demonstrated so far have typically point-like cross-sections,an optical field having a higher-dimensional cross-section may offer higher flexibility and/or efficiency in ***,we propose generating a nanoscale blade-like optical field in a coupled nanofiber pair(CNP)with a 1-nm-width central *** on a strong mode coupling-enabled slit waveguide mode,a sub-nm-thickness blade-like optical field can be generated with a cross-section down to~0.28 nm×38 nm at 1550 nm wavelength(i.e.,a thickness of~λ_(0)∕5000)and a peak-to-background intensity ratio(PBR)higher than 20 d *** slit waveguide mode of the CNP can be launched from one of the two nanofibers that are connected to a standard optical fiber via an adiabatical fiber taper,in which a fundamental waveguide mode of the fiber can be converted into a high-purity slit mode with high efficiency(>98%)within a CNP length of less than 10μm at 1550 nm *** wavelengthdependent behaviors and group velocity dispersion in mode converting processes are also investigated,showing that such a CNP-based design is also suitable for broadband and ultrafast pulsed *** results may open up new opportunities for studying light-matter interaction down to the sub-nm scale,as well as for exploring ultra-high-resolution optical technology ranging from super-resolution nanoscopy to chemical bond manipulation.

关键词： fiber waveguide optical

Acetyl coenzyme A carboxylase modulates lipogenesis and sugar homeostasis in Blattella germanica

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Insect Science 2024年第2期31卷 387-404页

作者： Xiaojin Pei Tiantian Bai Yuan Luo Zhanfeng Zhang Sheng Li Yongliang fan tong-Xian Liu Guangdong Provincial Key Laboratory of Insect Developmental Biology and Applied Technology and Institute of Insect Science and Technology School of Life SciencesSouth China Normal UniversityGuangzhouChina State Key Laboratory of Crop Stress Biology for Arid Areas and Key Laboratory of Integrated Pest management on Crops in Northwestern Loess Plateau Ministry of AgricultureNorthwest A&F UniversityYanglingShaanxiChina Guangmeiyuan R&D Center Guangdong Provincial Key Laboratory of Insect Developmental Biology and Applied TechnologySouth China Normal UniversityMeizhouGuangdong ProvinceChina Institute of Entomology Guizhou UniversityGuiyangChina

Lipid and sugar homeostasis is critical for insect development and *** this study,we characterized an acetyl coenzyme A carboxylase gene in Blattella germanica(BgACC)that is involved in both lipogenesis and sugar *** found that BgACC was dominantly expressed in the fat body and integument,and was significantly upregulated after *** of BgACC in 5th-instar nymphs did not affect their normal molting to the next nymphal stage,but it caused a lethal phenotype during adult ***-RNA interference(RNAi)significantly downregulated total free fatty acid(ffA)and triacylglycerol(TAG)levels,and also caused a significant decrease of cuticular hydrocarbons(CHCs).Repression of BgACC in adult females affected the development of oocytes and resulted in sterile females,but BgACC-RNAi did not affect the reproductive ability of ***,knockdown of BgACC also changed the expression of insulin-like peptide genes(BglLPs),which mimicked a physiological state of high sugar *** addition,BgACC was upregulated when *** were fed on a high sucrose diet,and repression of BgACC upregulated the expression of the glycogen synthase gene(BgGlyS).moreover,BgACC-RNAi increased the circulating sugar levels and glycogen storage,and a longevity assay suggested that BgACC was important for the survival of *** under conditions of high sucrose *** results confirm that BgACC is involved in multiple lipid biogenesis and sugar homeostasis processes,which further modulates insect reproduction and sugar *** study benefits our understanding of the crosstalk between lipid and sugar metabolism.

关键词： acetyl-CoA carboxylase German cockroach lipid biosynthesis sugar tolerance

machine learning solution for regional landslide susceptibility based on fault zone division strategy

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Journal of mountain Science 2024年第5期21卷 1745-1760页

作者： WANG Yunhao WANG Luqi LIU Songlin SUN Weixin LIU Pengfei ZHU Lin YANG Wenyu GUO tong School of Civil Engineering Chongqing UniversityChongqing 400045China Key Laboratory of New Technology for Construction of Cities in mountain Area Chongqing UniversityMinistry of EducationChongqing 400045China National Joint Engineering Research Center of Geohazards Prevention in the Reservoir Areas Chongqing UniversityChongqing 400045China Institute of Geological Environment monitoring Chongqing 401147China Chongqing 205 Geological Team Chongqing 401121China

Landslide susceptibility assessment is an essential tool for disaster prevention and management. In areas with multiple fault zones, the impact of fault zone on slope stability cannot be disregarded. This study performed qualitative analysis of fault zones and proposed a zoning method to assess the landslide susceptibility in Chengkou County, Chongqing municipality, China. The region within a distance of 1 km.f.om the faults was designated as sub-zone A, while the remaining area was labeled as sub-zone B. To accomplish the assessment, a dataset comprising 388 historical landslides and 388 non-landslide points was used to train the random.f.rest model. 10-fold cross-validation was utilized to select the training and testing datasets for the model. The results of the models were analyzed and discussed, with a focus on model performance and prediction uncertainty. By implementing the proposed division strategy based on fault zone, the accuracy, precision, recall, f-score, and AUC of both two sub-zones surpassed those of the whole region. In comparison to the results obtained for the whole region, sub-zone B exhibited an increase in AUC by 6.15%, while sub-zone A demonstrated a corresponding increase of 1.66%. moreover, the results of 100 random realizations indicated that the division strategy has little effect on the prediction uncertainty. This study introduces a novel approach to enhance the prediction accuracy of the landslide susceptibility mapping model in areas with multiple fault zones.

关键词： Landslide susceptibility mapping fault division strategy Random.f.rest GIS

Identification and characterization of genes related to m^(6)A modification in kiwifruit using RNA-seq and ATAC-seq

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Horticultural Plant Journal 2024年第5期10卷 1121-1133页

作者： Xiaoli Hu tong Li Changbin Xu Yanna Xu Congjun You Xinyi Li Jinli Gong Xiaolong Li Xuepeng Sun Collaborative Innovation Center for Efficient and Green Production of Agriculture in mountainous Areas of Zhejiang Province Key Laboratory of Quality and Safety Control for Subtropical Fruit and VegetableMinistry of Agriculture and Rural AffairsCollege of Horticulture ScienceZhejiang A&F UniversityHangzhouZhejiang 311300China

N6-methyladenosine(m^(6)A)RNA modification is a conserved mechanism that regulates the fate of RNA across eukaryotic *** its significance,a comprehensive analysis of m^(6)A-related genes in non-model plants,such as kiwifruit,is ***,we identified 36 m^(6)A-related genes in the kiwifruit genome according to homology and phylogenetic *** performed bioinformatics and evolutionary analyses of the writer,eraser,and reader families of m^(6)A *** of public RNA-seq data collected from samples under various biotic and abiotic stresses indicated that most m^(6)A-related genes were remarkably expressed under different *** construction of gene co-expression networks,we found significant correlations between several m^(6)A-related genes and transcription factors(Tfs)as well as receptor-like genes during the development and ripening of ***,we performed ATAC-seq assays on diverse kiwifruit tissues to investigate the regulatory mechanisms of m^(6)A-related *** identified 10 common open chromatin regions that were present in at least two tissues,and these regions might serve as potential binding sites for mADS protein,C2H2 protein,and other predicted *** study offers comprehensive insights into the gene family of m^(6)A-related components in kiwifruit,which will lay foundation for exploring mechanisms of post-transcriptional regulation involved in development and adaptation of kiwifruit.

关键词： Kiwifruit N^(6)-methyladenosine Phylogenetic analysis cis-element Co-expression network

fast efficient photon deceleration in plasmas by using two laser pulses at different frequencies

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matter and Radiation at Extremes 2024年第3期9卷 11-18页

作者： Y.X.Wang X.L.Zhu S.m.Weng P.Li X.f.Li H.Ai H.R.Pan Z.m.Sheng Key Laboratory for Laser Plasmas(moe) School of Physics and AstronomyShanghai Jiao Tong UniversityShanghai 200240China Collaborative Innovation Center of IfSA Shanghai Jiao Tong UniversityShanghai 200240China Research Center of Laser fusion of China Academy of Engineering Physics MianyangSichuan 621900China State key Laboratory of High field Laser Physics Shanghai Institute of Optics and Fine MechanicsChinese Academy of SciencesShanghai 201800China Tsung-dao Lee Institute Shanghai Jiao Tong UniversityShanghai 200240China

The generation of ultrashort high-power light sources in the mid-infrared(mid-IR)to terahertz(THz)range is of interest for applications in a number of fields,from.f.ndamental research to biology and *** conventional laser technology,photon deceleration in plasma wakes provides an alternative approach to the generation of ultrashort mid-IR or THz ***,we present a photon deceleration scheme for the efficient generation of ultrashort mid-IR or THz pulses by using an intense driver laser pulse with a relatively short wavelength and a signal laser pulse with a relatively long *** signal pulse trails the driver pulse with an appropriate time delay such that it sits at the front of the second wake bubble that is driven by the driver *** to its relatively long wavelength,the signal pulse will be subjected to a large gradient of the refractive index in the plasma wake ***,the photon deceleration in the plasma wake becomes faster and more efficient for signal pulses with longer *** greatly enhances the capacity and efficiency of photon deceleration in the generation of ultrashort high-power light sources in the long-wavelength IR and THz spectral ranges.

关键词： laser ultrashort photon

Electric tuning of plasmonic resonances in ultrathin gold nanoribbon arrays

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Photonics Research 2024年第08期12卷 1750-1759页

作者： ZHENXIN WANG ALEXEY V.KRASAVIN CHENXINYU PAN JUNSHENG ZHENG ZHIYONG LI XIN GUO ANATOLY V.ZAYATS LImIN tong PAN WANG State Key Laboratory of Extreme Photonics and Instrumentation College of Optical Science and Engineering Zhejiang University Department of Physics and London Centre for Nanotechnology King's College London Jiaxing Key Laboratory of Photonic Sensing & Intelligent Imaging Intelligent Optics & Photonics Research Center Jiaxing Research Institute Zhejiang University

Ultrathin plasmonic nanostructures offer an unparalleled opportunity for the study of light–matter interactions at the nanoscale and realization of compact nanophotonic devices. In this study, we introduce an ultrathin gold nanoribbon array and demonstrate an electric approach to actively tuning its plasmonic resonance, which leveraging the extreme light confinement capability in the ultrathin plasmonic nanostructure and a robust nanoscale electro-optical effect in indium tin oxide. Optimizing the design(to a total thickness as small as 12 nm.f.r a 2-nmthick gold nanoribbon array), we numerically demonstrate a spectral shift in the plasmonic resonance up to 36 nm along with an approximately 16% change in the transmission at a gate voltage below 1.7 V at the wavelength of1.47 μm. This work presents progress towards electric tuning of plasmonic resonances in ultrathin metallic nanostructures for various applications including surface-enhanced spectroscopy, spontaneous emission enhancement,and optical modulation.

关键词： Chemical sensors Diffraction limit Electrooptical effects Indium tin oxide Light matter interactions Nanostructures

mechanism of action of Wuzi Yanzong pill in the treatment of oligoasthenozoospermia in rats determined via serum metabolomics

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Journal of Traditional Chinese medical Sciences 2024年第2期11卷 180-190页

作者： Shen Z. Zhang Z. tong K. Wang C. Wang S. Zhao P. Gu m. Hu J. Tang Y. Liu Z. School of Chinese materia medica Beijing University of Chinese Medicine Beijing 102488 China Beijing tong Ren Tang Company Limited Beijing 100062 China School of Traditional Chinese medicine Beijing University of Chinese Medicine Beijing 102488 China China Beijing tong Ren Tang Group Co. Ltd. Beijing 100075 China faculty of Chinese medicine Macau University of Science and Technology Macau 999078 China Department of Pathology University of California San Diego La Jolla 92093 CA United States

Objective: To investigate the mechanism of action of Wuzi Yanzong pill (WYP) in rats with oligoasthenozoospermia (OAZ) via metabolomics and to provide a possible basis for improving this WYP-based treatment. methods: A rat model of OAZ was established by treating male Sprague–Dawley rats with glucosides from Tripterygium wilfordii Hook. f. Seventy-two rats were randomly divided into six groups: control, L-carnitine (positive control), model, and low-, medium-, and high-dose WYP groups. Rats in the experimental groups were treated with WYP for 4 weeks. At the end of the treatment period, sperm cell quality (density, motility, and viability) was assessed using a semen analysis system, mitochondrial membrane potential (mmP) was assessed using flow cytometry, and testicular injury was assessed using hematoxylin and eosin staining to validate the therapeutic effect of WYP in OAZ. further, serum metabolomics-based analysis was performed using high-performance liquid chromatography-mass spectrometry to identify differential metabolic pathways and possible mechanisms of action of WYP in OAZ treatment. Results: A rat model of OAZ was considered successfully-established after comparing the quality of spermatozoa in the model group to that in the control group. WYP-m and WYP-H treatments significantly improved sperm cell density, motility, and viability compared with those in the model group (all P mpared with the model group, both WYP-m and WYP-H treatments increased mmP values (P = .006 and P = .021 respectively), while there was no significant difference in the L-carnitine group. L-carnitine and WYP administration reversed damage to the testes to varying degrees compared with that in the model group. further, 44 differential metabolites and four metabolic pathways, especially autophagy pathway, related to OAZ were identified via metabolomics. Conclusions: WYP improves sperm cell quality and mmP in OAZ primarily via autophagy regulation. These findings can be emplo

关键词： Autophagy male infertility metabolomics Oligoasthenozoospermia Sperm cell quality Wuzi Yanzong pill