|
|
|
题名
|
作者
|
年代
|
出处
|
被引量
|
| 1 | Analysis of genotypes and phenotypes in Chinese children with tuberous sclerosis complex显示文摘Tuberous sclerosis complex(TSC) is a neurocutaneous syndrome with serious clinical presentations, an autosomal dominant genetic disorder involving multiple organs and systems. We retrospectively investigated the clinical manifestations and genotypes of 20 Chinese children with TSC to enable informed diagnostic and surveillance recommendations in China. A retrospective analysis of clinical manifestations in 20 children(7.00±5.30 years old) with TSC was conducted. A genetic testing of the genes TSC1 and TSC2 was performed in 14 children.The earliest manifestations of TSC were skin lesions(80% of patients) and seizures(75%). Fourteen of the children presented with retinal hamartomas, and 2 of these underwent eye enucleation at other hospitals through misdiagnosis. On magnetic resonance imaging, 18 children exhibited subependymal nodules, and 16 ones showed cortical nodules. 5 cases of non-renal hamartomas, 5 cases of multiple renal cysts, and 5 cases of cardiac rhabdomyomas were observed.The genotyping of TSC1 and TSC2 in 14 children revealed 11 with mutations in TSC2, 2 with mutations in TSC1, and no mutations of either gene in one patient. Eight of these observed mutations are reported here in for the first time. The illness presentations of the TSC2-mutated patients were more severe than that of patients carrying TSC1 mutations.There were differences in the mutations of TSC genes in Chinese children from those reported in other countries. The described clinical characteristics and genotyping will help pediatric neurologists to understand, diagnosis, and treat TSC. | Dayong Bai Junyang Zhao Li Li Jun Gao Xu Wang | 2017 | Science China(Life Sciences)2017,60,7: | 5 |
| 2 | Connectivity of the Mandelbrot set for the family of renormalization transformations显示文摘We show that the Mandelbrot set for the family of renormalization transformations of 2-dimensional diamond-like hierachical Potts models in statistical mechanics is connected. We also give an upper bound for the Hausdorff dimension of Julia set when it is a quasi-circle. | WANG XiaoGuang 1,QIU WeiYuan 1 , YIN YongCheng 1 , QIAO JianYong 2 & GAO JunYang 2 1 School of Mathematical Sciences, Fudan University, Shanghai 200433, China 2 Center of Mathematics, China University of Mining and Technology, Beijing 100083, China | 2010 | Science China Mathematics2010,53,3: | 3 |
| 3 | Accessing Alignments of Ontologies via IoT Based on SKOS Data Model显示文摘Many ontologies are provided to representing semantic sensors data.However,heterogeneity exists in different sensors which makes some service operators of Internet of Thing(IoT) difficult(such as such as semantic inferring,non-linear inverted index establishing,service composing) .There is a great deal of research about sensor ontology alignment dealing with the heterogeneity between the different sensor ontologies,but fewer solutions focus on exploiting syntaxes in a sensor ontology and the pattern of accessing alignments.Our solution infers alignments by extending structural subsumption algorithms to analyze syntaxes in a sensor ontology,and then combines the alignments with the SKOS model to construct the integration sensor ontology,which can be accessed via the IoT.The experiments show that the integration senor ontology in the SKOS model can be utilized via the IoT service,and the accuracy of our prototype,in average,is higher than others over the four real ontologies. | Xiulei Liu Xia Hou Junyang Yu Ying Gao Yue Zhang Yingying Zhang | 2017 | China Communications2017,14,6: | 3 |
| 4 | Misiurewicz points on the Mandelbrot-like set concerning renormalization transformation显示文摘We study Misiurewicz points on the parameter space about a family of rational maps Tλ concerning renormalization transformation in statistical mechanic. We determine the intersection points of the Julia set J(Tλ) and the positive real axis R+and discuss the continuity of the Hausdorff dimension HD(J(f)) about real parameter λ. | GAO JunYang QIAO JianYong ZHAI Yu | 2014 | Science China Mathematics2014,57,12: | 2 |
| 5 | Hepatoprotective versus Oncogenic Functions of STAT3 in Liver Tumorigenesis显示文摘 | Hua Wang Fouad Lafdil Lei Wang Ogyi Park Shi Yin Junyang Niu Andrew M. Miller Zhaoli Sun Bin Gao | 2011 | The American Journal of Pathology2011,,2: | 1 |
| 6 | Structure Identification for Force-Induced Reaction Using Single-Molecule Conductance Measurement显示文摘Spiropyran derivatives are prototype mechanophores with a promising application as molecular sensors because of their changeable structure under external force stimuli.However,the chemical structure evolution under external stimuli remains unclear due to the uncertainty and difficulty in distinguishing the structures of different ring-opened merocyanine isomers generated in the force-induced reaction.Here we identify the structure of isomers produced by the force-induced reaction of spiropyran derivatives using a single-molecule conductance measurement and an unsupervised clustering algorithm.We found that the original data from the single-molecule conductance measurement can be divided into four clusters through unsupervised clustering.By introducing a photoinduced reaction and theoretical calculation,we identified and attributed the four clusters of data to the multiple states of the molecular junctions.Our work demonstrates that a single-molecule break junction measurement can distinguish the isomers in the force-induced reaction,suggesting the great potential of single-molecule conductance measurement and unsupervised clustering approaches for structural analysis. | Jueting Zheng Wenli Gao Taige Lu Lijue Chen Luchun Lin Ruiyun Huang Yongxiang Tang Gang Dong Junyang Liu Yifei Pan Wengui Weng Wenjing Hong | 2023 | CCS Chemistry2023,5,8: | 0 |
| 7 | Single-cell transcriptomic analysis uncovers intratumoral heterogeneity and drug-tolerant persister in ALK-rearranged lung adenocarcinoma显示文摘Dear Editor,Non-small cell lung carcinoma(NSCLC)accounts for more than 85%of all lung cancers[1].Lung adenocarcinomas(ADCs)arising from never-smokers have been frequently found to be associated with oncogenic driver mutations,including anaplastic lymphoma kinase(ALK)rearrangement.Echinoderm microtubule-associated protein-like 4(EML4)is the most common fusion partner of the ALK gene[2].Tyrosine kinase inhibitors(TKIs)are currently the first-line treatment for advanced EML4-ALKrearranged lung ADC. | Hoi-Hin Kwok Huiyu Li Jiashuang Yang Junyang Deng Nerissa Chui-Mei Lee Timmy Wing-Kuk Au Alva Ko-Yung Sit Michael Kuan-Yew Hsin Stephanie Kwai-Yee Ma LydiaWai-Ting Cheung Luc Girard Junya Fujimoto Ignacio Ivan Wistuba Boning Gao John Dorrance Minna David Chi-Leung Lam | 2023 | Cancer Communications2023,43,8: | 0 |
| 8 | Identification of potential key genes in resveratrol biosynthesis via transcriptional analyses of berry development in grapevine(Vitis spp.)genotypes varying in trans-resveratrol content显示文摘Resveratrol is an important secondary metabolite not only owing to its function as a phytoalexin,but also its potential benefits to human health.In this study,the content of trans-resveratrol was documented in seven accessions of grapevine,in the seed,pulp and skin of berries,and at three developmental stages.The highest amount(2.99μg g^(−1) FW)was found in the skin of berries at the ripe stage from V.amurensis‘Tonghua-3'.Resveratrol was not detected in several samples,including skin of berries at the green hard or véraison stage from V.davidii‘Tangwei'.We carried out transcriptional profiling of developing‘Tonghua-3'and‘Tangwei'berries to identify gene expression patterns that may be linked with the difference in resveratrol content between these accessions.The expression levels of several differentially expressed genes(DEGs)with presumed function in resveratrol biosynthesis,including STILBENE SYNTHASEs(STSs),CINNAMATE 4-HYDROXYLASEs(C4Hs)and 4-COUMARATE-COA LIGASEs(4CLs),were significantly higher in‘Tonghua-3',than in'Tangwei'during the véraison and ripe stages.Gene ontology and Kyoto Encyclopedia of Genes and Genomes analyses suggested that these DEGs were enriched for multiple biological processes at the three stages of fruit development.Additionally,we identified a total of 36 transcription factors,including MYBs,WRKYs,ERFs,bHLHs and bZIPs,that were coexpressed with 17 STSs via a weighted gene co-expression network analysis,suggesting roles as regulators of resveratrol biosynthesis.Overall,these findings provide insight into genotypic differences in resveratrol biosynthesis in grapevine,as well as the molecular genetics of its regulation. | Xiuming Zhang Yifei Wu Yichu Zhang Xiangjing Yin Steve van Nocker Junqiang Guo Zhi Li Min Gao Junyang Song Xiping Wang | 2022 | Fruit Research2022,2,1: | 0 |
| 9 | Dynamics of a family of rational maps concerning renormalization transformation显示文摘Considering a family of rational maps Tnλconcerning renormalization transform ation,we give a perfect description about the dynamical properties of Tnλand the topological properties of the Fatou components F(Tnλ).Furthermore,we discuss the continuity of the Hausdorff dimension HD(J(Tnλ))about real param eter A. | Yuhan ZHANG Junyang GAO Jianyong QIAO Qinghua WANG | 2020 | Frontiers of Mathematics in China2020,15,4: | 0 |
| 10 | Enzyme@bismuth-ellagic acid:a versatile platform for enzyme immobilization with enhanced acid-base stability显示文摘In situ encapsulation is an effective way to synthesize enzyme@metal–organic framework biocatalysts;however,it is limited by the conditions of metal–organic framework synthesis and its acid-base stability.Herein,a biocatalytic platform with improved acid-base stability was constructed via a one-pot method using bismuth-ellagic acid as the carrier.Bismuth-ellagic acid is a green phenol-based metal–organic framework whose organic precursor is extracted from natural plants.After encapsulation,the stability,especially the acid-base stability,of amyloglucosidases@bismuth-ellagic acid was enhanced,which remained stable over a wide pH range(2–12)and achieved multiple recycling.By selecting a suitable buffer,bismuth-ellagic acid can encapsulate different types of enzymes and enable interactions between the encapsulated enzymes and cofactors,as well as between multiple enzymes.The green precursor,simple and convenient preparation process provided a versatile strategy for enzymes encapsulation. | Junyang Xu Guanhua Liu Ying He Liya Zhou Li Ma Yunting Liu Xiaobing Zheng Jing Gao Yanjun Jiang | 2023 | Frontiers of Chemical Science and Engineering2023,17,6: | 0 |
| 11 | Nickel-Carnosine complex:A new carrier for enzymes immobilization by affinity adsorption显示文摘Immobilization is an effective method to promote the application of enzyme industry for improving the stability and realizing recovery of enzyme.To some extent,the performance of immobilized enzyme depends on the choice of carrier material.Therefore,the development of new carrier materials has been one of the key issues concerned by enzyme immobilization researchers.In this work,a novel organic–inorganic hybrid material,nickel-carnosine complex(NiCar),was synthesized for the first time by solvothermal method.The obtained NiCar exhibits spherical morphology,hierarchical porosity and abundant unsaturated coordination nickel ions,which provide excellent anchoring sites for the immobilization of proteins.His-tagged organophosphate-degrading enzyme(Opd A)and x-transaminase(ω-TA)were used as model enzymes to evaluate the performance of NiCar as a carrier.By a simple adsorption process,the enzyme molecules can be fixed on the particles of NiCar,and the stability and reusability are significantly improved.The analysis of protein adsorption on NiCar verified that the affinity adsorption between the imidazole functional group on the protein and the unsaturated coordination nickel ions on NiCar was the main force in the immobilization process,which provided an idea way for the development of new enzyme immobilization carriers. | Junyang Xu Yanjun Jiang Liya Zhou Li Ma Zhihong Huang Jiafu Shi Jing Gao Ying He | 2021 | Chinese Journal of Chemical Engineering2021,34,10: | 0 |
| 12 | XMe - Xiamen Molecular Electronics Code:An Intelligent and Open-Source Data Analysis Tool for Single-Molecule Conductance Measurements显示文摘Charge transport characterization of single-molecule junctions is essential for the fundamental research of single-molecule physical chemistry and the development towards single-molecule electronic devices and circuits. Among the single-molecule conductance characterization techniques,the single-molecule break junction technique is widely used in tens of worldwide research laboratories which can generate a large amount of experimental data from thousands of individual measurement cycles. However,data interpretation is a challenging task for researchers with different research backgrounds,and the different data analysis approaches sometimes lead to the misunderstanding of the measurement data and even reproducibility issues of the measurement. It is thus a necessity to develop a user-friendly all-in-one data analysis tool that automatizes the basic data analysis in a standard and widely accepted way. In this work,we present the XMe Code (Xiamen Molecular Electronics Code),an intelligent all-in-one data analysis tool for the comprehensive analysis of single-molecule break junction data. XMe code provides end-to-end data analysis that takes in the original experimental data and returns electronic characteristics and even charge transport mechanisms. We believe that XMe Code will promote the transparency of the data analysis in single-molecule electronics and the collaborations among scientists with different research backgrounds. | Zhichao Pan Gang Dong Chi Shang Ruihao Li Tengyang Gao Luchun Lin Huicong Duan Xiaohui Li Jie Bai Yilin Lai Wenfeng Wu Jia Shi Junyang Liu Wenjing Hong | 2024 | Chinese Journal of Chemistry2024,42,3: | 0 |