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Myopia with X-linked retinitis pigmentosa results from a novel gross deletion of RPGR gene

International Journal of Ophthalmology(English edition) 2020年第8期13卷 1306-1311页

作者： Hui-Ping Li Shi-qin Yuan Xiao-guang Wang Xun-Lun Sheng Xiao-Rong Li Tianjin Medical University Eye Hospital Tianjin Key Laboratory of Retinal Functions and DiseasesEye Institute and School of OptometryTianjin 300384China Department of Ophthalmology Ningxia Eye HospitalPeople’s Hospital of Ningxia Hui Autonomous RegionFirst Affiliated Hospital of Northwest University for NationalitiesNingxia Clinical Research Center on Diseases of Blindness in EyeYinchuan 750002Ningxia Hui Autonomous RegionChina

AIM: To identify mutations with whole exome sequencing(WES) in a Chinese X-linked retinitis pigmentosa(XLRP) family. METHODS: Patients received the comprehensive ophthalmic evaluation. Genomic DNA was extracted from peripheral blood and subjected to Sure Select Human All Exon 6+ UTR exon capture kit. The exons were sequenced as 100 base paired reads on Illumina HiS eq2500 system. Only mutations that resulted in a change in amino acid sequence were selected. A pattern of inheritance of the RP family was aligned to identified causal ***: We analysed the data of WES information from XLRP family. The analysis revealed a hemizygous large genomic deletion of RPGR c.29113 del was responsible for this XLRP. The gross deletion lead to a frame-shift mutation and generate stop codon at 7 animo acid behind Asp(D10 Afs*7), which would serious truncate RPGR protein. The novel frame-shift mutation was found to segregate with retinitis pigmentosa(RP) phenotype in this family. Bilateral myopia was present on the male patients, but carrier female showed unilateral myopia without ***: Our study identifies a novel frame-shift mutation of RPGR in a Chinese family, which would expand the spectrum of RPGR mutations. The geno-phenotypic analysis reveals a correlation between RP and myopia. Although exact mechanism of RP related myopia is still unknown, but the novel frame-shift mutation will give our hit on studying the molecular pathogenesis of RP and myopia.

关键词： retinitis pigmentosa myopia retinitis pigmentosa GTPase regulator whole exome sequencing

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Transforming Multidisciplinary Customer Requirements to Product Design Specifications

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Chinese Journal of Mechanical Engineering 2017年第5期30卷 1069-1080页

作者： Xiao-Jie Ma Guo-Fu Ding Sheng-Feng qin Rong Li Kai-Yin Yan Shou-Ne Xiao guang-Wu Yang Institute of Advanced Design and Manufacturing Southwest Jiaotong University School of Design Northumbria University State Key Laboratory of Traction Power Southwest Jiaotong University

With the increasing of complexity of complex mechatronic products, it is necessary to involve multidis- ciplinary design teams, thus, the traditional customer requirements modeling for a single discipline team becomes difficult to be applied in a multidisciplinary team and project since team members with various disciplinary backgrounds may have different interpretations of the customers＇ requirements. A new synthesized multidisci- plinary customer requirements modeling method is pro- vided for obtaining and describing the common understanding of customer requirements （CRs） and more importantly transferring them into a detailed and accurate product design specifications （PDS） to interact with dif- ferent team members effectively. A case study of designing a high speed train verifies the rationality and feasibility of the proposed multidisciplinary requirement modeling method for complex mechatronic product development. This proposed research offersthe instruction to realize the customer-driven personalized customization of complex mechatronic product.

关键词： Complex mechatronic product Crowdsourcing - Multidisciplinary customer requirementsmodeling Disciplinary specialty language dictionary High-speed train

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Apoptosis of Human Pancreatic Carcinoma Cells Induced By All-Trans Retinoic Acid and Interferon

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Chinese Journal of Cancer Research 2009年第3期21卷 224-228页

作者： Xiao-hua Wang Yuan-qin Yin Ping Ma Cheng-guang Sui Fan-dong Meng Jiang you-hong Cancer Research Institute the First Affiliated Hospital China Medical University Shenyang 110001 China

Objective： To investigate the apoptosis of human pancreatic carcinoma PC3 cells induced by the combination of all-trans retinoic acid （ATRA） with interferon alpha （IFN-α）. Methods： PC3 cells were treated with ATRA and IFN-α. The inhibitory rate of PC3 cell proliferation was detected using MTT method. Cellular apoptosis was determined with flow cytometry. The percentage of PC3 cell apoptosis was assayed using TUNEL methods. Results： ATRA and IFN-α could inhibit cellular proliferation and induces cellular apoptosis of PC3 cells. The inhibitory effect was stronger when the ATRA and IFN-α were combined as a therapy. Conclusion： ATRA inhibits the proliferation of PC3 cells and induce the apoptosis of PC3 cells. The combination of IFN-α with ATRA may enhance these effects on PC3 cells.

关键词： All-trans retinoic acid (ATRA) Interferon-alpha (IFN-α) Apoptosis Human pancreatic carcinoma cells

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Upregulation of Spinal Voltage-Dependent Anion Channel 1 Contributes to Bone Cancer Pain Hypersensitivity in Rats

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Neuroscience Bulletin 2017年第6期33卷 711-721页

作者： Xiangpeng Kong Jinrong Wei Diyu Wang Xiaoju Zhu youlang Zhou Shusheng Wang guang-Yin Xu Guo-qin Jiang Jiangsu Key Laboratory of Translational Research and Therapy for Neuro-Psychiatric Diseases and Institute of Neuroscience The Second Affiliated Hospital Soochow University Center for Translational Medicine Affiliated Zhangjiagang Hospital of Soochow University

Voltage-dependent anion channel 1（VDAC1） is thought to contribute to the progression of tumor development. However, whether VDAC1 contributes to bone cancer pain remains unknown. In this study, we found that the expression of VDAC1 was upregulated in the L2–5 segments of the spinal dorsal horn at 2 and 3 weeks after injection of tumor cells into the tibial cavity. Intrathecal injection of a VDAC1 inhibitor significantly reversed the pain hypersensitivity and reduced the over-expression of Toll-like receptor 4（TLR4）. Intrathecal injection of minocycline, an inhibitor of microglia, also attenuated the pain hypersensitivity of rat models of bone cancer *** results suggest that VDAC1 plays a significant role in the development of complicated cancer pain, possibly by regulating the expression of TLR4.

关键词： Cancer-induced pain Spinal dorsal horn Voltage-dependent anion channel 1 Toll-like receptor 4 Microglia

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Temperature Fluetuations in Laser-Solid Interaction

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Chinese Physics Letters 1999年第8期16卷 550-552页

作者： WEI Xue-qin ZHENG Qi-guang LI Zai-guang The State Key Laboratory of Laser Technology Huazhong University of Science and TechnologyWuhan 430074

Epoxylite,polymethylmethacrylate and aluminum are irradiated by a cw CO2 laser and their temperature fluctuations are measured by the infrared thermal imagers. It is reported that the temperature fluctuation becomes stronger with increase of the laser-solid interaction time in the experiment, which is in good agreement with the theory prediction. Furthermore, the temperature fluctuation is experimentally studied while the material is melted or vaporized.

关键词： Solid laser interaction

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Electroretinogram and Histopathologic Changes of the Retina after Methanol Intoxication

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法医学杂志 2013年第1期29卷 5-11,16页

作者： CHEN Jie-min ZHU guang-you ZHAO Zi-qin XIA Wen-tao Shanghai Key Laboratory of Forensic Medicine Institute of Forensic Science Ministry of Justice P.R.China Shanghai 200063 China Department of Forensic Medicine Shanghai Medical College of Fudan University Shanghai 200032 China

In order to study the functional and structural alterations of the retina in SD rat model after methanol intoxication,35 rats were divided randomly into five groups administrated with saline,3-day high dose,7-day high dose,3-day low dose and 7-day low dose methanol *** retinal function of each group was assessed by flash electroretinogram(F-ERG) 3 and 7 days after methanol *** microstructure and ultrastructure of the retina were observed at the same *** high-dose methanol intoxication induced irreversible retinal functional and structural damages 3 days after poisoning,which included prolonged latency and reduced amplitude of the Max-reaction of *** injuries were aggravated 7 days after ***,the latency and amplitude of the Cone-reaction of F-ERG were also affected 3 days after poisoning,but there were no further worsening tendency 7 days after *** retinal histological analysis showed cellular edema,heteromorphy and disarrangement,tissular loosen of the inner nuclear layer and photoreceptors *** mitochondrial damage began at the photoreceptors layer and developed further into the inner nuclear *** low-dose methanol intoxication only caused transient damage of the *** results showed that the function and structure of the photoreceptor and inner nuclear layer were the primary target of methanol intoxication and that the rod cells were more sensitive to methanol intoxication than the cone *** mitochondrial damage developed from outer layer to inner layer of the retina.

关键词：甲醇中毒司法鉴定视网膜鉴定方法

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Chemical approaches towards installation of rare functional groups in bacterial surface glycans

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Chinese Journal of Natural Medicines 2022年第6期20卷 401-420页

作者： qin Chun-Jun DING Mei-Ru TIAN guang-Zong ZOU Xiao-Peng FU Jun-Jie HU Jing YIN Jian Key Laboratory of Carbohydrate Chemistry and Biotechnology Ministry of EducationSchool of BiotechnologyJiangnan UniversityWuxi 214122China Wuxi School of Medicine Jiangnan UniversityWuxi 214122China

Bacterial surface glycans perform a diverse and important set of biological roles,and have been widely used in the treatment of bacterial infectious *** majority of bacterial surface glycans are decorated with diverse rare functional groups,including amido,acetamidino,carboxamido and pyruvate *** functional groups are thought to be important constituents for the biological activities of *** synthesis of glycans bearing these functional groups or their variants is essential for the investigation of structure-activity relationships by a medicinal chemistry *** date,a broad choice of synthetic methods is available for targeting the different rare functional groups in bacterial surface *** article reviews the structures of naturally occurring rare functional groups in bacterial surface glycans,and the chemical methods used for installation of these groups.

关键词： Bacterial surface glycan Chemical synthesis Amido group Acetamidino group Carboxamido group Pyruvyl ketal

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An Exposure Meter of Lijiang Fiber-fed High-Resolution Spectrograph

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Research in Astronomy and Astrophysics 2023年第11期23卷 60-66页

作者： Xiao-guang Yu Kai-Fan Ji Xi-Liang Zhang Liang Chang Yun-Fang Cai Ying qin Zhen-Hong Shang Yunnan Observatories Chinese Academy of SciencesKunming 650011China Key Laboratory for the Structure and Evolution of Celestial Objects Chinese Academy of SciencesKunming 650216China University of Chinese Academy of Sciences Beijing 100049China Faculty of Information Engineering and Automation Kunming University of Science and TechnologyKunming 650500China

In 2016,an exposure meter was installed on the Lijiang Fiber-fed High-Resolution Spectrograph to monitor the coupling of starlight to the science fiber during *** on it,we investigated a method to estimate the exposure flux of the CCD in real time by using the counts of the photomultiplier tubes(PMT)of the exposure meter,and developed a piece of software to optimize the control of the exposure ***,by using flat-field lamp observations,we determined that there is a linear and proportional relationship between the total counts of the PMT and the exposure flux of the ***,using historical observations of different spectral types,the corresponding relational conversion factors were determined and obtained ***,the method was validated using actual observation data,which showed that all values of the coefficient of determination were greater than ***,software was developed to display the counts of the PMT and the estimated exposure flux of the CCD in real-time during the observation,providing a visual reference for optimizing the exposure time control.

关键词： telescopes instrumentation:spectrographs instrumentation:photometers

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Two Coordination Polymers Containing Linear Co(Ⅱ)-Trinuclear and Cu(Ⅱ)-Chain Assembled from 4-Fluorocinnamic Acid and N-Donor Ligand

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Chinese Journal of Structural Chemistry 2016年第2期35卷 286-292页

作者： KANG Hai-Xia FU Yu-qin XIN Ling-Yun LI Gui-Lian MA Lu-Fang LIU guang-Zhen College of Chemistry and Chemical Engineering Luoyang Normal University

Two new coordination polymers,named {[Co3（pfca）6（phen）2]·2H2O}n（1） and[Cu（pfca）2（4,4＇-bpy）（H2O）]n（2）（Hpfca = 4-fluorocinnamic acid and 4,4-bpy = 4,4-bipyridine）,were hydrothermally synthesized and characterized by elemental analysis,IR spectroscopy,thermogravimetric analysis（TGA）,and single-crystal X-ray *** 1 crystallizes in the triclinic crystal system,space group P1 with a = 10.939（3）,b = 12.430（4）,c = 13.815（4） A,α=78.436（3）,β= 70.261（3）,γ= 80.268（3）°,V= 1721.8（9） A^3,Dc = 1.508 g/cm^3,Mr= 1564.06,Z = 1,F（000） = 799,μ= 0.802 mm^（-1）,the final R = 0.0432 and wR = 0.1015 for 13250 observed reflections with I 2σ（I）.Complex 2 is of monoclinic system,space group P2／lc with a =15.4985（14）,b = 15.8424（14）,c = 10.9528（10） A,β= 107.1600（10）°,V= 2569.6（4） A^3,Dc = 1.468g/cm^3,Mr = 568.02,Z = 4,F（000） = 1164,μ= 0.906 mm^（-1）,the final R = 0.0371 and wR = 0.0842 for 18183 observed reflections with I〉 2σ（I）.Complex 1 features linear Co（Ⅱ）-trinuclear units,which are assembled into a final 3D supramolecular structure through π…π stacking interactions and F-H weak *** 2 consists of 1D polymeric Cu（Ⅱ）-chains that are further cohered together by extensive H-bonds.

关键词： 4-fluorocinnamic acid coordination polymers trinuclear chainmagnetic property

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Intimately coupled WS_(2) nanosheets in hierarchical hollow carbon nanospheres as the high-performance anode material for lithium-ion storage

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Rare Metals 2022年第4期41卷 1245-1254页

作者： Ze-Jun Zhao Yu-guang Chao Fang Wang Jia-Yi Dai Yi-Fan qin Xiao-Bing Bao Yong Yang Shao-Jun Guo State Key Laboratory of Solidification Processing Center of Advanced Lubrication and Seal MaterialsNorthwestern Polytechnical UniversityXi’an 710072China Department of Materials Science and Engineering College of EngineeringPeking UniversityBeijing 100871China

Lithium-ion hybrid capacitors(LIHCs)have drawn extensive attention in fleld of energy ***,the absence of appropriate electrode materials with rapid kinetics restricted the overall performance of the ***,hierarchical N,P-codoped hollow car-bon nanospheres coupling with WS_(2) nanosheets(N,P-codoped HCNS/WS_(2)NSs)were fabricated for boosting lithium storage ***,the WS_(2) nanosheets with several layers embedded in the N,P-codoped hollow carbon nanospheres could not only enhance the conduc-tivity of composites,but also provide abundant channels for the rapid transfer of *** a result,as-prepared N,P-codoped HCNS/WS_(2) NSs demonstrated superior rate performance and long-term cycling *** reversible discharge capacity of 725.2 mAh·g^(-1) could be preserved after 1000 cycles at a current density of 1.0 A·g^(-1).Fur-thermore,LIHCs devices were assembled by using N,P-codoped HCNS/WS_(2) NSs and activated carbon(AC)as the cathode and anode,which exhibited high energy density of 166.7 Wh·kg^(-1) and power density of 5312.4 W·kg^(-1).Last but not least,the capacity almost had no obvious deterioration after 6000 cycles at a high current density of 10.0 A·g^(-1).

关键词： N P-Codoped hollow nanospheres WS2 nanosheets Self-assembly Lithiumion anode Lithiumion hybrid capacitors

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