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| 1 | Neural progenitor cells from human induced pluripotent stem cells generated less autogenous immune response显示文摘The breakthrough development of induced pluripotent stem cells(iPSCs)raises the prospect of patient-specific treatment for many diseases through the replacement of affected cells.However,whether iPSC-derived functional cell lineages generate a deleterious immune response upon auto-transplantation remains unclear.In this study,we differentiated five human iPSC lines from skin fibroblasts and urine cells into neural progenitor cells(NPCs)and analyzed their immunogenicity.Through co-culture with autogenous peripheral blood mononuclear cells(PBMCs),we showed that both somatic cells and iPSC-derived NPCs do not stimulate significant autogenous PBMC proliferation.However,a significant immune reaction was detected when these cells were co-cultured with allogenous PBMCs.Furthermore,no significant expression of perforin or granzyme B was detected following stimulation of autogenous immune effector cells(CD3+CD8 T cells,CD3+CD8+T cells or CD3 CD56+NK cells)by NPCs in both PBMC and T cell co-culture systems.These results suggest that human iPSC-derived NPCs may not initiate an immune response in autogenous transplants,and thus set a base for further preclinical evaluation of human iPSCs. | HUANG Ke LIU PengFei LI Xiang CHEN ShuBin WANG LiHui QIN Li SU ZhengHui HUANG WenHao LIU JuLi JIA Bei LIU Jie CAI JingLei PEI DuanQing PAN GuangJin | 2014 | Science China(Life Sciences)2014，57，2： | 3 |
| 2 | Cortical and Subcortical Grey Matter Abnormalities in White Matter Hyperintensities and Subsequent Cognitive Impairment显示文摘Grey matter(GM)alterations may contribute to cognitive decline in individuals with white matter hyperintensities(WMH)but no consensus has yet emerged.Here,we investigated cortical thickness and grey matter volume in 23 WMH patients with mild cognitive impairment(WMH-MCI),43 WMH patients without cognitive impairment,and 55 healthy controls.Both WMH groups showed GM atrophy in the bilateral thalamus,fronto-insular cortices,and several parietal-temporal regions,and the WMH-MCI group showed more extensive and severe GM atrophy.The GM atrophy in the thalamus and fronto-insular cortices was associated with cognitive decline in the WMH-MCI patients and may mediate the relationship between WMH and cognition in WMH patients.Furthermore,the main results were well replicated in an independent dataset from the Alzheimer's Disease Neuroimaging Initiative database and in other control analyses.These comprehensive results provide robust evidence of specific GM alterations underlying WMH and subsequent cognitive impairment. | Wenhao Zhu Hao Huang Shiqi Yang Xiang Luo Wenzhen Zhu Shabei Xu Qi Meng Chengchao Zuo Yong Liu Wei Wang Alzheimer’s Disease Neuroimaging Initiative | 2021 | Neuroscience Bulletin2021，37，6： | 1 |
| 3 | Deletion of CHD8 in cerebellar granule neuron progenitors leads to severe cerebellar hypoplasia,ataxia,and psychiatric behavior in mice显示文摘CHD8 is a candidate gene for autism spectrum disorders and neurological development delay.It has been reported to be essential for neurogenesis in the cerebral cortex,but the function of CHD8 in cerebellum has not been comprehensively investigated.The potential relationship of cerebellum dysplasia with psychiatric disorders in patients with CHD8 mutations is still not clear.In this study,we establish different conditional knockout mouse models to investigate the roles of CHD8 in cerebellar development.Mice with neural stem cell-specific Chd8 deletion exhibit significant reduction of cerebellum volume and no layering structure is detected.Genetic deletion of Chd8 in cerebellar granule neuron progenitors(GNPs)leads to cerebellar hypoplasia,absent of proliferation layer and ectopic of Purkinje neuron.However,no substantial cerebellar dysplasia is detected in mice with Purkinje neuron-or oligodendrocyte-specific Chd8 ablation.Single-cell RNA sequencing indicates that ribosome-related genes and pathways are most significantly disrupted in GNPs,indicating the potential mechanism.Importantly,in addition to the ataxia phenotype,mice with GNPspecific Chd8 ablation present a neuropsychiatric phenotype in three-chamber and light/dark tests.Taken together,our results provide insights not only into the function of CHD8 in cerebellar development,but also the pathogenesis of neuropsychiatric disorders in patients with CHD8 mutations. | Xiang Chen Tong Chen Chen Dong Huiyao Chen Xinran Dong Lin Yang Liyuan Hu Huijun Wang Bingbing Wu Ye Yao Yu Xiong Man Xiong Yifeng Lin Wenhao Zhou | 2022 | Journal of Genetics and Genomics2022，49，9： | 1 |
| 4 | Iron Atom-Cluster Strategy Synthesis of Hierarchically Porous Fe-N-C Catalysts for Proton Exchange Membrane Fuel Cells显示文摘Developing nonprecious metal-nitrogen-doped carbon(M-N-C)catalysts with high activity and stability is critical to their widespread use in fuel cells;however,these catalysts still face considerable challenges.Herein,a novel iron atom-cluster strategy for the synthesis of iron-based N-C catalyst comprising Fe nanoparticles(Fe NPs)surrounded by Fe-N_(x) sites is developed for oxygen reduction reactions in an acidic fuel cell.Iron oxide NPs were incorporated into zeolitic imidazolate framework-8(ZIF-8)-derived carbon materials and pyrolyzed at high temperatures using NaCl as a modifi er to produce Fe NPs and Fe-N_(x) composite active sites.The half-wave potential of the optimized Fe_(NP)/FeNC-NaCl material was substantially improved to 0.81 V.Furthermore,even after 15,000 cycles,the half-wave potential of the catalyst remained essentially unchanged.As a cathode catalyst for fuel cells,it realized a high peak power density of 436 mW/cm^(2)under a practical H_(2)-air atmosphere.Therefore,this study presents a new approach for designing and synthesizing electrocatalytic materials with high catalytic activity and stability. | Wenhao Qiu Qing Han Xiaogang Yu Zhonghua Xiang | 2023 | Transactions of Tianjin University2023，29，6： | 0 |
| 5 | Method for the extraction of circulating nucleic acids based on MOF reveals cell-free RNA signatures in liver cancer显示文摘Cell-free RNA(cf RNA) allows assessment of health, status, and phenotype of a variety of human organs and is a potential biomarker to non-invasively diagnose numerous diseases. Nevertheless, there is a lack of highly efficient and bias-free cf RNA isolation technologies due to the low abundance and instability of cf RNA.Here, we developed a reproducible and high-efficiency isolation technology for different types of cell-free nucleic acids(containing cf RNA and viral RNA) in serum/plasma based on the inclusion of nucleic acids by metal-organic framework(MOF) materials, which greatly improved the isolation efficiency and was able to preserve RNA integrity compared with the most widely used research kit method. Importantly, the quality of cf RNA extracted by the MOF method is about 10-fold that of the kit method, and the MOF method isolates more than three times as many different RNA types as the kit method. The whole transcriptome mapping characteristics of cf RNA in serum from patients with liver cancer was described and a cf RNA signature with six cf RNAs was identified to diagnose liver cancer with high diagnostic efficiency(area under curve = 0.905 in the independent validation cohort) using this MOF method. Thus, this new MOF isolation technique will advance the field of liquid biopsy, with the potential to diagnose liver cancer. | Yuqing Sun Haixin Yu Shaoqing Han Ruoxi Ran Ying Yang Yongling Tang Yuhao Wang Wenhao Zhang Heng Tang Boqiao Fu Boshi Fu Xiaocheng Weng Song-Mei Liu Hexiang Deng Shuang Peng Xiang Zhou | 2024 | National Science Review2024，11，1： | 0 |
| 6 | Feasibility and physics potential of detecting ^(8)B solar neutrinos at JUNO显示文摘The Jiangmen Underground Neutrino Observatory(JUNO)features a 20 kt multi-purpose underground liquid scintillator sphere as its main detector.Some of JUNO's features make it an excellent location for^8B solar neutrino measurements,such as its low-energy threshold,high energy resolution compared with water Cherenkov detectors,and much larger target mass compared with previous liquid scintillator detectors.In this paper,we present a comprehensive assessment of JUNO's potential for detecting^8B solar neutrinos via the neutrino-electron elastic scattering process.A reduced 2 MeV threshold for the recoil electron energy is found to be achievable,assuming that the intrinsic radioactive background^(238)U and^(232)Th in the liquid scintillator can be controlled to 10^(-17)g/g.With ten years of data acquisition,approximately 60,000 signal and 30,000 background events are expected.This large sample will enable an examination of the distortion of the recoil electron spectrum that is dominated by the neutrino flavor transformation in the dense solar matter,which will shed new light on the inconsistency between the measured electron spectra and the predictions of the standard three-flavor neutrino oscillation framework.IfDelta m^(2)_(21)=4.8times10^(-5);(7.5times10^(-5))eV^(2),JUNO can provide evidence of neutrino oscillation in the Earth at approximately the 3sigma(2sigma)level by measuring the non-zero signal rate variation with respect to the solar zenith angle.Moreover,JUNO can simultaneously measureDelta m^2_(21)using^8B solar neutrinos to a precision of 20% or better,depending on the central value,and to sub-percent precision using reactor antineutrinos.A comparison of these two measurements from the same detector will help understand the current mild inconsistency between the value of Delta m^2_(21)reported by solar neutrino experiments and the KamLAND experiment. | Angel Abusleme Thomas Adam Shakeel Ahmad Sebastiano Aiello Muhammad Akram Nawab Ali Fengpeng An Guangpeng An Qi An Giuseppe Andronico Nikolay Anfimov Vito Antonelli Tatiana Antoshkina Burin Asavapibhop João Pedro Athayde Marcondes de André Didier Auguste Andrej Babic Wander Baldini Andrea Barresi Eric Baussan Marco Bellato Antonio Bergnoli Enrico Bernieri David Biare Thilo Birkenfeld Sylvie Blin David Blum Simon Blyth Anastasia Bolshakova Mathieu Bongrand Clément Bordereau Dominique Breton Augusto Brigatti Riccardo Brugnera Riccardo Bruno Antonio Budano Max Buesken Mario Buscemi Jose Busto Ilya Butorov Anatael Cabrera Hao Cai Xiao Cai Yanke Cai Zhiyan Cai Antonio Cammi Agustin Campeny Chuanya Cao Guofu Cao Jun Cao Rossella Caruso Cédric Cerna Jinfan Chang Yun Chang Pingping Chen Po-An Chen Shaomin Chen Shenjian Chen Xurong Chen Yi-Wen Chen Yixue Chen Yu Chen Zhang Chen Jie Cheng Yaping Cheng Alexander Chepurnov Davide Chiesa Pietro Chimenti Artem Chukanov Anna Chuvashova Gérard Claverie Catia Clementi Barbara Clerbaux Selma Conforti Di Lorenzo Daniele Corti Salvatore Costa Flavio Dal Corso Christophe De La Taille Jiawei Deng Zhi Deng Ziyan Deng Wilfried Depnering Marco Diaz Xuefeng Ding Yayun Ding Bayu Dirgantara Sergey Dmitrievsky Tadeas Dohnal Georgy Donchenko Jianmeng Dong Damien Dornic Evgeny Doroshkevich Marcos Dracos Frédéric Druillole Shuxian Du Stefano Dusini Martin Dvorak Timo Enqvist Heike Enzmann Andrea Fabbri Lukas Fajt Donghua Fan Lei Fan Can Fang Jian Fang Marco Fargetta Anna Fatkina Dmitry Fedoseev Vladko Fekete Li-Cheng Feng Qichun Feng Richard Ford Andrey Formozov Amélie Fournier Haonan Gan Feng Gao Alberto Garfagnini Alexandre Göttel Christoph Genster Marco Giammarchi Agnese Giaz Nunzio Giudice Franco Giuliani Maxim Gonchar Guanghua Gong Hui Gong Oleg Gorchakov Yuri Gornushkin Marco Grassi Christian Grewing Maxim Gromov Vasily Gromov Minghao Gu Xiaofei Gu Yu Gu Mengyun Guan Nunzio Guardone Maria Gul Cong Guo Jingyuan Guo Wanlei Guo Xinheng Guo Yuhang Guo Paul Hackspacher Caren Hagner Ran Han Yang Han Miao He Wei He Tobias Heinz Patrick Hellmuth Yuekun Heng Rafael Herrera Daojin Hong YuenKeung Hor Shaojing Hou Yee Hsiung Bei-Zhen Hu Hang Hu Jianrun Hu Jun Hu Shouyang Hu Tao Hu Zhuojun Hu Chunhao Huang Guihong Huang Hanxiong Huang Qinhua Huang Wenhao Huang Xingtao Huang Yongbo Huang Jiaqi Hui Wenju Huo Cédric Huss Safeer Hussain Antonio Insolia Ara Ioannisian Daniel Ioannisyan Roberto Isocrate Kuo-Lun Jen Xiaolu Ji Xingzhao Ji Huihui Jia Junji Jia Siyu Jian Di Jiang Xiaoshan Jiang Ruyi Jin Xiaoping Jing Cécile Jollet Jari Joutsenvaara Sirichok Jungthawan Leonidas Kalousis Philipp Kampmann Li Kang Michael Karagounis Narine Kazarian Amir Khan Waseem Khan Khanchai Khosonthongkee Patrick Kinz Denis Korablev Konstantin Kouzakov Alexey Krasnoperov Svetlana Krokhaleva Zinovy Krumshteyn Andre Kruth Nikolay Kutovskiy Pasi Kuusiniemi Tobias Lachenmaier Cecilia Landini Sébastien Leblanc Frederic Lefevre Liping Lei Ruiting Lei Rupert Leitner Jason Leung Demin Li Fei Li Fule Li Haitao Li Huiling Li Jiaqi Li Jin Li Kaijie Li Mengzhao Li Nan Li Nan Li Qingjiang Li Ruhui Li Shanfeng Li Shuaijie Li Tao Li Weidong Li Weiguo Li Xiaomei Li Xiaonan Li Xinglong Li Yi Li Yufeng Li Zhibing Li Ziyuan Li Hao Liang Hao Liang Jingjing Liang Jiajun Liao Daniel Liebau Ayut Limphirat Sukit Limpijumnong Guey-Lin Lin Shengxin Lin Tao Lin Jiajie Ling Ivano Lippi Fang Liu Haidong Liu Hongbang Liu Hongjuan Liu Hongtao Liu Hu Liu Hui Liu Jianglai Liu Jinchang Liu Min Liu Qian Liu Qin Liu Runxuan Liu Shuangyu Liu Shubin Liu Shulin Liu Xiaowei Liu Yan Liu Alexey Lokhov Paolo Lombardi Claudio Lombardo Kai Loo Chuan Lu Haoqi Lu Jingbin Lu Junguang Lu Shuxiang Lu Xiaoxu Lu Bayarto Lubsandorzhiev Sultim Lubsandorzhiev Livia Ludhova Fengjiao Luo Guang Luo Pengwei Luo Shu Luo Wuming Luo Vladimir Lyashuk Qiumei Ma Si Ma Xiaoyan Ma Xubo Ma Jihane Maalmi Yury Malyshkin Fabio Mantovani Francesco Manzali Xin Mao Yajun Mao Stefano MMari Filippo Marini Sadia Marium Cristina Martellini Gisele Martin-Chassard Agnese Martini Davit Mayilyan Axel Müller Ints Mednieks Yue Meng Anselmo Meregaglia Emanuela Meroni David Meyhöfer Mauro Mezzetto Jonathan Miller Lino Miramonti Salvatore Monforte Paolo Montini Michele Montuschi Nikolay Morozov Pavithra Muralidharan Massimiliano Nastasi Dmitry VNaumov Elena Naumova Igor Nemchenok Alexey Nikolaev Feipeng Ning Zhe Ning Hiroshi Nunokawa Lothar Oberauer Juan Pedro Ochoa-Ricoux Alexander Olshevskiy Domizia Orestano Fausto Ortica Hsiao-Ru Pan Alessandro Paoloni Nina Parkalian Sergio Parmeggiano Teerapat Payupol Yatian Pei Nicomede Pelliccia Anguo Peng Haiping Peng Frédéric Perrot Pierre-Alexandre Petitjean Fabrizio Petrucci Luis Felipe Piñeres Rico Oliver Pilarczyk Artyom Popov Pascal Poussot Wathan Pratumwan Ezio Previtali Fazhi Qi Ming Qi Sen Qian Xiaohui Qian Hao Qiao Zhonghua Qin Shoukang Qiu Muhammad Rajput Gioacchino Ranucci Neill Raper Alessandra Re Henning Rebber Abdel Rebii Bin Ren Jie Ren Taras Rezinko Barbara Ricci Markus Robens Mathieu Roche Narongkiat Rodphai Aldo Romani Bedřich Roskovec Christian Roth Xiangdong Ruan Xichao Ruan Saroj Rujirawat Arseniy Rybnikov Andrey Sadovsky Paolo Saggese Giuseppe Salamanna Simone Sanfilippo Anut Sangka Nuanwan Sanguansak Utane Sawangwit Julia Sawatzki Fatma Sawy Michaela Schever Jacky Schuler Cédric Schwab Konstantin Schweizer Dmitry Selivanov Alexandr Selyunin Andrea Serafini Giulio Settanta Mariangela Settimo Muhammad Shahzad Vladislav Sharov Gang Shi Jingyan Shi Yongjiu Shi Vitaly Shutov Andrey Sidorenkov FedorŠimkovic Chiara Sirignano Jaruchit Siripak Monica Sisti Maciej Slupecki Mikhail Smirnov Oleg Smirnov Thiago Sogo-Bezerra Julanan Songwadhana Boonrucksar Soonthornthum Albert Sotnikov Ondrej Sramek Warintorn Sreethawong Achim Stahl Luca Stanco Konstantin Stankevich DušanŠtefánik Hans Steiger Jochen Steinmann Tobias Sterr Matthias Raphael Stock Virginia Strati Alexander Studenikin Gongxing Sun Shifeng Sun Xilei Sun Yongjie Sun Yongzhao Sun Narumon Suwonjandee Michal Szelezniak Jian Tang Qiang Tang Quan Tang Xiao Tang Alexander Tietzsch Igor Tkachev Tomas Tmej Konstantin Treskov Andrea Triossi Giancarlo Troni Wladyslaw Trzaska Cristina Tuve Stefan van Waasen Johannes van den Boom Guillaume Vanroyen Nikolaos Vassilopoulos Vadim Vedin Giuseppe Verde Maxim Vialkov Benoit Viaud Cristina Volpe Vit Vorobel Lucia Votano Pablo Walker Caishen Wang Chung-Hsiang Wang En Wang Guoli Wang Jian Wang Jun Wang Kunyu Wang Lu Wang Meifen Wang Meng Wang Ruiguang Wang Siguang Wang Wei Wang Wenshuai Wang Xi Wang Xiangyue Wang Yangfu Wang Yaoguang Wang Yi Wang Yifang Wang Yuanqing Wang Yuman Wang Zhe Wang Zheng Wang Zhimin Wang Zongyi Wang Apimook Watcharangkool Lianghong Wei Wei Wei Yadong Wei Liangjian Wen Christopher Wiebusch Steven Chan-Fai Wong Bjoern Wonsak Diru Wu Fangliang Wu Qun Wu Wenjie Wu Zhi Wu Michael Wurm Jacques Wurtz Christian Wysotzki Yufei Xi Dongmei Xia Yuguang Xie Zhangquan Xie Zhizhong Xing Benda Xu Donglian Xu Fanrong Xu Jilei Xu Jing Xu Meihang Xu Yin Xu Yu Xu Baojun Yan Xiongbo Yan Yupeng Yan Anbo Yang Changgen Yang Huan Yang Jie Yang Lei Yang Xiaoyu Yang Yifan Yang Haifeng Yao Zafar Yasin Jiaxuan Ye Mei Ye Ugur Yegin Frédéric Yermia Peihuai Yi Xiangwei Yin Zhengyun You Boxiang Yu Chiye Yu Chunxu Yu Hongzhao Yu Miao Yu Xianghui Yu Zeyuan Yu Chengzhuo Yuan Ying Yuan Zhenxiong Yuan Ziyi Yuan Baobiao Yue Noman Zafar Andre Zambanini Pan Zeng Shan Zeng Tingxuan Zeng Yuda Zeng Liang Zhan Feiyang Zhang Guoqing Zhang Haiqiong Zhang Honghao Zhang Jiawen Zhang Jie Zhang Jingbo Zhang Peng Zhang Qingmin Zhang Shiqi Zhang Tao Zhang Xiaomei Zhang Xuantong Zhang Yan Zhang Yinhong Zhang Yiyu Zhang Yongpeng Zhang Yuanyuan Zhang Yumei Zhang Zhenyu Zhang Zhijian Zhang Fengyi Zhao Jie Zhao Rong Zhao Shujun Zhao Tianchi Zhao Dongqin Zheng Hua Zheng Minshan Zheng Yangheng Zheng Weirong Zhong Jing Zhou Li Zhou Nan Zhou Shun Zhou Xiang Zhou Jiang Zhu Kejun Zhu Honglin Zhuang Liang Zong Jiaheng Zou | 2021 | Chinese Physics C2021，45，2： | 0 |
| 7 | Hydrogen Sulfide Improves Rice Seed Germination by Regulating Aluminum Absorption,Internal Antioxidant Enzyme System and Osmotic Balance under Aluminum Toxicity Conditions显示文摘Uncovering the mechanism of hydrogen sulfide(H2S)in improving rice seed germination under aluminum(Al)toxicity conditions is important for rice production in acidic soil.In the present study,an Al sensitive rice variety Kasalath was used.Pretreatment with 0.1 mmol/L sodium hydrosulfide(NaHS,H2S donor)under 70 mmol/L AlCl3(indicated as Al+NaHS treatment)increased rice seed germination by 27.95%,germination potential by 474.16%,and the germination index by 43.44%,compared with Al treatment.The treatment of Al+NaHS reduced the Al content in rice seeds by 16.31%and 32.11%and increased the internal H2S content by 3.82%and 8.90%at 3 and 5 d of treatment,respectively,compared with Al treatment.Al+NaHS treatment significantly increased the activities of superoxide dismutase(SOD)。 | WEI Qianqian KONG Yali XIANG Xingjia ZHU Lianfeng LIU Jia TIAN Wenhao JIN Qianyu YU Yijun ZHANG Junhua ZHU Chunquan | 2023 | Rice science2023，30，4： | 0 |
| 8 | CYP2C9^(*)3 Increases the Ibuprofen Response of Hemodynamically Significant Patent Ductus Arteriosus in the Infants with Gestational Age of More Than 30 Weeks显示文摘Hemodynamically significant patent ductus arteriosus(hsPDA)is a severe condition in newborns.Ibuprofen is an effective treatment to reduce the severe complications and the need for surgical treatment.Several single-nucleotide polymorphisms(SNPs)were related to the ibuprofen metabolism,treatment effects,and the onset of side effects.The effects of SNPs on hsPDA response after ibuprofen treatment are unknown.Therefore,in this study,we recruited hsPDA patients with standard ibuprofen treatment.Those patients had participated in China Neonatal Genomes Project(CNGP,ClinicalTrials.gov Identifier:NCT03931707)with next-generation sequencing data.We reanalyzed the sequencing data and compared the allele frequencies of known ibuprofen-related SNPs between ibuprofen Responder and Non-responder groups.In total,185 hsPDA patients were recruited with gestational age(GA)ranging from 24 to 40 weeks.No significant differences were detected in the basic information,period of ibuprofen treatment,rate of conservative treatment,complications,and side effects between ibuprofen Responder group and Non-responder group.Totally,17 hsPDA carried CYP2C9^(*)3 and one with CYP2C9^(*)2 were detected.In the GA group of more than 30 GA weeks(GA>30 wks group),we found higher allele frequency of CYP2C9^(*)3 in Responder group than in Non-responder group(16%vs.0,p=0.0391).In the GA group of less than 30 GA weeks(GA≤30 wks group),the sum allele frequency of CYP2C9^(*)3 and CYP2C9^(*)2 had no stastical difference between two groups(Responder group vs.Non-responder group,13%vs.11%,p=0.768).Therefore,we came to conclude that genetic tests of CYP2C9^(*)3 site may benefit the prediction of ibuprofen treatment outcome for hsPDA patients with gestational age of more than 30 weeks. | Xiang Chen Yuxi Chen Tiantian Xiao Xinran Dong Yulan Lu Yanyan Qian Huijun Wang Wenhao Zhou | 2022 | Phenomics2022，2，1： | 0 |
| 9 | Genetic Architecture of Childhood Kidney and Urological Diseases in China显示文摘Kidney disease is manifested in a wide variety of phenotypes,many of which have an important hereditary component.To delineate the genotypic and phenotypic spectrum of pediatric nephropathy,a multicenter registration system is being imple-mented based on the Chinese Children Genetic Kidney Disease Database(CCGKDD).In this study,all the patients with kidney and urological diseases were recruited from 2014 to 2020.Genetic analysis was conducted using exome sequencing for families with multiple affected individuals with nephropathy or clinical suspicion of a genetic kidney disease owing to early-onset or extrarenal features.The genetic diagnosis was confirmed in 883 of 2256(39.1%)patients from 23 provinces in China.Phenotypic profiles showed that the primary diagnosis included steroid-resistant nephrotic syndrome(SRNS,23.5%),glomerulonephritis(GN,32.2%),congenital anomalies of the kidney and urinary tract(CAKUT,21.2%),cystic renal disease(3.9%),renal calcinosis/stone(3.6%),tubulopathy(9.7%),and chronic kidney disease of unknown etiology(CKDu,5.8%).The pathogenic variants of 105 monogenetic disorders were identified.Ten distinct genomic disorders were identified as pathogenic copy number variants(CNVs)in 11 patients.The diagnostic yield differed by subgroups,and was highest in those with cystic renal disease(66.3%),followed by tubulopathy(58.4%),GN(57.7%),CKDu(43.5%),SRNS(29.2%),renal calcinosis/stone(29.3%)and CAKUT(8.6%).Reverse phenotyping permitted correct identification in 40 cases with clinical reassessment and unexpected genetic conditions.We present the results of the largest cohort of children with kidney disease in China where diagnostic exome sequencing was performed.Our data demonstrate the utility of family-based exome sequencing,and indicate that the combined analysis of genotype and phenotype based on the national patient registry is pivotal to the genetic diagnosis of kidney disease. | Ye Fang Hua Shi Tianchao Xiang Jiaojiao Liu Jialu Liu Xiaoshan Tang Xiaoyan Fang Jing Chen Yihui Zhai Qian Shen Guomin Li Li Sun Yunli Bi Xiang Wang Yanyan Qian Bingbing Wu Huijun Wang Wenhao Zhou Duan Ma Jianhua Mao Xiaoyun Jiang Shuzhen Sun Ying Shen Xiaorong Liu Aihua Zhang Xiaowen Wang Wenyan Huang Qiu Li Mo Wang Xiaojie Gao Yubin Wu Fang Deng Ruifeng Zhang Cuihua Liu Li Yu Jieqiu Zhuang Qing Sun Xiqiang Dang Haitao Bai Ying Zhu Siguang Lu Bili Zhang Xiaoshan Shao Xuemei Liu Mei Han Lijun Zhao Yuling Liu Jian Gao Ying Bao Dongfeng Zhang Qingshan Ma Liping Zhao Zhengkun Xia Biao Lu Yulong Wang Mengzhun Zhao Jianjiang Zhang Shan Jian Guohua He Huifeng Zhang Bo Zhao Xiaohua LI Feiyan Wang Yufeng Li Hongtao Zhu Xinhui Luo Jinghai Li Jia Rao Hong Xu | 2021 | Phenomics2021，1，3： | 0 |
| 10 | Space Habitat Astronautics:Multicolour Lighting Psychology in a 7-Day Simulated Habitat显示文摘During space missions,astronauts live in a confined technological environment,completely isolated and deprived of the variety and variation found in the environment on Earth.This circumstance has a strong impact on the psycho-physiological states of the crew.Particularly in light of the plans for long-duration missions,new research needs to be carried out.The goal of this study,conducted at Xiangtan Central Hospital in China,was to test whether multicolour lighting can improve people’s psychological state in an isolated and confined environment over a period of seven days.Twenty participants(10 male and 10 female)were randomly divided into two groups:one group that was exposed to multicolour lighting and a control group,which was exposed to a static,monotonous white interior.The participants’psychological state was recorded on the first day,the fourth day,and the seventh day.The results of the control group showed that the participants’negative emotions and anxiety continued to increase over time,whereas the group randomly exposed to multicolour lighting that changed every three hours did not show any significant increase in negative emotions and anxiety.Moreover,the random change of light colour in the isolated environment appeared to help the participants increase their sense of surprise,thereby counteracting monotony.Finally,during this experiment,it was observed that when people who are accustomed to being connected to social networks were deprived of this,they experienced insomnia and unaccustomed reactions,in particular on the first days of deprivation.This article contributes to future space exploration and to social and psychological support of life in isolated and confined environments. | Ao Jiang Irene Lia Schlacht Xiang Yao Bernard Foing Zhixiong Fang Stephen Westland Caroline Hemingray Wenhao Yao | 2022 | Space(Science & Technology)2022，，1： | 0 |
| 11 | Trigger control characteristics of fuze-recoil simulation system based on electromagnetic launcher显示文摘Fuze is the information processing and control unit of the ammunition, so the quality of the fuze becomes one of the most important aspects of ammunition detection. Since using recoil force is a common method to the arm fuze, its dynamic simulation test has always been the focus of the fuze test research. A new fuze recoil environmental simulation method is proposed based on the electromagnetic launcher. Then the trigger control characteristics of the fuze recoil simulation system and the influence of the trigger position on the recoil force are studied. The results of the study show that although the pulse width of the armature force curve can be changed by adjusting the trigger position, due to the limit of the range, there also exists the contradiction that the electromagnetic pulse width gets narrow with the increase of electromagnetic force peak. Thus, it cannot meet the requirements of the fuze launch recoil simulation. In order to make the recoil force close to the actual environment, the multi-stage trigger control characteristics are analyzed, and the influence of trigger position on recoil environmental force characteristics is studied. Then a fuze launch recoil environmental simulation platform is established and continuous electromagnetic force is achieved by using the trigger strategy.Finally, the experiment is performed to simulate the fuze launch recoil environment and show the feasibility and effectiveness of the proposed theoretical analysis. The major research work of this paper includes studying the composition and basic principle of the simulation system, establishing a launch model to analyze the single-stage and multi-stage coil fuze launch recoil characteristics,designing the test device to verify the correctness and validity of the research. This paper draws the conclusions that the feasibility of the fuze launch environmental simulation based on the electromagnetic launcher is verified, the trigger position has a great influence on force peak continuity, the problems of low maximum overload peak and short peak duration in the multi-stage coil fuze launch environmental simulation can be effectively solved through adjusting the trigger position, the system has creative and extensive application prospects. | Wenhao Wang Shihua Bi Hongjun Xiang Chao Zhan Xichao Yuan | 2017 | Journal of Systems Engineering and Electronics2017，28，3： | 0 |
| 12 | Non-cooperative target pose estimation based on improved iterative closest point algorithm显示文摘For localisation of unknown non-cooperative targets in space,the existence of interference points causes inaccuracy of pose estimation while utilizing point cloud registration.To address this issue,this paper proposes a new iterative closest point(ICP)algorithm combined with distributed weights to intensify the dependability and robustness of the non-cooperative target localisation.As interference points in space have not yet been extensively studied,we classify them into two broad categories,far interference points and near interference points.For the former,the statistical outlier elimination algorithm is employed.For the latter,the Gaussian distributed weights,simultaneously valuing with the variation of the Euclidean distance from each point to the centroid,are commingled to the traditional ICP algorithm.In each iteration,the weight matrix W in connection with the overall localisation is obtained,and the singular value decomposition is adopted to accomplish high-precision estimation of the target pose.Finally,the experiments are implemented by shooting the satellite model and setting the position of interference points.The outcomes suggest that the proposed algorithm can effectively suppress interference points and enhance the accuracy of non-cooperative target pose estimation.When the interference point number reaches about 700,the average error of angle is superior to 0.88°. | ZHU Zijian XIANG Wenhao HUO Ju YANG Ming ZHANG Guiyang WEI Liang | 2022 | Journal of Systems Engineering and Electronics2022，33，1： | 0 |
| 13 | Structure-Property Relationship of Conjugated Polymers Utilizing Isomerized Fused Benzothiadiazole-Based Building Blocks显示文摘Only a few fused benzothiadizole(BTz)based conjugated polymers have been applied in organic field-effect transistors(OFETs),and their performances were low,mainly due to the lack of available building blocks and in-depth study of the relationship between structure and performance.Herein,we report two isomeric fused BTz-based building blocks(M1 and M2)and their copolymers PBTzVBTz-T and PBTzVBTz-TT,PTVT-T and PTVT-TT.DFT study showed all polymers had nearly planar backbone geometries,and PBTzVBTz-TT exhibited the most linear backbone.PTVT-T and PTVT-TT exhibited red-shift absorption spectra and deeper LUMO level than those of PBTzVBTz-T and PBTzVBTz-TT.Thin film microstructure study demonstrated PBTzVBTz-TT and PTVT-TT had edge-on molecular orientation,while the others had the mixed molecular orientation of edge-on and face-on.Furthermore,PBTzVBTz-TT had the longest coherence length in thin film.All these features of PBTzVBTz-TT resulted in its highest carrier mobility of 0.1 cm^(2)·V^(–1)·s^(–1) among these polymers. | Jingyu Liu Wenhao Li Kaihu Xian Zhongwei Liu Yanru Li Xiang Yao Long Ye Yan Zhao Zhuping Fei | 2023 | Chinese Journal of Chemistry2023，41，19： | 0 |
| 14 | An asymmetric catalytic multi-component reaction enabled the green synthesis of isoserine derivatives and semi-synthesis of paclitaxel显示文摘Paclitaxel(Taxol^(@))as a cornerstone of chemotherapy remains in high demand.The semi-synthesis of paclitaxel via coupling of Taxus leaf-produced baccatin III and manually synthesized isoserine(C-13 side chain)is one of the solutions to avoid environmental damage caused by the sacrifice of the whole Taxus brevifolia.A green synthesis of the C-13 side chain is pivotal to the green production of paclitaxel.We herein report a green synthesis of isoserine derivatives and the semi-synthesis of paclitaxel via a straightforward assembly of isoserines based on an Rh2(TPA)3(OAc)/CPA1 co-catalyzed asymmetric multi-component reaction of ethyl diazoacetate,triethyl silanol and N-(anthrancen-9-ylmethyl)benzaldimine.The method is featured by improved atom economy,effective mass yield,and environmental factors compared to our previous racemic method and BMS company's semi-synthesis method.And the method allows for rapid access to paclitaxel derivatives with varied C-13 side chains. | Taoda Shi Xinglin Li Yukai Li Xiang Fu Linna Wu Dengqing Wu Wenhao Hu | 2023 | Green Synthesis and Catalysis2023，4，1： | 0 |
| 15 | Working principle and application of photocatalytic optical fibers for the degradation and conversion of gaseous pollutants显示文摘Photocatalytic optical fibers are promising for the degradation of gaseous and volatile pollutants in air due to their high specific surface area,high light utilization efficiency,easy regeneration,and sustainability.In particular,photocatalytic optical fibers have proven highly useful for the removal and conversion of different kinds of air pollutants in air.However,these fibers suffer from low photocatalytic degradation efficiencies.In this review,we have focused on introducing photocatalytic quartz optical fibers and photocatalytic plastic optical fibers for the degradation and transformation of gas-phase air pollutants.The principle of photocatalytic optical fibers and main methods for improving their photocatalytic and light utilization efficiencies based on semiconductor photocatalytic coatings are summarized.Moreover,the Langmuir-Hinshelwood kinetic rate equation was summarized to analyze the photocatalytic reduction of gaseous pollutants.Finally,an outlook on the future of photocatalytic optical fibers toward the removal and conversion of gaseous air pollutants is discussed. | Wenhao Xiang Jilin Yuan Yongwu Wu Hongyang Luo Chuanbao Xiao Nianbing Zhong Mingfu Zhao Dengjie Zhong Yuanyuan He | 2022 | Chinese Chemical Letters2022，33，8： | 0 |
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