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| 1 | De novo mutation in ATP6V1B2 impairs lysosome acidification and causes dominant deafness-onychodystrophy syndrome显示文摘 | Yongyi Yuan Jianguo Zhang Qing Chang Jin Zeng Feng Xin Jianjun Wang Qingyan Zhu Jing Wu Jingqiao Lu Weiwei Guo Xukun Yan Hui Jiang Binfei Zhou Qi Li Xue Gao Huijun Yuan Shiming Yang Dongyi Han Zixu Mao Ping Chen Xi Lin Pu Dai | 2014 | Cell Research2014，24，11： | 13 |
| 2 | A novel mutation in the MITF may be digenic with GJB2 mutations in a large Chinese family of Waardenburg syndrome type Ⅱ显示文摘Waardenburg syndrome typeⅡ(WS2) is associated with syndromic deafness.A subset of WS2,WS2A,accounting for approximately 15%of patients,is attributed to mutations in the microphthalmia-associated transcription factor(MITF) gene.We examined the genetic basis of WS2 in a large Chinese family.All 9 exons of the MITF gene,the single coding exon(exon 2) of the most common hereditary deafness gene GJB2 and the mitochondrial DNA(mtDNA) 12S rRNA were sequenced.A novel heterozygous mutation c.[742_743delAAinsT;746_747delCA]in exon 8 of the MITF gene co-segregates with WS2 in the family.The MITF mutation results in a premature termination codon and a truncated MITF protein with only 247 of the 419 wild type amino acids.The deaf proband had this MITF gene heterozygous mutation as well as a c.[109G>A]+ [235delC]compound heterozygous pathogenic mutation in the GJB2 gene.No pathogenic mutation was found in mtDNA 12S rRNA in this family.Thus,a novel compound heterozygous mutation,c.[742_743delAAinsT;746_747delCA]in MITF exon 8 was the key genetic reason for WS2 in this family,and a digenic effect of MITF and GJB2 genes may contribute to deafness of the proband. | Xukun Yan Tianyu Zhang Zhengmin Wang Yi Jiang Yan Chen Hongyan Wang Duan Ma Lei Wang Huawei Li | 2011 | Journal of Genetics and Genomics2011，38，12： | 8 |
| 3 | The R2R3-MYB transcription factor GhMYB1a regulates flavonol and anthocyanin accumulation in Gerbera hybrida显示文摘Anthocyanins and flavonols have vital roles in flower coloration,plant development,and defense.Because anthocyanins and flavonols share the same subcellular localization and common biosynthetic substrates,these pathways may compete for substrates.However,the mechanism regulating this potential competition remains unclear.Here,we identified GhMYB1a,an R2R3-MYB transcription factor involved in the regulation of anthocyanin and flavonol accumulation in gerbera(Gerbera hybrida).GhMYB1a shares high sequence similarity with that of other characterized regulators of flavonol biosynthesis.In addition,GhMYB1a is also phylogenetically grouped with these proteins.The overexpression of GhMYB1a in gerbera and tobacco(Nicotiana tabacum)resulted in decreased anthocyanin accumulation and increased accumulation of flavonols by upregulating the structural genes involved in flavonol biosynthesis.We further found that GhMYB1a functions as a homodimer instead of interacting with basic helix-loop-helix cofactors.These results suggest that GhMYB1a is involved in regulating the anthocyanin and flavonol metabolic pathways through precise regulation of gene expression.The functional characterization of GhMYB1a provides insight into the biosynthesis and regulation of flavonols and anthocyanins. | Chunmei Zhong Yi Tang Bin Pang Xukun Li Yuping Yang Jing Deng Chengyong Feng Lingfei Li Guiping Ren Yaqin Wang Jianzong Peng Shulan Sun Shan Liang Xiaojing Wang | 2020 | Horticulture Research2020，7，1： | 7 |
| 4 | MOF-derived Co-MOF,O-doped carbon as trifunctional electrocatalysts to enable highly efficient Zn-air batteries and water-splitting显示文摘Development of high-efficiency non-noble electrocatalysts for oxygen reduction reaction(ORR),oxygen evolution reaction(OER)and hydrogen evolution reaction(HER)is urgently needed for high-performance Zn-air batteries and overall water splitting.Here,a facile strategy to synthesize novel Co-MOF,O-doped carbon(Co-MOF-T)based on Zn,Co-doped glucosamine and ZIF-8 by pyrolysis at temperature T was demonstrated.The prepared Co-MOF-800 showed a superior oxygen reduction reaction(ORR)activity comparable to that of commercial Pt/C catalyst.In addition,this catalyst shows great potential in the overall water splitting due to the excellent oxygen evolution reaction(OER)and hydrogen evolution reaction(HER)activities.Based on the trifunctional activity,the primary Zn-air batteries using a Co-MOF-800 air electrode achieved a high open-circuit voltage of 1.38 V,a specific capacity of 671.6 mAh g^(-1) Zn,and a prominent peak power density of 144 mW cm^(-2).Also,the rechargeable Zn-air batteries based on CoMOF-800 air electrode could be smoothly run for 510 cycles with a low voltage gap of 0.58 V.Finally,the trifunctional Co-MOF-800 catalyst was applied to boost the electrochemical water splitting,demonstrating its promising potential as a green energy material for practical applications. | Xinde Duan Na Pan Can Sun Kexin Zhang Xukun Zhu Mingdao Zhang Li Song Hegen Zheng | 2021 | Journal of Energy Chemistry2021，30，5： | 5 |
| 5 | Zbed3 participates in the subcortical maternal complex and regulates the distribution of organelles显示文摘我们以前识别了 subcortical 在老鼠为早 embryogenesis 和女富饶是必要的母亲的建筑群(SCMC ) 。然而, SCMC 由影响女富饶的分子的机制大部分仍然是 uncharacterized。这里,我们报导那新奇母亲的蛋白质,包含 3 的锌手指床类型(Zbed3 ) ,参予 SCMC。母亲的 Zbed3 的弄空导致女性的减少的肥沃,因为在变异的胚胎的一个比例的损害并且推迟的发展。母亲的 Zbed3 的损失在影响卵母细胞和接合子导致不对称的 zygotic 分割和细胞器的反常分布,类似于与破坏核心 SCMC 基因在女性观察的显型。进一步的调查表明这些显型与微导管的破坏动力学或细胞质的格子(CPL ) 的形成被联系。Zbed3 的稳定性和本地化取决于,但是没被要求为, SCMC 的形成。因此,我们的数据作为调停的下游的蛋白质之一建议 Zbed3 SCMC 功能并且在女富饶提供进一步的卓见进 SCMC 和 CPL 的角色。 | Zheng Gao Xiaoxin Zhang Xingjiang Yu Dandan Qin Yi Xiao Yang Yu Yunlong Xiang Xiaoqing Nie Xukun Lu Wenbo Liu Zhaohong Yi Lei Li | 2018 | Journal of Molecular Cell Biology2018，10，1： | 2 |
| 6 | Formative pluripotent stem cells show features of epiblast cells poised for gastrulation显示文摘The pluripotency of mammalian early and late epiblast could be recapitulated by naïve embryonic stem cells(ESCs)and primed epiblast stem cells(EpiSCs),respectively.However,these two states of pluripotency may not be sufficient to reflect the full complexity and developmental potency of the epiblast during mammalian early development.Here we report the establishment of self-renewing formative pluripotent stem cells(fPSCs)which manifest features of epiblast cells poised for gastrulation.fPSCs can be established from different mouse ESCs,pre-/early-gastrula epiblasts and induced PSCs.Similar to pre-/early-gastrula epiblasts,fPSCs show the transcriptomic features of formative pluripotency,which are distinct from naïve ESCs and primed EpiSCs.fPSCs show the unique epigenetic states of E6.5 epiblast,including the super-bivalency of a large set of developmental genes.Just like epiblast cells immediately before gastrulation,fPSCs can efficiently differentiate into three germ layers and primordial germ cells(PGCs)in vitro.Thus,fPSCs highlight the feasibility of using PSCs to explore the development of mammalian epiblast. | Xiaoxiao Wang Yunlong Xiang Yang Yu Ran Wang Yu Zhang Qianhua Xu Hao Sun Zhen-Ao Zhao Xiangxiang Jiang Xiaoqing Wang Xukun Lu Dandan Qin Yujun Quan Jiaqi Zhang Ng Shyh-Chang Hongmei Wang Naihe Jing Wei Xie Lei Li | 2021 | Cell Research2021，31，5： | 2 |
| 7 | A Systematic Investigation of Complement and Coagulation-Related Protein in Autism Spectrum Disorder Using Multiple Reaction Monitoring Technology显示文摘Autism spectrum disorder(ASD)is one of the common neurodevelopmental disorders in children.Its etiology and pathogenesis are poorly understood.Previous studies have suggested potential changes in the complement and coagulation pathways in individuals with ASD.In this study,using multiple reactions monitoring proteomic technology,16 of the 33 proteins involved in this pathway were identified as differentially-expressed proteins in plasma between children with ASD and controls.Among them,CFHR3,C4BPB,C4BPA,CFH,C9,SERPIND1,C8A,F9,and F11 were found to be altered in the plasma of children with ASD for the first time.SERPIND1 expression was positively correlated with the CARS score.Using the machine learning method,we obtained a panel composed of 12 differentially-expressed proteins with diagnostic potential for ASD.We also reviewed the proteins changed in this pathway in the brain and blood of patients with ASD.The complement and coagulation pathways may be activated in the peripheral blood of children with ASD and play a key role in the pathogenesis of ASD. | Xueshan Cao Xiaoxiao Tang Chengyun Feng Jing Lin Huajie Zhang Qiong Liu Qihong Zheng Hongbin Zhuang Xukun Liu Haiying Li Naseer Ullah Khan Liming She | 2023 | Neuroscience Bulletin2023，39，11： | 1 |
| 8 | Synthesis and Herbicidal Activity of 1-Phenoxyacetyl-3-arylimidazolidine-2,4-dione Compounds显示文摘In order to find new herbicidal compounds, twelve novel 1-phenoxyacetyl-3-arylimidazolidine-2,4-dione compounds were designed and synthesized by substructure combination strategy using 3-arylimidazolidine-2,4-dione as the intermediate. The structures of the target compounds were confirmed by ~1H NMR and IR. The preliminary bioassay results showed that most of the target compounds had good inhibition against rape and barnyardgrass at the concentration of 100 mg/L. Especially, compound H3 and H5 showed 100% inhibitory activity against rape. | Han Jintao Wang Xiuli Liu Yu Cong Yunbo Dong Wenkai Chai Hongwei Li Xukun | 2018 | Plant Diseases and Pests2018，9，2： | 0 |
| 9 | Review of key technologies of climbing robots显示文摘In recent years,the robot industry has developed rapidly,and researchers and enterprises have begun to pay more attention to this industry.People are barely familiar with climbing robots,a kind of special robot.However,from their practical value and scientific research value,climbing robots should studied further.This paper analyzes and summarizes the key technologies of climbing robots,introduces various kinds of climbing robots,and examines their advantages and disadvantages to provide a reference for future researchers.Many countries have studied climbing robots and made some achievements.However,due to the complexity of climbing robots,their climbing efficiency and accuracy need to be further improved.The new structure can improve the climbing efficiency.This paper analyzes climbing robots such as mechanical arms,magnetic attraction,and claws.Optimization methods and path planning can improve the accuracy of work.This paper involves some control methods,including complex intelligent control methods such as behavior-based robot control.This paper also investigates various kinematic planning methods and expounds and summarizes various path planning algorithms,including machine learning and reinforcement learning of artificial intelligence,ant colony algorithm,and other algorithms.Therefore,by analyzing the research status of climbing robots at home and abroad,this paper summarizes three important aspects of climbing robots,namely,structural design,control methods,and climbing strategies,elaborates on the achievements and existing problems of these key technologies,and looks forward to the future development trend and research direction of climbing robots. | Ye DAI Shikun LI Xukun RUI Chaofang XIANG Xinlei NIE | 2023 | Frontiers of Mechanical Engineering2023，18，4： | 0 |
| 10 | NR5A2 connects zygotic genome activation to the first lineage segregation in totipotent embryos显示文摘Zygotic genome activation(ZGA)marks the beginning of the embryonic program for a totipotent embryo,which gives rise to the inner cell mass(ICM)where pluripotent epiblast arises,and extraembryonic trophectoderm.However,how ZGA is connected to the first lineage segregation in mammalian embryos remains elusive.Here,we investigated the role of nuclear receptor(NR)transcription factors(TFs),whose motifs are highly enriched and accessible from the 2-cell(2C)to 8-cell(8C)stages in mouse embryos.We found that NR5A2,an NR TF strongly induced upon ZGA,was required for this connection.Upon Nr5a2 knockdown or knockout,embryos developed beyond 2C normally with the zygotic genome largely activated.However,4–8C-specific gene activation was substantially impaired and Nr5a2-deficient embryos subsequently arrested at the morula stage.Genome-wide chromatin binding analysis showed that NR5A2-bound cis-regulatory elements in both 2C and 8C embryos are strongly enriched for B1 elements where its binding motif is embedded.NR5A2 was not required for the global opening of its binding sites in 2C embryos but was essential to the opening of its 8C-specific binding sites.These 8C-specific,but not 2C-specific,binding sites are enriched near genes involved in blastocyst and stem cell regulation,and are often bound by master pluripotency TFs in blastocysts and embryonic stem cells(ESCs).Importantly,NR5A2 regulated key pluripotency genes Nanog and Pou5f1/Oct4,and primitive endoderm regulatory genes including Gata6 among many early ICM genes,as well as key trophectoderm regulatory genes including Tead4 and Gata3 at the 8C stage.By contrast,master pluripotency TFs NANOG,SOX2,and OCT4 targeted both early and late ICM genes in mouse ESCs.Taken together,these data identify NR5A2 as a key regulator in totipotent embryos that bridges ZGA to the first lineage segregation during mouse early development. | Fangnong Lai Lijia Li Xiaoyu Hu Bofeng Liu Ziqi Zhu Ling Liu Qiang Fan Huabin Tian Kai Xu Xukun Lu Qing Li Kong Feng Lijuan Wang Zili Lin Hongyu Deng Jinsong Li Wei Xie | 2023 | Cell Research2023，33，12： | 0 |
| 11 | Alkaline hydrogen production promoted by small-molecule modification on flowerlike Co_(2)(OH)_(2)CO_(3)显示文摘Developing a low-cost and high-efficiency nonprecious metal-based catalyst for hydrogen evolution reaction(HER) is of great significance for the utilization of hydrogen energy.In this work,we report a molecular-modification strategy to fabricate a self-supported hydrogen evolution electrode,specially by grafting the macrocyclic molecules(HHTP=2,3,6,7,10,11-hexahydroxytriphenylene) on the surface of a cobaltous dihydroxy carbonate(COC) seed layer.The HHTP-COC electrode is endowed with a rodlike structure,which provides favorable access for charge transportation and mass exchange.The macrocyclic molecule structure in HHTP can be grafted on COC and improve the electrical conductivity,while the interaction between HHTP and COC induces the rearrangement of charge configuration on the surface.Due to the combination effects of several aspects,the HHTP-COC electrode achieves astonishing HER activity,with a low overpotential of 61.0 mV(η_(10),at the current density of 10 mA cm^(-2)) and excellent stability in alkaline condition.This kind of interface engineering based on the organic molecules can be applied to the design and manufacture of electrocatalysts in the field of energy conversion and storage. | Yue Liu Huan Zhang Chen Yang Ziyang Xu Yiyang Shi Xukun Zhu Xinde Duan Ling Qin Yachao Jin Li Song Mingdao Zhang Hegen Zheng | 2023 | Journal of Energy Chemistry2023，，9： | 0 |
| 12 | The oncogenomic function of androgen receptor in esophageal squamous cell carcinoma is directed by GATA3显示文摘Dear Editor,Esophageal carcinoma(EC)is the 6th leading cause of cancer death worldwide.In China,esophageal squamous cell carcinoma(ESCC)is the predominant histological subtype of EC and the 4th leading cause of death from cancer.1,2 While esophagectomy has been central to the standard of care for localized ESCC,relapse often occurs rapidly.For advanced ESCC,multimodality therapies incorporating systemic chemotherapies and/or radiotherapy have yielded limited clinical benefit.Despite extensive research efforts,no targeted therapies have yet been approved for the treatment of advanced ESCC.Interestingly,ESCC is strongly characterized by a male-predominant propensity,as both incidence and mortality rate are 2–3-fold higher in males than in females.1 Previous studies have revealed a possible association between androgen receptor(AR)signaling and male ESCC.3,4 AR is a ligand-dependent transcription factor that regulates target gene expression through binding to androgen-responsive elements(AREs)in the presence of androgens.Conversely,AR antagonists(e.g.,enzalutamide)compete with androgens to bind AR and inhibit its binding to AREs.5,6 To date,while the genomic function of AR has been extensively studied in prostate cancer,5,7,8 it remains unknown how AR exerts its oncogenic functions in ESCC at a genome-wide scale.Addressing this question is of high clinical relevance as it will establish a mechanistic basis for a promising therapeutic strategy targeting the AR transcription axis for ESCC patients. | Furong Huang Hongyan Chen Xiaolin Zhu Tongyang Gong Xukun Li William Hankey Hongyan Wang Zhong Chen Qianben Wang Zhihua Liu | 2021 | Cell Research2021，31，3： | 0 |
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