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| 1 | Dosage effect of multiple genes accounts for multisystem disorder of myotonic dystrophy type 1显示文摘Multisystem manifestations in myotonic dystrophy type 1(DM1)may be due to dosage reduction in multiple genes induced by aberrant expansion of CTG repeats in DMPK,including DMPK,its neighboring genes(SIX5 or DMWD)and downstream MBNLh However,direct evidence is lacking.Here,we develop a new strategy to generate mice carrying multigene heterozygous mutations to mimic dosage reduction in one step by injection of haploid embryonic stem cells with mutant Dmpk,Six5 and Mbnll into oocytes.The triple heterozygous mutant mice exhibit adult-onset DM1 phenotypes.With the additional mutation in Dmwd,the quadruple heterozygous mutant mice recapitulate many major manifestations in congenital DM1.Moreover,muscle stem cells in both models display reduced sternness,providing a unique model for screening small molecules for treatment of DM1.Our results suggest that the complex symptoms of DM1 result from the reduced dosage of multiple genes. | Qi Yin Hongye Wang Na Li Yifu Ding Zhenfei Xie Lifang Jin Yan Li Qiong Wang Xinyi Liu Liuqing Xu Qing Li Yongjian Ma Yanbo Cheng Kai Wang Cuiqing Zhong Qian Yu Wei Tang Wanjin Chen Wenjun Yang Fan Zhang Chen Ding Lan Bao Bin Zhou Ping Hu Jinsong Li | 2020 | Cell Research2020，30，2： | 7 |
| 2 | Organophosphate esters cause thyroid dysfunction via multiple signaling pathways in zebrafish brain显示文摘Organophosphate esters(OPEs)are widespread in various environmental media,and can disrupt thyroid endocrine signaling pathways.Mechanisms by which OPEs disrupt thyroid hormone(TH)signal transduction are not fully understood.Here,we present in vivo-in vitro-in silico evidence establishing OPEs as environmental THs competitively entering the brain to inhibit growth of zebrafish via multiple signaling pathways.OPEs can bind to transthyretin(TTR)and thyroxine-binding globulin,thereby affecting the transport of TH in the blood,and to the brain by TTR through the blood-brain barrier.When GH3 cells were exposed to OPEs,cell proliferation was significantly inhibited given that OPEs are competitive inhibitors of TH.Cresyl diphenyl phosphate was shown to be an effective antagonist of TH.Chronic exposure to OPEs significantly inhibited the growth of zebrafish by interfering with thyroperoxidase and thyroglobulin to inhibit TH synthesis.Based on comparisons of modulations of gene expression with the Gene Ontology and Kyoto Encyclopedia of Genes and Genomes databases,signaling pathways related to thyroid endocrine functions,such as receptor-ligand binding and regulation of hormone levels,were identified as being affected by exposure to OPEs.Effects were also associated with the biosynthesis and metabolism of lipids,and neuroactive ligand-receptor interactions.These findings provide a comprehensive understanding of the mechanisms by which OPEs disrupt thyroid pathways in zebrafish. | Zhenfei Yan Chenglian Feng Xiaowei Jin Fangkun Wang Cong Liu Na Li Yu Qiao Yingchen Bai Fengchang Wu John P.Giesy | 2022 | Environmental Science and Ecotechnology2022，，4： | 2 |
| 3 | Toxicity mechanisms and bioavailability of copper to fish based on an adverse outcome pathway analysis显示文摘Copper(Cu)exists in a variety of forms in different aquatic environments,and affects their bioavailability.In this study we provide a systematic review on toxicity of Cu which focuses on identifying evidence in the mechanisms of Cu toxicity,and apply an adverse outcome pathway(AOP)analysis to identify multiple potential mechanisms and their interactions of Cu toxicity to fish.This analysis process included the mechanisms of behavior toxicant,oxidative toxicant,ion regulation disruption toxicity,as well as endocrine disruption toxicity.It was found that at low levels of Cu exposure,swimming,avoid predators,locating prey and other sensory functions will be impaired,and the organism will suffer from metabolic alkalosis and respiratory acidosis following the inhibition of the carbonic anhydrase active.The main pathway of acute toxicity of Cu to fish is the inhibition of the Na^(+)/K^(+)-ATPase enzyme,and lead to reduced intracellular sodium absorption,as well as Cu-induced increased cell permeability,in turn resulting in increased sodium ion loss,leading to cardiovascular collapse and respiratory insufficiency.The endocrine disruption toxicity of Cu to fish caused growth inhibition and reproductive reduction.In addition,there are several key pathways of Cu toxicity that are affected by hardness(e.g.,Ca^(2+))and intracellular DOC concentrations,including inhibiting Cu-induction,improving branchial gas exchange,altering membrane transport functions,decreasing Na+loss,and increasing Na+uptake.The results of the AOP analysis will provide a robust framework for future directed research on the mechanisms of Cu toxicity. | Wei Liao Ziwei Zhu Chenglian Feng Zhenfei Yan Yajun Hong Daqing Liu Xiaowei Jin | 2023 | Journal of Environmental Sciences2023，，5： | 1 |
| 4 | Effects of thickness on the nanocrystalline structure and semi- conductor-metal transition characteristics of vanadium dioxide thin films 显示文摘 | Luo Zhenfei Zhou Xun Yan Dawei | 2014 | Thin Solid Films2014，550，1： | 1 |
| 5 | Review of insulin-like growth factor 1 signaling pathway and its role in protection against brain diseases显示文摘Insulin-like growth factor 1(IGF-1) is a pluripotent growth factor,with multiple functions in the peripheral and central nervous systems. Increasing evidence suggests that IGF-1 fine-tunes the development of the central nervous system,ensuring proper neuronal differentiation,maturation,and connectivity. It supports neuronal survival and axon growth,and acts on myelinating Schwann cells and oligodendroglia. The biological functions of IGF-1 are modulated by the IGF-1 signaling pathway. Recent studies have proposed the modulation of the members of the IGF-1/IGF-1 signaling pathway as treatment for neuropathologies. In this study,we introduce the structure of IGF-1/2 and its receptors,with the intra-cellular interactions. Further,we review the therapeutic effects of IGF-1 in different models of brain diseases,via activation of different cellular mechanisms. | Min Wei Lun Dong Hengzhu Zhang Zhenfei Teng Xiaodong Wang Zhengcun Yan Lei She Yuping Li Xingdong Wang | 2017 | Translational Neuroscience and Clinics2017，3，4： | 0 |
| 6 | Influences of uncertainties to the generation feasible region for medium- and long-term electricity transaction显示文摘For the implementation of power market in China,medium-and Iong-term security checks are essential for bilateral transactions,of which the electricity quantity that constitutes the generation feasible region(GFR)is the target.However,uncertainties from load forecasting errors and transmission contingencies are threats to medium-and Iong-term electricity tradi ng in terms of their in flue nces on the GFR.In this paper,we prese nt a graphic distortio n pattern in a typical threegenerator system using the Monte Carlo method and projection theory based on security constrained economic dispatch.The underlying potential risk to GFR from uncertainties is clearly visualized,and their impact characteristics are discussed.A case study on detailed GFR distortion was included to dem on strate the effectiveness of this visualization model.The result implies that a small uncertainty could distort the GFR to a remarkable extent and that different line-contingency precipitates disparate the GFR distortion patterns,thereby eliciting great emphasis on load forecasting and line reliability in electricity transacti ons. | Yuyun Yang Zhenfei Tan Zhilin Jiang Jun Yao Xingqiang Wang Mingyuan Wang Yan Xie Zhiyun Hu | 2020 | Global Energy Interconnection2020，3，6： | 0 |
| 7 | Self-assembly behavior of disaccharide-containing supra-amphiphiles显示文摘In this paper,supra-amphiphilic compounds containing disaccharides and azobenzene ends have been constructed via dynamic covalent bond.It was found that the slight structural difference of the disaccharides made significant difference in the self-assembled morphologies.Namely,three kinds of azodisaccharide supra-amphiphiles were found to assemble into different morphologies,with the only difference in chemical structure from the disaccharides.More importantly,the structural difference between the disaccharides,including lactoside,maltoside and cellobioside was trivial.Molecular simulation revealed the packing of molecules was due to the different contribution from hydrogen bonds.The above results clearly indicated the contribution of saccharide packing,especially the related hydrogen bonding,to the final morphology of the assembled structures. | Zhenfei Gao Tiannan Wang Zikun Rao Hui Yan Ran Zhang Guosong Chen | 2023 | Chinese Chemical Letters2023，34，1： | 0 |
| 8 | Suspension of Australian National Electricity Market in 2022 Necessitates Mechanism Evolution Ensuring Power Supply Security显示文摘The National Electricity Market(NEM)in Australia was suspended during June 15-23,2022,with a primary attribution to the lack of available generation capacity.This incident is noteworthy because it was the first market suspension in NEM’s history and took place in a major energy exporting country.In this letter,we review the outline and impacts of the incident.From the perspectives of market regulation,electricity supply,and electricity demand,we identify three underlying causes of the market suspension and offer four recommendations for the market mechanism evolution to ensure power supply security. | Zhenfei Tan Hua Geng Xiaoyuan Xu Sijie Chen Zheng Yan | 2023 | Journal of Modern Power Systems and Clean Energy2023，11，2： | 0 |
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