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81.
82.
83.
Hui-Yung Song Yi-Ping Yang Yueh Chien Wei-Yi Lai Yi-Ying Lin Shih-Jie Chou Mong-Lien Wang Chien-Ying Wang Hsin-Bang Leu Wen-Chung Yu Chian-Shiu Chien 《International journal of molecular sciences》2021,22(5)
The late-onset type of Fabry disease (FD) with GLA IVS4 + 919G > A mutation has been shown to lead to cardiovascular dysfunctions. In order to eliminate variations in other aspects of the genetic background, we established the isogenic control of induced pluripotent stem cells (iPSCs) for the identification of the pathogenetic factors for FD phenotypes through CRISPR/Cas9 genomic editing. We adopted droplet digital PCR (ddPCR) to efficiently capture mutational events, thus enabling isolation of the corrected FD from FD-iPSCs. Both of these exhibited the characteristics of pluripotency and phenotypic plasticity, and they can be differentiated into endothelial cells (ECs). We demonstrated the phenotypic abnormalities in FD iPSC-derived ECs (FD-ECs), including intracellular Gb3 accumulation, autophagic flux impairment, and reactive oxygen species (ROS) production, and these abnormalities were rescued in isogenic control iPSC-derived ECs (corrected FD-ECs). Microarray profiling revealed that corrected FD-derived endothelial cells reversed the enrichment of genes in the pro-inflammatory pathway and validated the downregulation of NF-κB and the MAPK signaling pathway. Our findings highlighted the critical role of ECs in FD-associated vascular dysfunctions by establishing a reliable isogenic control and providing information on potential cellular targets to reduce the morbidity and mortality of FD patients with vascular complications. 相似文献
84.
Xuan Ge Jun Xu Qiaodan Hu Ren Qiu Pingsheng Lai Yanfeng Han Fan Yang Yongquan Zhou Jingyu Qin Pengfei Yu Wenquan Lu Jianguo Li 《Journal of the American Ceramic Society》2021,104(12):6207-6226
Although remarkable development of titanate-based glasses has been achieved, challenge remains to elucidate the correlation between structure and glass-forming properties in these systems due to their complex structure that is inconsistent with the classic Zachariasen's model. In this work, we aim to correlate the structural evolution of titanate melts to their glass-forming ability (GFA). The prototypical material barium dititanate (BaTi2O5, BT2) melts with different GFA were rendered by controlled melting atmospheres, and the corresponding structural changes were determined using in situ high-energy synchrotron X-ray diffraction combined with empirical potential structure refinement and ab initio molecular dynamics. The results show that BT2 melt in reducing atmosphere shows poor GFA but that in oxidizing atmosphere presents good GFA. Structural analysis demonstrates the mean coordination number of [TiOm] polyhedra is analogous in the melt under two different atmospheres but an enhanced heterogeneous cations aggregation takes place in the melt under reducing atmosphere, which is closely related to oxygen-deficiencies. Furthermore, we reveal that the enhanced heterogeneous cations aggregation promotes crystallization (and therefore hinders glass formation) through disordering the distribution of [TiOm] and [BaOn] polyhedra, changing the connectivity between these polyhedra, creating more crystal-like Ti-Ti clusters, and decreasing topological disorder of BT2 melt. Our work provides a new viewpoint to understand the GFA of titanates melt from structural heterogeneity beyond the previous perspectives that only focus on [TiOm] polyhedra. 相似文献
85.
Hongtu He Hongshen Liu Yen-Ting Lin Conghang Qu Jiaxin Yu Seong H. Kim 《Journal of the American Ceramic Society》2021,104(9):4718-4727
Thermal tempering is an industrial process widely used to make soda lime silica (SLS) glass panels stronger and tougher. During the tempering process, the upper and bottom sides of the glass may experience different cooling rates, and thus, their properties could be different. This study characterized changes in surface composition and subsurface glass network structures as well as indentation and wear resistance properties of the air- and tin-sides of 6-mm-thick SLS window panels faced toward the upper and sliding roller sides during thermal tempering. The results showed that although the chemical and structural differences detected with X-ray photoelectron spectroscopy and specular reflection infrared spectroscopy are subtle, there are large differences in nanoindentation behaviors and mechanochemical wear properties of the SLS glass surface. The findings of this study provide further insights into the performance difference between the air- and tin-sides of the SLS glass panel treated with thermal tempering. 相似文献
86.
87.
Prof. Dr. Chun-Chi Chen Prof. Dr. Jian Min Dr. Lilan Zhang Dr. Yu Yang Prof. Dr. Xuejing Yu Prof. Dr. Rey-Ting Guo 《Chembiochem : a European journal of chemical biology》2021,22(8):1317-1328
Cytochrome P450s are heme-thiolate enzymes that participate in carbon source assimilation, natural compound biosynthesis and xenobiotic metabolism in all kingdoms of life. P450s can catalyze various reactions by using a wide range of organic compounds, thus exhibiting great potential in biotechnological applications. The catalytic reactions of P450s are driven by electron equivalents that are sourced from pyridine nucleotides and delivered by cognate or matching redox partners (RPs). The electron transfer (ET) route from RPs to P450s involves one or more redox center-containing domains. As the rate of ET is one of the main determinants of P450 efficacy, an in-depth understanding of the P450 ET pathway should increase our knowledge of these important enzymes and benefit their further applications. Here, the various P450 RP systems along with current understanding of their ET routes will be reviewed. Notably, state-of-the-art structural studies of the two main types of self-sufficient P450 will also be summarized. 相似文献
88.
运用往复线性摩擦试验方法,搭配自制的摩擦试验夹具,模拟织造过程中氧化铝纤维束-筘齿的摩擦行为,研究加载力、预加张力和摩擦频率对悬空状态下氧化铝纤维束摩擦磨损性能的影响.结果表明:随着加载力的增加,氧化铝纤维束所受摩擦力及长丝断裂根数增加,摩擦系数减小;在预加张力为0.40 N时,氧化铝纤维束所受摩擦力和摩擦系数出现最小值,磨损程度也最小;在摩擦稳定阶段,摩擦频率增加,则氧化铝纤维束所受摩擦力先下降,后略有上升,当摩擦频率增加至5 Hz时,氧化铝纤维束的摩擦系数较1 Hz时增加18.7%,磨损程度也最为严重. 相似文献
89.
Qi Shuang Xiang WenXin Cai LiXun Liu XiaoKun Shao ChunBing Ning FangMao Shi JinHua Yu WeiWei 《中国科学:技术科学(英文版)》2021,64(12):2577-2585
Science China Technological Sciences - Mixed-mode I-II crack-based fatigue crack propagation (FCPI-II) usually occurs in engineering structures; however, no theoretical formula or effective... 相似文献
90.
Zhenkun Ke Xin Cao Chuanli Shan Lifen Shi Pingping Wang Yong Yang Fengyang Zhao Jiedong Cui Jinwei Li Gang Zhou Min Guan Tao Yu 《Ceramics International》2021,47(14):19605-19613
The purpose of the current work was to research the effect of alkali metal oxide on the structure, thermal properties, viscosity and chemical stability in the glass system (R2O–CaO–B2O3–SiO2) systematically. Because the glass would emulsify when Li2O was added to the glass batch, this article did not discuss Li2O. The results showed that when the amount of Na2O was less than 4 mol.%, there was a higher interconnectivity of borate and silicate sub-networks in glass, as more mixed Si–O–B bonds were present in glass. The glass samples exhibited excellent thermal properties and chemical stabilities. As the amount of Na2O exceeded 4 mol.%, the interconnectivity of borate and silicate sub-networks was weakened. The thermal properties and chemical stabilities of the glass samples were reduced. The connectivity of the silicate sub-network was weakened slightly as the Na/K ratio varied, and the coefficient of thermal expansion (CTE) of the glass samples gradually increased, and the resistance to thermal shock (RTS) value gradually decreased. Moreover, the viscosity of the glass samples decreased with the ratio of Na/Si and Na/K increased. 相似文献