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1.
Spinal muscular atrophy (SMA) is an autosomal recessive hereditary neuromuscular disease. Exon 7 and 8 of survival of motor neuron 1 (SMN1) gene or only exon 7 homology deletion leads to the failure to produce a full-length SMN gene. The copy number of SMN2 gene with high homology of SMN1 affects the degree of disease and was the target gene for targeting therapy, in which splicing silencer in intron 7 was the key to suppress the inclusion of exon 7. In this study, we projected to use CRISPR/Case 9 for the targeted editing of intronic-splicing silencer (ISS) sequence to promote the inclusion of SMN2 exon 7 and increase the production of SMN2 full-length (FL) gene expression. It happens that there was a protospacer adjacent motif (PAM) at one end of the ISS sequence according to the design of sgRNA. The recombinant vector of sgRNA HSMN2 CRISPR/Case 9 was constructed and transfected into HEK293 cells. Sequencing results showed that the ISS sequence could be edited accurately and targeting in the predicted direction, in which deleting small fragments, inserting small amounts and mutation. Quantitative analysis of RT-PCR products by restriction enzyme of DdeI digestion showed that the FL of SMN2 increased by 8% (P < 0.05). In the primary cultured chondrocytes of SMA mice, in which sgRNA HSMN2 CRISPR/Case9 recombinant vector transfection could increase the SMN2 FL gene by 23% (P < 0.05) and significantly improve SMN protein levels (P < 0.05). CRISPR/Case 9 is an effective tool for gene editing and therapy of hereditary diseases, but it is rarely reported in the treatment of SMA diseases. This study shows that CRISPR/Case 9 was first used for the precision target of ISS sequence editing, which can effectively promote the production of SMN2 FL gene expressions, in which there was an important clinical reference value.  相似文献   
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A conducting and anticorrosive coating is crucial for the application of metal bipolar plates (BP) in proton exchange membrane fuel cell (PEMFC). In this work, a Ti3C2Tx (T)-carbon black (C)-acrylic epoxy (AE) coating is prepared on 304 stainless steel (SS) with enhanced corrosion resistance and conductivity. The corrosion resistance of the T-C-AE coating is investigated in a 0.5 M H2SO4 solution as compared to the AE, T, and T-AE coatings. The T-C-AE coated 304SS exhibits the strongest corrosion resistance with the most positive corrosion potential and the lowest corrosion current density of 0.00673 μA cm?2 in all the samples, while retaining intact and compact surface morphology with the lowest metal ion dissolution even after immersed for 720 h. The addition of Ti3C2Tx and carbon black into the AE matrix greatly decreases interfacial contact resistance (ICR), and the T-C-AE coating achieves a low ICR of 15.5 mΩ cm?2 under 140 N cm?2 compaction force. The excellent anticorrosion performance is mainly attributed to the physical barrier and the cathodic protection provided by the stacked Ti3C2Tx (MXene) nanosheets in the T-C-AE coating. This eco-friendly, conducting, and anticorrosive T-C-AE coating has a good application prospect on SS BP of PEMFC.  相似文献   
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Highly efficient electrocatalysts composed of earth-abundant elements are desired for water-splitting to produce clean and renewable chemical fuel. Herein, a heteroatomic-doped multi-phase Mo-doped nickel phosphide/nickel sulfide (Mo-NiPx/NiSy) nanowire electrocatalyst is designed by a successive phosphorization and sulfuration method for boosting overall water splitting (both oxygen and hydrogen evolution reactions (HER)) in alkaline solution. As expected, the Mo-NiPx/NiSy electrode possesses low overpotentials both at low and high current densities in HER, while the Mo-NiPx/NiSy heterostructure exhibits high active performance with ultra-low overpotentials of 137, 182, and 250 mV at the current density of 10, 100, and 400 mA cm−2 in 1 m KOH solution, respectively, in oxygen evolution reaction. In particular, the as-prepared Mo-NiPx/NiSy electrodes exhibit remarkable full water splitting performance at both low and high current densities of 10, 100, and 400 mA cm−2 with 1.42, 1.70, and 2.36 V, respectively, which is comparable to commercial electrolysis.  相似文献   
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Shen  Liang  Shen  Hao  Gao  Mingming  Liu  Yajuan  Huang  Xia 《Neural Processing Letters》2019,49(1):1-18
Neural Processing Letters - This paper is concerned with the global Mittag-Leffler synchronization schemes for the Caputo type fractional-order BAM neural networks with multiple time-varying delays...  相似文献   
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The microstructure and electrochemical behavior of Mg–6Al–1Zn, Mg–6Al–1Zn–1Ga, Mg–6Al–1Zn–1Sn, and Mg–6Al–1Zn–0.5Sn–0.5Ga as anode materials in a 3.5 wt% NaCl solution are compared systematically. The results show that Sn alloying refines the second-phases of Mg–6Zn–1Al by promoting tiny granular Mg17Al12 phases containing Sn, and inspires their disperse distribution. However, the Ga results in the formation of semicontinuous reticular Ga containing Mg17Al12 phases. The comparison of discharge tests indicates that Mg–6Al–1Zn–1Sn has the highest discharge activity, and Mg–6Al–1Zn–1Ga displays the largest hydrogen evolution corrosion resistance in 3.5 wt% NaCl solution at 298 K. The synergy of Ga and Sn can shorten discharge activation time and promote low discharge potential. In addition, the utilization efficiencies of the alloys decrease as follows: Mg–6Al–1Zn–1Ga > Mg–6Al–1Zn–0.5Sn–0.5Ga > Mg–6Al–1Zn–1Sn > Mg–6Zn–1Al. This study illustrates that the Mg–6Al–1Zn–0.5Sn–0.5Ga alloy has acceptable utilization efficiency and desirable electrochemical activity, which implies that doping Ga and Sn obtains a balance between discharge activity and utilization efficiencies.  相似文献   
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This paper deals with the problem of fault estimation and accommodation for a class of networked control systems with nonuniform uncertain sampling periods. Firstly, the reason why the adaptive fault diagnosis observer cannot be applied to networked control systems is analyzed. Based on this analysis, a novel robust fault estimation observer is constructed to estimate both continuous‐time fault and system states by using nonuniformly discrete‐time sampled outputs. Furthermore, using the obtained states and fault information, a nonuniformly sampled‐data fault tolerant control law is designed to preserve the stability of the closed‐loop system. The proposed scheme can not only guarantee the impact of continuous‐time uncertainties and discrete‐time sampled estimation errors on the faulty system to satisfy a H performance index but also repress the negative effect of the unknown intersample behavior of continuous‐time fault by use of an inequality technique. Finally, simulation results are included to demonstrate the feasibility of the proposed method. Copyright © 2014 John Wiley & Sons, Ltd.  相似文献   
10.
A two-stage microfiltration and ultrafiltration (MF/UF) for peanut protein recovery and water recycling from aqueous extraction processing (AEP) were investigated. Peanut protein aggregates and alkaline water were obtained using flat and wound MF/UF and alkaline water recycled in AEP. With this approach, most of the wastewater could be recycled to produce reusable water to solve wastewater problems in aqueous oil extraction processing from oilseeds. The result showed that a little oil and protein were lost (1.21% and 4.35%, respectively) during the recycling of permeate in AEP, which was still acceptable. Peanut protein aggregations (PPAs) were characterised by analysis with Fourier-transform infrared spectroscopy (FTIR), sodium dodecyl sulphate–polyacrylamide gel electrophoresis (SDS-PAGE), high-performance gel filtration chromatography (HPGFC) and binding force analysis. Solubility analysis suggested that PPA had molecular structures with more hydrophobic groups on the surface than in the peanut protein isolate (PPI). In vitro, PPA was digested by both pepsin and trypsin more than PPI. FTIR profiles demonstrated there were more β-sheet and less α-helix in PPA, which means much more aggregation. In addition, binding force analysis showed hydrophobic interaction was the major force that restricted dissolution of PPA and disulfide bonds were of second importance, which provided a possible physical treatment opportunity for improving the solubility of PPA.  相似文献   
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