Properties of Non-Aminoglycoside Compounds Used to Stimulate Translational Readthrough of PTC Mutations in Primary Ciliary Dyskinesia

Primary ciliary dyskinesia (PCD) is a rare disease with autosomal recessive inheritance, caused mostly by bi-allelic gene mutations that impair motile cilia structure and function. Currently, there are no causal treatments for PCD. In many disease models, translational readthrough of premature termination codons (PTC-readthrough) induced by aminoglycosides has been proposed as an effective way of restoring functional protein expression and reducing disease symptoms. However, variable outcomes of pre-clinical trials and toxicity associated with long-term use of aminoglycosides prompt the search for other compounds that might overcome these problems. Because a high proportion of PCD-causing variants are nonsense mutations, readthrough therapies are an attractive option. We tested a group of chemical compounds with known PTC-readthrough potential (ataluren, azithromycin, tylosin, amlexanox, and the experimental compound TC007), collectively referred to as non-aminoglycosides (NAGs). We investigated their PTC-readthrough efficiency in six PTC mutations found in Polish PCD patients, in the context of cell and cilia health, and in comparison to the previously tested aminoglycosides. The NAGs did not compromise the viability of the primary nasal respiratory epithelial cells, and the ciliary beat frequency was retained, similar to what was observed for gentamicin. In HEK293 cells transfected with six PTC-containing inserts, the tested compounds stimulated PTC-readthrough but with lower efficiency than aminoglycosides. The study allowed us to select compounds with minimal negative impact on cell viability and function but still the potential to induce PTC-readthrough.     

Photopolymerization kinetics of bis-aromatic based urethane acrylate macromonomers in the presence of reactive diluent

The present work deals with the photopolymerization of bis-aromatic based urethane acrylate macromonomers in the presence of excess end capping agent as reactive diluent and estimation of their kinetic parameters. Formulations were made by independently homogenizing the macromonomers with photoinitiators of three different classes. Three different compositions of photoinitiators were used to study the effect of concentration of photoinitiator on cure kinetics. These compositions obtained were tested for photo curing performance using photo DSC under polychromatic radiation. The heat flows against time were recorded for all formulations under isothermal condition and the rates of polymerization, peak maximum times as well as the percentage conversions were estimated. It was observed that due to a longer timescale for reaction diffusion, formulations with macromonomer containing propoxylated backbone showed higher conversions than the corresponding ethoxylated analogue. The photopolymerization and kinetic estimations of the formulations including evaluation of kinetic model are discussed.     

高温超导电缆的研发现状和发展趋势

与常规电缆相比,高温超导电缆具有体积小、重量轻、容量大、电流密度高、损耗低、环境友好等优势,为未来电网提供了一种新的电力传输方式。随着高温超导线材取得的重要研究进展,国际上相继开展了高温超导电缆的研发,已有多条超导电缆工程成功地进行了挂网示范运行。本文介绍高温超导电缆的研发进展情况,并简单对高温超导电缆的发展趋势和关键技术做出展望。     

Turbo编码类型的盲识别方法

提出一种有效的识别方法,解决了Turbo编码类型的盲识别问题。首先,在理论分析Turbo码固有结构的基础上,通过特定的拆分和组合方式从Turbo编码序列中获取一路递归系统卷积码,和二路线性分组码,从而根据此特性利用秩统计的方法实现Turbo码的判别;然后对递归系统卷积码输出数据进行差分运算,推导出差分后结果仅与寄存器中存放的数据有关,因此通过一阶累积量的方法可以准确获取归零状态的位置,从而实现对Turbo码的归零特性判别。仿真结果表明:该方法能够实现对Turbo编码类型的识别,且具有较好的容错性能,最后也验证了该方法在较高误码条件下具有较好的识别效果。     

酯化反应釜用全自动分水器

研制了一种结构简单、分水效率高的全自动油水分离器,介绍了其原理和设计计算方法。该分水器能实现水多出水、油多出油的连续排放,结合排出水的称量、记录、报警,有利于实现树脂生产反应深度、速度和终点提示,提高树脂生产装置的自动化水平。     

一款800V VDMOS终端结构的设计

设计了一款800 V VDMOS终端结构,采用场限环(FLR)与场板(FP)相结合的方式,在场限环上添加多晶硅场板与金属场板,有效地降低了表面电场峰值。通过调整终端结构,在135μm的有效终端长度上实现了848 V的击穿电压,最大表面电场为2.34×105 V/cm,小于工业界判断器件击穿场强标准(2.5×105 V/cm),且电场分布比较均匀,终端结构的稳定性和可靠性高。     

Strong Surface‐Termination Effect on Electroresistance in Ferroelectric Tunnel Junctions

Tunnel electroresistance in ferroelectric tunnel junctions (FTJs) has attracted considerable interest, because of a promising application to nonvolatile memories. Development of ferroelectric thin‐film devices requires atomic‐scale band‐structure engineering based on depolarization‐field effects at interfaces. By using FTJs consisting of ultrathin layers of the prototypical ferroelectric BaTiO₃, it is demonstrated that the surface termination of the ferroelectric in contact with a simple‐metal electrode critically affects properties of electroresistance. BaTiO₃ barrier‐layers with TiO₂ or BaO terminations show opposing relationships between the polarization direction and the resistance state. The resistance‐switching ratio in the junctions can be remarkably enhanced up to 10⁵% at room temperature, by artificially controlling the fraction of BaO termination. These results are explained in terms of the termination dependence of the depolarization field that is generated by a dead layer and imperfect charge screening. The findings on the mechanism of tunnel electroresistance should lead to performance improvements in the devices based on nanoscale ferroelectrics.     

Public interest and early termination of PPP contracts. Can fair and reasonable compensations be determined?

This article analyzes the different ways to terminate public-private-partnership (PPP) contracts early. It discusses how early terminations should be addressed to mitigate the consequences in terms of attractiveness, bankability, unfairness and litigation. The different ways to compensate private partners are examined. A new methodology for calculating the due compensation is proposed whenever governments voluntarily terminate a PPP contract based on the public interest. We posit that early termination clauses of contracts that comprise fair and reasonable compensations are required to provide meaningful incentives and contestability to acceptable returns on essential public services avoiding political and conflicting issues.     

化工企业员工退出机制探讨

从理顺人力资源劳动关系角度,探讨化工企业如何在法律法规的基础上,建立灵活规范的员工柔性退出机制,运用经济手段、协商方式,让有志于自我发展的富余员工走向市场,化工企业的用工制度走出一条切实可行的道路。     

最新特高压晶闸管

研究使晶闸管有效面积比极大化的阴极图形及结终端结构设计和工艺技术,实质性提高特高压(UH-V)晶闸管综合性能参数水平,易于折中协调突出重点性能参数。研制成6.3 kA/7.5 kV及5.5 kA/8.5 kV最新UHV晶闸管,可完全满足最新+800 kV,6.25 kA,10 GW及±1 100 kV,5.5 kA,12.1 GW换流阀的应用要求。