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1.
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.  相似文献   
2.
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.  相似文献   
3.
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。  相似文献   
4.
贾双荣 《建筑电气》2013,(12):35-37
介绍武汉二七长江大桥的直击雷防护、侧击雷防护措施和电涌保护器的设置.以及接地设计、接地电阻的检测、施工要求等。重点介绍大桥利用基础桩基内外边缘的结构钢筋(包括钢护筒)作为接地板的做法。  相似文献   
5.
阐述了常用的电梯超载保护装置的型式和目前的检测方法。针对配备机房称重式加载保护装置的电梯难以利用普通模拟加载装置进行检测的问题,设计了一套新型电梯模拟加载装置,并介绍了其相应的测试方法。该加载装置具有操作方便、简单易携.精度高.重复性好.容错率高等优点。  相似文献   
6.
针对原始遍历式匹配模式算法的时间复杂度高以及给视频图像目标跟踪系统的计算能力带来极大考验的问题,提出一种基于提前终止判决的归一化互相关匹配算法(zero-mean normalized cross correlation based on early termination condition,ZNCC-ETC).在基于盒子滤波优化的遍历式ZNCC匹配算法(ZNCC based on Box-Filtering,ZNCC-BF)的基础上,发现ZNCC-BF算法在遍历匹配过程中存在着一个适当的阈值,一旦模板图与候选图的互相关累加值超过这个阈值,则后续任何计算即为冗余计算,而ZNCC-ETC则采用提前终止判决法实现图像的加速匹配跟踪,并通过对比实验进行验证与分析跟踪结果.分析结果表明:相对于标准ZNCC以及改进的ZNCC-BF,ZNCC-ETC算法能够在保证全局最大值收敛能力的前提下,进一步降低匹配过程中的计算量,实现加速匹配跟踪的目的.  相似文献   
7.
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.  相似文献   
8.
High Efficiency Video Coding (HEVC) is a new video coding standard achieving about a 50% bit rate reduction compared to the popular H.264/AVC High Profile with the same subjective reproduced video quality. Better coding efficiency is attained, however, at the cost of significantly increased encoding complexity. Therefore, fast encoding algorithms with little loss in coding efficiency is necessary for HEVC to be successfully adopted for real applications. In this paper we propose a fast encoding technique applicable to HEVC all intra encoding. The proposed fast encoding technique consists of coding unit (CU) search depth prediction, early CU splitting termination, and fast mode decision. In CU search depth prediction, the depth of encoded CU in the current coding tree unit (CTU) is limited to predicted range, which is mostly narrower than the full depth range. Early CU splitting skips mode search of its sub-CUs when rate distortion (RD) cost of current CU is below the estimated RD cost at the current CU depth. RD cost and encoded CU depth distribution of the collocated CTU of the previous frame are utilized both to predict the encoding CU depth search range and to estimate the RD cost for CU splitting termination. Fast mode decision reduces the number of candidate modes for full rate distortion optimized quantization on the basis of the low complexity costs computed in the preceding rough mode decision step. When all these three methods are applied, proposed fast HEVC intra encoding technique reduces the encoding time of the reference encoder by 57% on the average, with only 0.6% of coding efficiency loss in terms of Bjontegaard delta (BD) rate increase under the HEVC common test conditions.  相似文献   
9.
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。  相似文献   
10.
谭剑云 《上海化工》2012,37(9):20-23
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。  相似文献   
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