Translational Read-Through Therapy of RPGR Nonsense Mutations

X-chromosomal retinitis pigmentosa (RP) frequently is caused by mutations in the retinitis pigmentosa GTPase regulator (RPGR) gene. We evaluated the potential of PTC124 (Ataluren, Translama^TM) treatment to promote ribosomal read-through of premature termination codons (PTC) in RPGR. Expression constructs in HEK293T cells showed that the efficacy of read-through reagents is higher for UGA than UAA PTCs. We identified the novel hemizygous nonsense mutation c.1154T > A, p.Leu385* ({"type":"entrez-nucleotide","attrs":{"text":"NM_000328.3","term_id":"1677500221"}}NM_000328.3) causing a UAA PTC in RPGR and generated patient-derived fibroblasts. Immunocytochemistry of serum-starved control fibroblasts showed the RPGR protein in a dot-like expression pattern along the primary cilium. In contrast, RPGR was no longer detectable at the primary cilium in patient-derived cells. Applying PTC124 restored RPGR at the cilium in approximately 8% of patient-derived cells. RT-PCR and Western blot assays verified the pathogenic mechanisms underlying the nonsense variant. Immunofluorescence stainings confirmed the successful PTC124 treatment. Our results showed for the first time that PTC124 induces read-through of PTCs in RPGR and restores the localization of the RPGR protein at the primary cilium in patient-derived cells. These results may provide a promising new treatment option for patients suffering from nonsense mutations in RPGR or other genetic diseases.     

基于单神经元PID的真空炉自适应温度控制

根据真空热处理系统的特点,将单神经元PID控制算法应用到真空热处理系统的温度控制上。根据神经网络的非线性逼近能力和自学习自适应的特点,将单神经元网络与PID控制结合实现对真空炉温度的控制,以达到提高真空炉温度控制品质的目的。并通过计算机仿真软件进行仿真试验,仿真结果表明单神经元PID控制系统可以对控制参数自整定,其对温度控制更加稳健,具有更强的抗干扰能力和鲁棒性。经过搭建真空炉温度控制系统试验平台验证后发现,应用单神经元PID控制的真空炉系统的温升过程表现出了良好的稳定性,但是温度控制的响应速度和保温的精度略有下降。要想进一步提高温控品质,需要就单神经元PID控制方法在响应速度和控制精度上做进一步改进。     

热处理工艺对15CrMoR钢带状组织和冲击性能的影响

利用光学显微镜、扫描电镜和冲击试验机等仪器研究了15CrMoR钢中带状组织在热处理过程中的演变及其对冲击性能的影响。结果表明:奥氏体化后缓冷会出现铁素体-珠光体带状组织, 快冷则可以抑制带状组织的出现,但快冷会导致粒状贝氏体等非平衡组织形成。粒状贝氏体组织中的贝氏体铁素体基体和马氏体-奥氏体岛在回火过程中逐渐沿Cr、Mo等碳化物形成元素偏聚的区域分解出碳化物,形成富碳化物带,降低钢的横向冲击性能。15CrMoR钢加热到1100 ℃并保温7 h后可消除带状组织,改善钢的横向冲击性能。     

脉冲电磁处理参数对YT15硬质合金服役寿命的影响

采用COMSOL Multiphysics有限元分析软件对脉冲电磁处理设备进行仿真建模,利用正交试验的方法研究了磁通密度、磁脉冲个数、脉冲电压、电脉冲组数对YT15硬质合金刀具服役寿命的影响规律。通过极差分析得到了各因素的影响主次关系以及最佳脉冲电磁处理参数,并分析了切削过程的主切削力及磨损量变化,结果表明:脉冲电磁处理可使YT15硬质合金刀具服役寿命增长57.2%;最佳的处理参数组合为磁通密度2T,磁脉冲个数10,脉冲电压0.8V,电脉冲组数20;磁通密度和磁脉冲个数对其寿命影响较大,脉冲电压和电脉冲组数影响较弱;脉冲电磁处理后的刀具切削力较未处理有所降低,后刀面磨损速度明显减缓。     

小样产品试织时常见问题处理的探讨

本文结合多年的实践经验，针对纺织专业学生使用江阴通源生产的SGA598型半自动织样机设计试织时，出现的松紧边、锁边问题、张力控制等常见问题进行了探讨。     

Thermal treatment of waste photovoltaic module for recovery and recycling: Experimental assessment of the presence of metals in the gas emissions and in the ashes

The rapid expansion of the photovoltaic (PV) module market in the last years will determine in the near future a remarkable growth of corresponding waste. Then, the hazardous materials contained in the modules, such as Cd, Pb and Cr, could be released in the environment if the waste panels will not be handled adequately. Recycling processes of silicon crystalline panels, finalized to separate PV cells from the glass, involve the removal of the EVA (Ethylene Vinyl Acetate) layer through different methods, as the thermal treatment. During this treatment, some hazardous components can be released due to thermal degradation process. In this paper the metals released in the gas emissions and in the ashes due to the thermal treatment of modules were evaluated. For this purpose, three samples of crystalline panels were heated in furnace up to 600 °C and the complete degradation of the EVA was obtained. A mass balance between the sample and its components, before and after treatment, was performed in order to assess the weight loss percentage. Finally, after thermal treatment a qualitative analysis on the separated PV cell surface was performed by SEM-EDS (Scanning Electron Microscope equipped with Energy Dispersive Spectrometer).     

向家坝二纵围堰柔性结构段基础施工技术初探

向家坝水电站二期纵向围堰上游段座落于厚达45～62 m的覆盖层上，为提高接头段深厚覆盖层地基承载力，且保证导流期不同江水位时的堰体稳定，优化调整方案为，将接头段和导水段改为柔性结构。详细介绍了柔性结构段基础处理中所涉及的施工程序、施工技术和异常情况处理等。二纵围堰经过了2009年和2010年两个汛期的考验，安全监测显示与实际运行表明，柔性结构体结构稳定。将导水结构堰体改为柔性结构体，不仅节约了投资，而且保证了二期截流工程按施工总进度计划实施。     

Formation and Degradation of Beta-casomorphins in Dairy Processing

Milk proteins including casein are sources of peptides with bioactivity. One of these peptides is beta-casomorphin (BCM) which belongs to a group of opioid peptides formed from β-casein variants. Beta-casomorphin 7 (BCM7) has been demonstrated to be enzymatically released from the A1 or B β-casein variant. Epidemiological evidence suggests the peptide BCM 7 is a risk factor for development of human diseases, including increased risk of type 1 diabetes and cardiovascular diseases but this has not been thoroughly substantiated by research studies. High performance liquid chromatography coupled to UV-Vis and mass spectrometry detection as well as enzyme–linked immunosorbent assay (ELISA) has been used to analyze BCMs in dairy products. BCMs have been detected in raw cow's milk and human milk and a variety of commercial cheeses, but their presence has yet to be confirmed in commercial yoghurts. The finding that BCMs are present in cheese suggests they could also form in yoghurt, but be degraded during yoghurt processing. Whether BCMs do form in yoghurt and the amount of BCM forming or degrading at different processing steps needs further investigation and possibly will depend on the heat treatment and fermentation process used, but it remains an intriguing unknown.     

陕西煤灰纱出现的特征性规律与防治

煤灰纱严重影响纺织产品质量,同时也是难以解决的技术问题。文章分析了煤灰纱出现的特征性规律,试图找到防治煤灰纱的措施,从而找到切实可行的技术解决途径。