A Comprehensive Review of Genetically Engineered Mouse Models for Prader-Willi Syndrome Research

Prader-Willi syndrome (PWS) is a neurogenetic multifactorial disorder caused by the deletion or inactivation of paternally imprinted genes on human chromosome 15q11-q13. The affected homologous locus is on mouse chromosome 7C. The positional conservation and organization of genes including the imprinting pattern between mice and men implies similar physiological functions of this locus. Therefore, considerable efforts to recreate the pathogenesis of PWS have been accomplished in mouse models. We provide a summary of different mouse models that were generated for the analysis of PWS and discuss their impact on our current understanding of corresponding genes, their putative functions and the pathogenesis of PWS. Murine models of PWS unveiled the contribution of each affected gene to this multi-facetted disease, and also enabled the establishment of the minimal critical genomic region (PWScr) responsible for core symptoms, highlighting the importance of non-protein coding genes in the PWS locus. Although the underlying disease-causing mechanisms of PWS remain widely unresolved and existing mouse models do not fully capture the entire spectrum of the human PWS disorder, continuous improvements of genetically engineered mouse models have proven to be very powerful and valuable tools in PWS research.     

响应面法优化甘薯脆片干燥工艺

为探究甘薯脆片热泵干燥最佳工艺,在单因素试验基础上,以烘干温度、烘干时间、切片厚度、汽漂时间为影响因素,以含油率及感官评分为响应值,用Box-Behnken试验设计建立响应面分析模型。结果表明,甘薯脆片烘干最佳工艺为:烘干温度74℃、切片厚度2.7 mm、汽漂时间3 min,烘干时间为3.5 h,在此优化条件下,甘薯脆片油炸后感官评分为88.75分,含油率为7.84%,在此条件下得到的产品色香味俱全。     

大涡模拟Smagorinsky模型用于磨粒流精密加工喷嘴的质量控制研究

为研究固液两相磨粒流加工喷嘴小孔过程中的流场分布、涡旋形成规律及涡旋的存在对磨粒流加工的影响机制，采用Smagorinsky亚格子模型对磨粒流加工喷嘴小孔的流道进行大涡数值模拟，并使用磨粒流对变直径喷嘴工件进行加工试验。数值模拟发现磨粒流流体中磨粒与壁面的碰撞与剪切作用随流体的速度增大而增大，同一截面的速度存在速度差，其中还伴随涡旋的存在；通过试验研究发现：经固液两相磨粒流加工后的喷嘴小孔表面质量得到明显提高，喷嘴经过四次不同入口速度的磨粒流加工后大孔处表面粗糙度Ra由1.24 μm降至0.542 μm，小孔处表面粗糙度Ra由1.21 μm降至0.437 μm。结论显示固液两相磨粒流加工技术可有效提高被加工喷嘴工件的内表面质量，加工时同一截面的速度存在速度差，速度差的存在利于涡旋的形成，涡旋的存在利于提高磨粒流加工过程的剪切作用，有助于获得高质量的喷嘴小孔内通道表面。     

Suppression of forced vibration due to chip segmentation in ultrasonic elliptical vibration cutting of titanium alloy Ti–6Al–4V

Ultrasonic elliptical vibration cutting of titanium alloy Ti–6Al–4V is investigated in this research. Because products made of Ti–6Al–4V alloy are usually designed for possessing low-rigidity structures or good-quality cut surfaces, machining requirements such as low cutting forces and slow rate of tool wear need to be fulfilled for realization of their precision machining. Therefore, the ultrasonic elliptical vibration cutting is applied as a novel machining method for those products. Machinability of Ti–6Al–4V alloy by the ultrasonic elliptical vibration cutting with cemented carbide tools is examined to figure out suitable cutting conditions for precision machining of Ti–6Al–4V alloy. As experimental results, generated chips, cutting forces, and profiles of cut surfaces are indicated. A forced vibration problem occurred due to the segmented chip formation, which is also well-known in the ordinary non-vibration cutting. Therefore, characteristics of the forced vibration due to the chip segmentation are investigated in this research. Through the experiments, it is found that the frequency and magnitude of the forced vibration have relation with the average uncut chip thickness and cutting width. Especially, it is found that the averaging effect can suppress the forced vibration, i.e. the chip segmentation tends to occur randomly over the large cutting width, and hence the force fluctuations with random phases tend to cancel each other as the cutting width increases relatively against the average uncut chip thickness. Based on the investigations, a new practical strategy to suppress the forced vibration due to chip segmentation is proposed and verified. Using the proposed method significantly decreased cutting forces and good quality of surfaces are obtained when the forced vibration is suppressed compared to the ordinary non-vibration cutting results. Therefore, the results suggest that the precision machining can be realized without sacrificing the machining efficiency by increasing the width of cut and decreasing the average uncut chip thickness.     

Control Model for Large‐Sized Gantry Type Linear Motor Slider

At present, many industrial carrier devices utilize linear motor sliders. However, a heavier load requires a linear motor (which is an example of a direct drive device) to get higher power, compared with a rotary motor with a ball screw slider (which is an example of an indirect drive device). In order to obtain higher power at a lower cost, a linear slider with multiple motors can be utilized, for example, a gantry type linear motor slider. Moving the gantry type slider requires two linear motors that are set up in parallel to enable synchronization control. Some conventional synchronization control methods have been proposed for the parallel twin linear slider; however, a large‐scaled gantry type linear motor slider has two unique problems: mechanical distortion caused by the limitations of installation environment and coupling caused by joints with low stiffness. This paper proposes a control model to solve these problems, and an identification method of each parameter. Furthermore, the effectiveness of the control model is verified by comparison with simulation results and experimental results.     

门架式堆取料机在铜冶炼中的生产实践

针对铜冶炼工艺的特点,从门架式堆取料机对前后工序衔接、结构、原理出发,介绍了其在熔炼炉系统和吹炼炉系统中的应用情况。经过试生产期的生产实践证明,门架式堆取料机具有场地利用率高、取料量大,自动化程度高和安全环保等优点。     

枞树型核电轮槽精铣刀制造技术优化设计

以枞树型叶根精铣刀为例,阐述了可换硬质合金型线刀体和刀片精铣刀的优化设计、制造工艺、建模及优化设计,进行切削过程加工策略优化和加工过程模拟,设计制造了锥柄专用工装及检测环规、磨削刀片后角专用工装,采用NUMROTO软件编制刀片加工工步。该项目研制的可换刀片精铣刀型线精度完全能达到进口铣刀水平,能满足大部分轮槽与叶根加工对型线精度的要求,可以大幅度节约刀具费用。     

Investigations of geometrical structure and morphology of samples made of hard machinable materials after wire electrical discharge machining and vibro‐abrasive finishing

In this article the results concerned influence of processing conditions of the wire electrical discharge machining and vibro‐abrasive machining on the surface layer and morphology of samples made of hard machinable materials used in aircraft industry like: Titanium 5553 β, Inconel 617, Hastelloy X and Magnesium AZ31 have been presented. For this purpose the cubic and cylindrical samples made of hard machinable alloys have been prepared using optimal electric parameters of wire‐cut electrical discharge machining and finally they have been polished using circular vibratory finishing technology and different ceramic shaped stones.     

Wire electrical discharge machinable SiC with GNPs and GO as the electrically conducting filler

SiC based composites filled with graphene nano-platelets (GNPs) or graphene oxide (GO) prepared by rapid hot-pressing exhibit sufficient electrical conductivity for their machinability by wire electro-discharge machining (WEDM). Composites microstructure anisotropy caused by graphene alignment as a consequence of rapid hot pressing was confirmed by measuring of electrical conductivity and thermal diffusivity. Electrical conductivity increased significantly with increased weight fraction of graphene in both measured directions. Highest value of 2031 S/m was obtained for composites with 15 wt. % of GNPs in parallel direction and only 1246 S/m in perpendicular direction to aligned GNPs. Thermal diffusivity is 63.3 mm²/s in parallel and only 23.3 mm²/s in perpendicular direction. The increase of the electrical conductivity has resulted in successful WEDM. The MRR was almost doubled when the filler concentration increased from 5 wt. % GNPs/GO to 15 wt. % GNPs. At the same time, the surface roughness decreased.