Mutations in NMNAT1 cause Leber congenital amaurosis and identify a new disease pathway for retinal degeneration

Leber congenital amaurosis (LCA) is a blinding retinal disease that presents within the first year after birth. Using exome sequencing, we identified mutations in the nicotinamide adenine dinucleotide (NAD) synthase gene NMNAT1 encoding nicotinamide mononucleotide adenylyltransferase 1 in eight families with LCA, including the family in which LCA was originally linked to the LCA9 locus. Notably, all individuals with NMNAT1 mutations also have macular colobomas, which are severe degenerative entities of the central retina (fovea) devoid of tissue and photoreceptors. Functional assays of the proteins encoded by the mutant alleles identified in our study showed that the mutations reduce the enzymatic activity of NMNAT1 in NAD biosynthesis and affect protein folding. Of note, recent characterization of the slow Wallerian degeneration (Wld(s)) mouse model, in which prolonged axonal survival after injury is observed, identified NMNAT1 as a neuroprotective protein when ectopically expressed. Our findings identify a new disease mechanism underlying LCA and provide the first link between endogenous NMNAT1 dysfunction and a human nervous system disorder.     

国家治理现代化与企业战略重构

公共治理作为外部治理机制影响企业的战略决策行为。我国当前最突出的公共治理改革就是建设现代化的国家治理体系。基于产权性质与政治关联的视角检验了国家治理变迁后的企业战略重构行为。在整体上发现了国家治理现代化建设以来我国上市公司战略重构的证据;有政治关联的企业更能顺应环境变化及时进行战略调整;国有产权推动了国家治理现代化建设以来企业的战略重构,但是其作用区域主要体现在缩减关系型战略支出方面,国有企业战略重构的主动性不足。本文的研究可用以评价国家治理现代化建设的效率与效果,为未来改革与发展的路径安排提供经验依据,为企业未来可持续发展提供对策建议。     

Generating semi-algebraic invariants for non-autonomous polynomial hybrid systems

Hybrid systems are dynamical systems with interacting discrete computation and continuous physical processes, which have become more common, more indispensable, and more complicated in our modern life. Particularly, many of them are safety-critical, and therefore are required to meet a critical safety standard. Invariant generation plays a central role in the verification and synthesis of hybrid systems. In the previous work, the fourth author and his coauthors gave a necessary and sufficient condition for a semi-algebraic set being an invariant of a polynomial autonomous dynamical system, which gave a confirmative answer to the open problem. In addition, based on which a complete algorithm for generating all semi-algebraic invariants of a given polynomial autonomous hybrid system with the given shape was proposed. This paper considers how to extend their work to non-autonomous dynamical and hybrid systems. Non-autonomous dynamical and hybrid systems are with inputs, which are very common in practice; in contrast, autonomous ones are without inputs. Furthermore, the authors present a sound and complete algorithm to verify semi-algebraic invariants for non-autonomous polynomial hybrid systems. Based on which, the authors propose a sound and complete algorithm to generate all invariants with a pre-defined template.     

基于模拟有限差分的嵌入式离散裂缝数学模型

嵌入式离散裂缝模型划分网格时不需要考虑油藏内的裂缝形态,只需对基岩系统进行简单的网格剖分,可以大大降低网格划分的复杂度,从而能够提高计算效率.并且该模型可以将现有成熟的油藏数值模拟技术和离散裂缝网络模型有机地结合起来,能精细地模拟流体在裂缝性油藏中的流动.本文模型求解采用模拟有限差分方法,该方法基于单个网格的节点和面信息构造数值计算格式,理论上适用于任何复杂网格系统,且具有良好的局部守恒性,将其推广到嵌入式离散裂缝模型后,克服了该模型基于有限差分方法求解时不能有效处理全张量形式的渗透率以及不适用于复杂边界形状裂缝性油藏的局限性.最后通过实际算例验证了本文方法的正确性和优越性.     

东濮凹陷胡庆地区原油地球化学特征与油源分析

研究东濮凹陷胡庆地区原油地球化学特征及其来源.利用有机地球化学分析方法,对原油与烃源岩进行饱和烃色谱、饱和烃与芳烃色谱-质谱分析,剖析了两者的分子地球化学特征及油源.研究表明:胡庆地区发育3类原油,第一类原油具有极高伽马蜡烷,质量色谱图中甾烷呈"L"形特征,来自第二台阶的沙一段烃源岩,在沙一段储层中富集形成自生自储常温...     

纳米复合技术改善工业ZnO压敏电阻避雷器微观结构及电性能的研究

运用纳米复合技术对工业ZnO压敏电阻避雷器的制备工艺的改进,研制出压敏电压340 V/mm,漏电流小于1μA,非线性系数大于56的压敏电阻器。根据ZnO晶粒生长动力学方程,研究了纳米复合ZnO粉体的烧结过程以及烧结过程中的晶粒生长规律,用扫描电镜和能谱对瓷体显微结构及面扫成分进行了分析,讨论了添加纳米复合粉的ZnO压敏电阻避雷器微观组织与综合性能的关系。实验表明,添加纳米复合粉体是降低大规模生产成本,提高经济效益,并得到综合性能优良产品的有效方法。     

基于淀粉经济、无毒的比色潜指纹检测

构建了一种基于淀粉的经济、无毒、实时的潜指纹比色检测方法.利用淀粉与指纹之间的氢键和范德华力作用,使淀粉有效识别出无孔和多孔基底上的潜指纹.通过将不同颜色的植物染料混合进淀粉,解决了由淀粉与基底之间顺色导致的检测对比度差的难题.该方法还表现出对老化潜指纹和重叠潜指纹的良好检测效果.     

基于因子分析的陕西省城市投资环境综合评价

选取自然资源环境、经济环境、基础设施环境、社会服务环境、人口劳动力环境和社会文化环境6个子系统的18个指标构建了城市投资环境指标体系,以陕西省下属的10个地级市为研究对象,采用因子分析的方法,对陕西省城市投资环境进行了实证分析。     

Spatial prisoner’s dilemma games with increasing size of the interaction neighborhood on regular lattices

We studied the evolution of cooperation in the prisoner’s dilemma game on a square lattice where the size of the interaction neighborhood is considered. Firstly, the effects of noise and the cost-to-benefit ratio on the maintenance of cooperation were investigated. The results indicate that the cooperation frequency depends on the noise and cost-to-benefit ratio: cooperation reaches a climax as noise increases, but it monotonously decreases and even vanishes with the ratio increasing. Furthermore, we investigated how the size of the interaction neighborhood affects the emergence of cooperation in detail. Our study demonstrates that cooperation is remarkably enhanced by an increase in the size of the interaction neighborhood. However, cooperation died out when the size of the interaction neighborhood became too large since the system was similar to the mean-field system. On this basis, a cluster-forming mechanism acting among cooperators was also explored, and it showed that the moderate range of the neighborhood size is beneficial for forming larger cooperative clusters. Finally, large-scale Monte Carlo simulations were carried out to visualize and interpret these phenomena explicitly.     

两水平部分因析设计的最小低阶效应混杂准则(英文)

根据效应分层原理,低阶效应比高阶效应更重要.在这种情况下,以别名集的结构为基础,提出了最小低阶效应混杂准则对2n-m设计进行排序.研究了新准则与其它最优性准则,即最大分辨度、最小低阶混杂、纯净效应和最大估计容量准则的联系.给出了具有16和32个水平组合的最小低阶效应混杂设计.