Biochemical defects in ABCR protein variants associated with human retinopathies

Mutations in the gene encoding ABCR (ABCA4), a photoreceptor-specific ATP-binding cassette (ABC) transporter, are responsible for autosomal recessive Stargardt disease (STGD), an early onset macular degeneration, and some forms of autosomal recessive cone-rod dystrophy and autosomal recessive retinitis pigmentosa. Heterozygosity for ABCA4 mutations may also represent a risk factor for age-related macular degeneration (AMD), although this idea is controversial. An ongoing challenge in the analysis of ABCA4-based retinopathies arises from the observation that most of the ABCA4 sequence variants identified so far are missense mutations that are rare in both patient and control populations. With the current sample size of most sequence variants, one cannot determine statistically whether a particular sequence variant is pathogenic or neutral. A related challenge is to determine the degree to which each pathogenic variant impairs ABCR function, as genotype-phenotype analyses indicate that age of onset and disease severity correlate with different ABCA4 alleles. To address these questions, we performed a functional analysis of human ABCR and its variants. These experiments reveal a wide spectrum of biochemical defects in these variants and provide insight into the transport mechanism of ABCR.     

倒立摆控制方法的研究

运用牛顿动力学方法对单级倒立摆系统进行了数学建模,并对基于经典控制理论的PID控制法、基于自适应神经网络模糊控制方法进行了仿真对比分析研究。     

一类不确定时滞系统的滑模控制

考虑了一类同时存在状态不确定性和控制不确定性的时滞系统的滑模控制问题,通过采用李亚普诺夫方法和线性矩阵不等式(LMI)技术,给出了闭环系统渐近稳定的充分条件,而且滑模控制律和滑模面的设计可以利用线性矩阵不等式的解得到,证明了该滑模控制律能保证状态轨迹被驱动到指定的切换面上。仿真实验表明了算法的有效性。     

变点理论在极值分布阈值选取中的应用

极值理论已经被广泛应用于解决金融、保险等领域的厚尾分布问题，而阚值的选取一直是极值理论实际应用的难点和重点。在已有研究成果基础上。本文尝试引入变点理论进行定量化的阈值选取。分析了变，最理论进行阚值选取的基本原理和方法，利用Burr分布产生的随机样本进行模拟，得到了较好的效果。并在此基础上运用S＆P500和Danish火灾保险数据进行了实证分析。     

几类组合恒等式的概率证法

本文通过建立概率模型,分别运用完备事件组、全概率公式及随机变量的数字特征等方法,举例证明了几类组合恒等式;既是概率论在证明组合恒等式方面的广泛应用,又充分展现了概率证法的独到之处。     

Reverse Programmed SONOS Memory Technique for 0.18 μm Embedded Utilization

A 4 Mb embedded silicon-oxide-nitride-oxide-silicon (SONOS) memory was developed with a 0.18 μm CMOS logic compatible technology. A reverse programming array architecture was proposed to reduce the chip area, enhance the operating window, and increase the read speed. The charge distribution was analyzed to optimize the programming and erase conditions considering both the operating speed and the endurance performance. The final test chip has a good endurance of 105 cycles and a data retention time of at least 10 years.     

Enolate chelating N-heterocyclic carbene complexes of Fe(II): Synthesis, structure and their catalytic activity for ring-opening polymerization of ε-caprolactone

The reaction of anhydrous FeBr2 with two equivalents of anionic N-heterocyclic carbene (NaL1 and NaL2), which are generated in situ by the reaction of the corresponding salt [4-R-C6H4COCH2{CH- (NCHCHNiPr)}Br] (R = OCH3, H2L1Br, 1; R = F, H2L2Br, 2) with two equivalents of NaN(SiMe3)2, affords bis-ligand Fe(II) complexes of L12Fe (3) and L22Fe (4) in high yield, respectively. Attempt to synthesize mono-ligand Fe(II) bromide by the 1:1 molar ratio of NaL to FeBr2 is unsuccessful, and the same com- plexes of 3 and 4 were obtained. Both 3 and 4 have been depicted by elemental analysis and X-ray structure determination. Preliminary studies show that both 3 and 4 can be used as single-component catalyst for the ring-opening polymerization of ε-caprolactone, and the catalytic activity of 3 is higher than that of 4.     

一类相关类负风险和风险过程的破产概率

引入一类相关负风险和风险过程,负风险和类之间的相关性定义为稀疏相关结构,主要研究类之间的相关性对破产概率的影响.     

山西省入境旅游市场分析及发展对策

入境旅游是旅游业的重要组成部分.对入境旅游市场的研究分析,是制定行之有效的旅游发展战略的前提.本文主要采用市场竞争态模型定量分析了山西省近几年入境旅游客源市场和目的地市场的竞争态势,并对入境旅游市场竞争态进行动态分析.结果表明：①目前山西入境旅游客源市场发育不成熟,仅少数近程客源地市场份额较大;②山西入境旅游目的地市场发展不平衡,市场范围有待扩展;③山西省在全国入境旅游市场中的地位逐步上升.在分析的基础上,提出了拓展山西入境旅游市场的对策.     

中频交流脉冲电源的研制

研制了150kHz中频交流脉冲电源,给出了中频交流脉冲电源的设计思路和实现方案.该电源采用全桥逆变拓朴电路、PWM控制方式、输出功率叠加的方案,具有输出变压器原边平均电流和副边峰值电流的过流保护功能.其输出峰值电压0~1200V连续可调,频率1~150kH z可调,脉宽占空比10%~40%可调,输出平均电流0~5A,最大输出功率5kW.利用该电源进行了等离子体化学气相沉积和反应溅射实验,证明该电源各项指标运行正常,工作稳定,具有良好的应用价值.