ARSACS, a spastic ataxia common in northeastern Québec, is caused by mutations in a new gene encoding an 11.5-kb ORF

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS or SACS) is an early onset neurodegenerative disease with high prevalence (carrier frequency 1/22) in the Charlevoix-Saguenay-Lac-Saint-Jean (CSLSJ) region of Quebec. We previously mapped the gene responsible for ARSACS to chromosome 13q11 and identified two ancestral haplotypes. Here we report the cloning of this gene, SACS, which encodes the protein sacsin. The ORF of SACS is 11,487 bp and is encoded by a single gigantic exon spanning 12,794 bp. This exon is the largest to be identified in any vertebrate organism. The ORF is conserved in human and mouse. The putative protein contains three large segments with sequence similarity to each other and to the predicted protein of an Arabidopsis thaliana ORF. The presence of heat-shock domains suggests a function for sacsin in chaperone-mediated protein folding. SACS is expressed in a variety of tissues, including the central nervous system. We identified two SACSmutations in ARSACS families that lead to protein truncation, consistent with haplotype analysis.     

基于JMASE的地空导弹联合建模与仿真

以六自由度地空导弹拦截仿真为背景,阐述联合建模与仿真环境(JMASE)的基本概念和功能描述,介绍基于JMASE-SIMULINK的工程级与交战级一体化联合建模与仿真方法,给出某地空导弹全系统模型及交战仿真结果。仿真实践表明:通过将SIMULINK模型自动转换为JMASE兼容的C 模型组件,并在JMASE环境下组装、配置和运行,则获得SIMULINK工程级武器系统建模能力的同时,也获得JMASE提供的具有一致时空及环境效应描述的交战级仿真能力,既提高了建模与仿真的效率,又促进了模型重用和互操作,是实现工程级与交战级联合建模与仿真的有效方法。     

Morphology of polar ionospheric O^＋ ion upflow： FAST observations during quiet time

With energetic ion measurements on FAST satellite, the morphologic features of quiet-time ionospheric O ion upflowing at altitudes of 2000 to 4000 km are drawn out for the first time. The pre-noon cusp/cleft is the predominant region of upflowing occurrence for O with lower energy. Meanwhile the pre-dawn sector near the equatorward edge of the plasma convection dominates the occurrence for the higher energy O ions. No matter whether the energy is lower or higher, the upflows occur often over a wide MLT range of lower latitudes outside the auroral oval. The upflowing within the pre-midnight (21:00― 22:00 MLT) auroral oval carrys larger energy fluxes, with extremely large fluxes for higher energy O appearing near the polar cap boundary. For altitudes of 2000―4200 km under observation, the ion conics occur much more frequently than ion beams. Ion beams are rarely found below 3000 km, while the conics occur uniformly over the observed altitudes.     

基于DDS技术的FM信号发生器的设计及其FPGA实现

以FPGA为主要硬件,采用直接数字频率合成技术结合嵌入式锁相环,开发出了一种具有数字调制功能的FM信号发生器,并在自行研制的ALTERA Cyclone实验板上得到实现.经调试,该信号发生器的频率分辨率为0.596 HZ,最高输出载波频率达到10 MHz,同时具有输出相位连续,抗干扰能力强等优点.     

OCS命令流解析器的设计与实现

通过分析LAMOST观测控制系统中的命令流的解析方法,依据LAMOST科学目标的要求和观测运行的特点,利用数据库映射技术,提出了命令解析模型.在该模型的基础上,进一步从数据库和解析过程两方面进行设计.最终实现了各层命令集之间的解析.     

Pseudo-particle modeling for gas flow in microchannels

The velocity profiles and temperature distributions of gas flow in microchannels, for Knudsen numbers ranging from 0.01 to 0.20, are investigated with pseudo-particle modeling (PPM). It has been found that the velocity profiles are mainly affected by Knudsen number and the external force fields applied. When Knudsen number was increased, the slip velocities on the walls increased at the beginning, and then decreased. The temperature distributions were also significantly affected by the external force. The Darcy friction factor increased with increasing Knudsen number, and its variation with Mach number under increased Knudsen number was similar to the so-called premature laminar-turbulent transition observed in experiments.     

Experimental investigation of incremental reactivity of di-tert-butyl peroxide

Large quantities of di-tert-butyl peroxide (DTBP) have been emitted into the troposphere due to human activities. Its role in the atmospheric photochemical reaction has not been understood. This study presents the results of the photochemical reactions of DTBP and NOx, which have been simulated in a self-made smog chamber under the temperature of (29±1)℃. Both the wall decays of ozone and NO2 could be neglected, compared to the results in simulative experiments. The effective intensity of UV light used in the experiments was 1.28×10-3 s-1, which was expressed by the rate constant of NO2 photolysis in purified air. The reaction mechanism was proposed according to our results and reports of other researchers. The maximum values of incremental reactivity (IR) in the three simulative ex- periments were 9.53×10-2, 5.23×10-2 and 3.78×10-2, respectively. The incremental reactivity decreased with the increase of initial concentrations of DTBP. The IR value of DTBP obtained in this study was comparable to that of acetylene reported in our previous research.     

浅谈工业产品质量指标在企业管理中的作用

介绍了工业产品质量指标的构成,论述了我国工业产品质量指标的历史,探讨了工业产品质量指标在企业管理中的作用。     

不同负荷游泳训练对大鼠红细胞膜的影响

目的探讨不同负荷游泳训练对大鼠的运动能力、抗氧化酶SOD的活性、脂质过氧化产物MDA含量、Na -K -ATP酶和Ca2 -ATP活性的影响。方法选取雄性SD大鼠60只,随机分为三大组,即对照组(C组,20只)、小负荷游泳训练组(L组,20只)、大负荷游泳训练组(H组,20只),测试不同负荷游泳训练对大鼠红细胞膜抗氧化能力和ATP酶活性的影响。结果小负荷的运动训练可以降低安静时红细胞膜上MDA含量,提高抗氧化酶SOD活性,大负荷的训练则产生相反的结果;力竭运动后小负荷训练组红细胞膜Na -K -ATP酶活性增加,大负荷训练组Na -K -ATP酶活性下降;力竭运动后即刻各组RBCM上Ca2 -ATP酶活性都有下降,安静时小负荷的运动训练组Ca2 -ATP酶活性提高,但没有显著性差异,大负荷的运动训练使Ca2 -ATP酶活性显著下降。结论小负荷的运动训练可以提高机体的运动能力,而大负荷的训练会对机体产生不良影响;适宜负荷的运动训练不但可以提高安静时酶的活性,而且可以提高酶活性的储备力,延缓运动性疲劳的发生及抵抗力竭运动对机体造成运动损伤。     

论明清工商会馆的经济管理功能

通过对目前检索到的196所山陕会馆资料的分析，论证了工商会馆在中国现代化征兆出现的历史条件下，所发挥的经济管理功能．认为工商会馆是富有中国特色的经济和社会管理制度的创新，其对中国早期市场经济因素的产生具有重要的推动作用．