Exclusion of BMP6 as a candidate gene for cleidocranial dysplasia

Cleidocranial dysplasia (CCD) is an autosomal dominant, generalized skeletal dysplasia in humans that has been mapped to the short arm of chromosome 6. We report linkage of a CCD mutation to 6p21 in a large family and exclude the bone morphogenetic protein 6 gene (BMP6) as a candidate for the disease by cytogenetic localization and genetic recombination. CCD was linked with a maximal two-point LOD score of 7.22 with marker D6S452 at theta = 0. One relative with a recombination between D6S451 and D6S459 and another individual with a recombination between D6S465 and CCD places the mutation within a 7 cM region between D6S451 and D6S465 at 6p21. A phage P1 genomic clone spanning most of the BMP6 gene hybridized to chromosome 6 in band region p23-p24 using FISH analysis, placing this gene cytogenetically more distal than the region of linkage for CCD. We derived a new polymorphic marker from this same P1 clone and found recombinations between the marker and CCD in this family. The results confirm the map position of CCD on 6p21, further refine the CCD genetic interval by identifying a recombination between D6S451 and D6S459, and exclude BMP6 as a candidate gene.     

广播发射集散控制系统

本文介绍了广播发射台利用计算机技术、网络技术、遥控遥测技术、现代测试手段，对设备进行自动化控制与管理；实现设备技术指标自动测试，发射台无人值班、有人留守，建立机器的计算机档案制度，确保安全优质播出的方法和经验。     

粉末材料中微夹杂缺陷的超声波检验

介绍了用超声波方法检测微夹杂缺陷的一些试验结果，比较了不同频率、不同试验方法检测能力的差异，并通过解剖试验对检出缺陷的性质、尺寸作了比较。     

某微型起爆器引起的一起误爆事故分析

该文分析某微型起爆器引起的一起误爆事故，指出该起爆器设计上的缺陷及其对作业安全的威胁，提出防患建议。     

珠江三角洲土坝和堤围震害初步预测

本项预测采用概率方法,即首先根据广东省水利部门提出的55座土坝和59个重点的座标(见附图),进行概率地震危险性分析,给出它们在一定预测年限T(取50年和100年)的地震烈度Ⅰ的超越概率;其次根据近年我国发生的几次强震时处于不同烈度区内的土坝和堤防的震害资料,形成表示土坝和土堤地震易损性的震害概率矩阵;然后,根据上述两方面结果用概率公式评价各土坝和堤围点在预测年限内各震害等级的超越概率,并取10％超越概率为标准,估计震害等级。结果表明,珠江三角洲土工构筑物的地震危害性南部高于北部。     

Study on the dispersion characteristic of the plasma-loaded relativistic BWO

The dispersion characteristic of the plasma-loaded relativistic backward wave oscillator has been analyzed. The theoretical model has been established and the numerical calculations accord with the experimental results, which provides some useful suggestions on the designing of slow-wave structure of BWO.     

Modeling and control of magnet power supply system with switch-moderipple regulator

This paper presents the dynamic performance analysis and closed-loop design of a new power supply system for a high-current magnet load. This new system introduces a series switch-mode ripple regulator (SMRR) into a phase-controlled rectifier to improve system dynamic response and to reduce the harmonic content in the magnet current. In this paper, the new system control configuration is explained. The small signal dynamic model is established. The function of the SMRR as a speed-up feed-forward path during dynamic transients is assessed. The criteria of the closed-loop regulator design are identified. The theoretical analyses are accompanied by computer simulation and experimental results. The systematical design procedure is outlined by an example which offers better performance than ever reported on high-current magnet power supplies in terms of fast dynamic response (closed-loop bandwidth several kHz) and low harmonic content (less than 10 ppm) in the magnet current     

Molecular recognition with C-clamp porphyrins: synthesis, structural, and complexation studies

The cholesterolaemic effect of 2 hypercholesterolaemic diets was tested in 12 rat inbred strains. Diet I is a commercial diet supplemented with 2.0% (w/w) cholesterol and 5.0% (w/w) olive oil; diet II is identical to diet I with addition of 0.5% (w/w) sodium cholate. Strains with the highest plasma cholesterol response after diet I (BN and LEW) also had the highest cholesterol response after diet II (hyperresponders, mean response > 3.5 mmol/l). In the strains DA, SHR, BC, WAG, LOU, PVG and BUF the strain mean cholesterol response remained below 1.3 mmol/l after both diets (hyporesponders). Strains F344 and OM had an intermediate cholesterol response after both diets (normoresponders, mean response between 1.3 and 3.5 mmol/l). Only in the strains LOU, PVG and SHR there appeared to be a significant higher cholesterol response after diet II when compared with the cholesterol response after diet I. In the strain WKY this difference was of a borderline significance (P = 0.052) and this strain turned from a normoresponder after diet I into a hyperresponder after diet II. Liver cholesterol levels as measured after feeding diet II for two weeks also appeared to be strain-specific. No correlation was found between the plasma cholesterol response after diet II and the liver cholesterol levels. Changes in plasma phospholipid and triglyceride levels have been measured for both diet I and diet II. For group means a correlation between the cholesterol response and the change in phospholipid levels was found (r = 0.86 for diet I, P < 0.001 and r = 0.76 for diet II, P < 0.01). No such correlation was found for triglyceride levels.     

米塔尔钢铁集团的全球并购发展战略

概述了米塔尔的并购经过和并购特点,着重探讨了米塔尔的管理模式、人员情况、钢铁厂布局、生产经营、主要战略伙伴、研发机构及其生产、采购与销售策略。系统地分析了米塔尔在中国的发展,指出米塔尔在全球钢铁业整合中处于领先地位,米塔尔来中国投资看中的是中国的原料和市场,且运作步伐明显加快,会对中国钢铁企业构成很大的威胁。     

粘土矿物对低渗透储层性质的影响——以鄂尔多斯盆地三叠系低渗透储层为例

粘土矿物是低渗砂岩储层的主要胶结物，对储层物性及开发过程中储层物性变化趋势有着重要的影响和控制作用。另外，粘土矿物也蕴含着大量沉积环境介质的物理化学信息，认识其自然规律，可以有效地指导预测有利储层，认识和改造油层。以鄂尔多斯盆地砂岩储层中的粘土矿物为研究对象，探讨分析粘土矿物对低渗储层性质的影响，该研究对含油气盆地低渗储层有效开发具有一定的参考价值。