Protein C Osaka 10 with aberrant propeptide processing: loss of anticoagulant activity due to an amino acid substitution in the protein C precursor

We studied the molecular basis of protein C deficiency in a family with a history of thromboembolic disease. An approximately 50% reduction in anticoagulant activity despite normal levels of protein C amidolytic activity and antigen was detected in plasma from the proband. All the exons and intron/exon junctions of the protein C gene were studied using a strategy that combined polymerase chain reaction amplification with DNA sequencing of the amplified fragments. We identified a C-to-A change at nucleotide number 1387 of the protein C gene in the proband and his mother, and this mutant was designated protein C Osaka 10. The C-to-A change resulted in the substitution of Ser for Arg at position -1, which is the processing protease cleavage site. The mutant protein C was partially purified from plasma of the patient's mother using barium adsorption followed by ion-exchange column chromatography. It eluted at the same sodium chloride concentration as normal protein C, and thus gamma-carboxylation of the mutant protein appeared to be normal. The apparent molecular weight of this mutant protein C was the same as that of the normal protein on immunoblotting. Amino-terminal sequence analysis showed that the light chain of the mutant protein C had an additional Ser at position-1. Thus, the loss of anticoagulant activity of protein C Osaka 10 can be explained by alteration of the conformation of the Gla domain by the additional Ser in the mutant molecule.     

铝锂合金力学性能的各向异性

针对当前困扰铝锂合金实际应用的各向异性问题，概述关于铝锂合金中各向异性的表现规律，产生机理及对于降低各向异性所采用的方法等方面的研究现状和进展，对铝锂合金各向异性表现与微观组织结构的关系以及目前克服各向异性所采用方法的局限性进行分析。就此提出，若彻底解决这一问题则必须采用更先进的合金化方法及热加工工艺，研制新一代铝锂合金的观点和研究方向。     

Inter- and intra-molecular ionic interactions of polyampholyte: Carboxymethyl-2-diethylaminoethylcellulose

Carboxymethyl-2-diethylaminoethylcellulose (CM-DEAE cellulose) was prepared by etherification of carboxymethylcellulose with diethylaminoethyl chloride in a NaOH solution. The behaviour of CM-DEAE cellulose in aqueous solution was studied by viscosity and GPC measurements. The degree of substitution (DS) and existing states of DEAE substituents were examined by the use of proton NMR, and those of the CM substituents were observed with FT-IR. The results reveal that the reduced viscosity and apparent molecular size of CM-DEAE cellulose vary with the concentration of sodium chloride and changes in pH and DS. These phenomena can be explained in terms of inter- and intra-molecular ionic interactions.     

Crystallization behaviour of Ni-Co-base metallic glasses by isochronal and isothermal annealing

The crystallization behaviour of three amorphous alloys, Co₅₀Ni₂₅Si₁₅B₁₀, Ni₅₀Co₂₅Si₁₅B₁₀ and Ni₅₀Co₂₅P₁₅B₁₀, was studied by means of differential thermal analysis in conjunction with scanning transmission electron microscopy. Isochronal annealing showed a strong dependence of crystallization on scan rate over the range of 1.99 to 20.70 K min^–1. At high Co/Ni ratios, a sequential two-stage crystallization process involving primary MS-I phase followed by MS-II phase precipitation was observed. At low Co/Ni ratios MS-I and MS-II crystallization were concurrent and inseparable. Replacement of the metalloid Si with P as the glass-former dramatically reduced the activation energy for crystallization as well as the crystallization temperature. A mechanistic understanding of these findings was pursued in light of TEM/STEM microanalysis     

AVO 异常检测技术及其应用

本文介绍了一系列简单而实用的 AVO 异常显示、检测和增强的新技术。包括:共炮检距(或相近)显示技术、振幅比值显示技术、滚动显示技术、线性转换显示技术、抛物线转换显示技术和 AVO 增强技术。文中以实例说明了上述各种方法的应用情况。     

浅谈大干电站技改工程施工建设管理

大干水电站技改工程从开工建设至首台机组发电历时17个月,建设速度在同类型电站中算相当快的。从工程概况、工程建设及科学管理的角度介绍大干水电站技改工程建设管理的经验。     

提高延迟焦化装置苛刻度的探索

介绍了镇海炼化延迟焦化装置为提高苛刻度所采取的降低循环比、缩短生焦时间、提高加热炉出口温度等措施情况。实施降低循环比的措施后,液体产品收率增加了1.96%,焦炭收率下降了1.7%,处理量提高了4.7%。缩短生焦周期后,Ⅰ、Ⅱ套焦化装置的处理量分别提高了6.2%、7.2%。同时,炉出口温度需根据不同的原料油性质和不同的产品要求来确定。对于炉管烧焦周期,应以经济性为原则,科学权衡利弊后确定。介绍了加强除焦、行车设备维护保养所采取的一些措施。     

CARS光谱测温及仪器狭缝函数对测量的影响

设计了一种利用光参量振荡器(OPO)来代替染料激光器的宽带相干反斯托克斯喇曼散射(CARS)光谱测温实验装置。为了提高CARS测量温度的精度,测量和分析了检测系统中狭缝函数对测量结果的影响,确定了狭缝函数的具体形式。将测量结果与热电偶读数比较,发现在温度范围300K～1200K内,温度测量的不确定度小于2%。     

可编程序控制器在广告中的应用

目前厂家在销售商品时都运用广告进行宣传。广告的形式一般有这么几种:电视上的立体画面及声音;广播电台中声音宣传;街头或商场中的大屏幕电子显示系统;报纸上杂志上的文字宣传;街头巷尾的灯箱广告。前面4种形式价格较高,但显示的信息量较多;而普通的灯箱广告画布上印的宣传内容大多静止不动,传递的信息量有限,优点是价格较低。若采用滚动画布广告,将多幅画面连接起来装在露天灯箱中,画布用电动机拖动,可上下移动进行循环显示,信息量大,动态效果好,可以对多种商品进行介绍或宣传,在街头构成一道风景线。同时也适用于大商场中对商品信息以及销售情况进行介绍。画布可以定期更换,方便不同商品需要。这种方式投资在2500元左右。     

Si衬底上横向外延GaN材料的微结构和光学性质的研究

用氢化物气相外延 (HVPE)方法在Si(III)衬底上成功横向外延生长出晶体质量较好的GaN薄膜材料。透射电子显微镜 (TEM )的研究结果表明 ,横向外延区域GaN的位错密度明显减小。由于SiO2 掩膜腐蚀角的不同 (分别为 90°和 6 6°) ,导致了横向外延GaN材料一些独特的微观形貌。微区拉曼光谱由数百条拉曼散射曲线组成 ,每一条曲线有三个振动模 ,分别对应Si振动模式 (5 2 0cm- 1) ,E2 模式 (5 6 6cm- 1)和E1(LO)模式(732cm- 1)。在垂直条纹方向 ,峰位和峰宽没有明显的变化 ,而峰强约 5 μm会发生周期性变化