Detection of a new mutation (1467-A) for the pedigree withmucopolysaccharidosis type Ⅱ from a Chinese family

Mucopolysaccharidosis type II (MPS II) is a disabling, fatal monogenic disease caused by abnormal metabolism of the mucopolysaccharides[1]. Gene mutation is the basic cause of MPS II and investigation of the IDS gene muta-tion is the premise for prenatal gene diagnosis and gene therapy. MPS II is worldwide distributed with high inci-dence and serious results. To study the IDS gene of Chi-nese MPS II patients is important not only in carrying out one-family policy but also in upraisi…     

Recovery Properties of Polysulfone Hollow Fiber Chelating Membrane Modified with Thiourea for Mercury （ Ⅱ ）

The adsorption isotherms of the polysulfone hollow fiber chelating membrane modified with thionrea as chelating groups for Hg^2＋ were determined. The effects of mobile phase conditions and the operating parameters on removal performance of the chelating membrane for Hg^2＋ were also investigated. The recovery of Hg^2＋ decreased at low pH and the optimum range of pH was from 6 to 7. The feed concentration effected on recovery of Hg^2＋ at the specified loading amount of Hg^2＋. The Hg^2＋ could be removed from different concentration feed solution by chelating membrane. The increase of feed flow rate led to slight decrease of recovery of Hg^2＋ at the specified loading amount of Hg^2＋. The chelating membrane could be operated at height feed flow rate and a large-scale removal of H^2＋ could be realized. With the increase of load amount, Hg^2＋ recovery decreased, but the saturation degree of chelating membrane increased. According to required recovery of H^2＋ and the saturation degree of chelating membrane, the optimum loading amount of Hg^2＋ should be selected in the actual removal of H^2＋.     

一种含多苯并咪唑配体的单核Zn(II)配合物的合成、Hirshfeld表面分析和量化计算研究(英文)

以多苯并咪唑配体(NTB)和ZnCl2为原料合成了一种新型单核金属配合物,[Zn(NTB)Cl]2·(ZnCl4)·4(CH3OH), (1).对化合物(1)进行紫外、红外吸收光谱、CHN元素分析和X-射线单晶衍射表征,结果表明：该化合物在三斜晶系P-1空间群结晶,晶胞参数a＝1.3874(2) nm,b＝1.4095(1) nm,c＝1.6214(2) nm;α＝86.440(3)°,β＝87.296(2)°,γ＝70.699(3)°,V＝2.9855(6) nm3;吸收系数μ＝1.520 mm－1,计算密度ρc＝1.504 mg·m－3,晶格中电子数F(000)＝1384,对于衍射强度I〉2σ(I)的9 770个可观测衍射点,残差因子R1＝0.0777, 权重残差因子wR2＝0.2068.晶体中堆积中,来自于两个配位阳离子[Zn(NTB)Cl]＋ 的各两个N—H…O和一个N—H…Cl氢键,以及来自于4个溶剂甲醇的O—H…Cl氢键作用将晶体结构中的各个组成离子连接起来形成复杂的三维网络结构.通过Hirshfeld表面分析对分子间作用进行讨论,表明化合物中分子间作用力主要来自于N—H…O氢键.通过密度泛函理论(DFT)RB3LYP/6-31G(d)方法对晶体结构中一个[Zn(NTB)Cl]＋单元进行了理论优化,并对其分子轨道、自然键轨道、mulliken电荷布局进行分析.Hirshfeld表面分析化合物堆积作用主要来自于N—H…O/Cl、O—H…Cl氢键作用,自然键轨道分析表明NTB三个—CH2—基团成键轨道与相邻的咪唑基的C—N反键轨道稳定化能较小,有利于配体形成Δ和Λ两周不同的空间构型,mulliken电荷布局表明三个苯并咪唑氮原子相连氢原子带有较强的正电荷(约0.28 a.u.),有利于氢键的形成.     

钻机底座的一种简化计算

本文针对ZJ-32Ⅱ型钻机井架和底座的具体构造和传力特点,提出一种对其底座进行强度计算的简化力学模型。这是一个支承于刚性基础上的平面刚架模型。文中对这一模型的可行性进行了理论上的探讨,并提供了实际计算和其真实工作状况的有关资料。这一模型可极大地简化计算工作,但却不致引起过大的误差,而且偏于安全。文中并简要讨论了极限分析方法在底座设计中的应用及局部应力等问题。     

Increased incidence of unpartnered single chromatids in metaphase II oocytes in 39,X(XO) mice

Since rare cases of sex chromosome anomalies such as XXX and XXY were observed in the offspring of our XO breeder mice, we performed a cytogenetic analysis of metaphase II oocytes of XO mice to determine whether any changes in chromosomal configurations occur. We found a significantly increased incidence of unpartnered single chromatids in metaphase II oocytes of XO mice. Such single chromatids may contribute to embryonic aneuploidy. In addition, the tendency of the X-chromosome to segregate non-randomly to the oocyte rather than to the polar body was confirmed.     

[Ni(dien)2](NO3)2的合成与晶体结构

合成出硝酸镍与二乙烯三胺(dien)的新的配位化合物，并分析讨论该配合物的晶体结构。     

基于MPEG心理声学模型II的自适应音频水印算法

音频水印作为保护音频作品的版权和秘密通信的可行方法,成为近年数字水印研究领域中的一个热点。针对目前音频水印算法研究中自适应性能没有得到重视的问题,提出一种基于心理声学模型II(以下称模型II)的自适应盲检测音频水印算法,令算法具备良好的自适应性和稳健性。     

一对罕见的同构异素体的合成和晶体构造

[Co(maa)2(H2O)2](1)和[Cu(maa)2(H2O)2](2)是一对罕见的同构异素体，其中maa表示2—甲氧基乙酸。它们可以在甲醇溶液中以高产率制备出来。这两种同构异素体同属单斜晶系，空间群为P2l/n,其具体的空间大小：1为a6.985(1)，b10．102(2)，c7．132(1)(A)，β97．15(1)^o，V499.34(14)(A^3)；2为：a6.955(1)，b10．123(2)，c7．226(1)(A)，β96．57(1)^o，V505．41(14)(A^3)。这两种同构异素体有相同的配位几何构型，每个金属原子都处在一个稍微扭曲的八面体中间。所有羧酸根和水分子上的O原子都参与了氢键的形成，配合物分子则由这些氢键结合成为一个具有三维的空间结构。     

MRP生产计划与控制系统的探讨

根据生产计划与控制的基本功能要求，分析了我国现行生产计划与控制理论及其实践中存在的问题。指出ＭＲＰ是解决这些问题和实现生产计划与控制基本功能的有效方法，并分析了ＭＲＰ系统有效运行的基础。