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The result of uroscopy of the proband was strong positive. There was a novel deletion mutation of c.876-877 del TC in the coding region of exon 6 of IDS gene, which was a hemizygous mutation. However, the mutation of his mother and sister was a heterozygous mutation. Detection of the exon 6 of IDS gene showed that the mutation was not found among normal controls and other patients with MPS I, IV, and VI other than MPS II. Homology comparison of amino acid sequences from different species showed that the phenylalanine glutamine of the mutation site of c.876-877 del TC located in p.292-293 was highly conserved. The activity of IDS enzyme of the proband was only 2.3 nmol/4 h/mL, which was much lower than normal; but the activity of IDS enzyme of his father, mother and sister was 641.9 nmol/4 h/mL, 95.8 nmol/4h/mL and 103.2 nmol/4h/mL, respectively.

结果显示:先证者尿检呈强阳性;其IDS基因exon 6编码区内存在c.876-877 del TC新缺失突变,为半合子突变,而其母、其姐为杂合突变;正常对照和其他非II型MPS患者的IDS基因exon 6的检测结果均未发现该突变;不同物种氨基酸序列的同源性比对显示: c.876-877 del TC突变所在的位置即p.292-293的苯丙氨酸谷氨酰胺高度保守;酶活性测定的结果显示:先证者的IDS酶活性仅为2.3 nmol/4 h/mL,大大低于正常值,而其父的为641.9 nmol/4 h/mL,其母的血浆酶活性为95.8 nmol/ 4h/mL,其姐的为103.2 nmol/4 h/mL。

With weight average method, the experimental results of hardness variation in Fe-C Martensite with C contents are explained by valence electron structure satisfactorily.

采用计权平均方法,用价电子结构理论解释了Fe-C马氏体的硬度随含C量变化的实验结果。

The results showed that both the break point on the Ia-c curve and the minimum of the derivative variation corresponding to the λa-c curve agreed very well with the critical micelle concentration of the surfactant in aqueous solution as measured by surface tension technique.

以对二甲氨基苯甲腈为探针,测定它在表面活性剂(C12TABr、SDS、C12E23、C12-3-12·2Br)水溶液中的第二重荧光强度和对应的特征波长对表面活性剂浓度曲线,由Ia-c 曲线的转折点或λa-c曲线对应的一阶导数极小点可以获得临界胶团浓度。

Make sure your bedroom is well-ventilated and at a comfortable temperature (below 24°C and above 12°C).

确保你的寝室有良好的通风,温度控制在适宜的范围内(24°C 以下, 12°C 以上)。。

Plusing the some edits collected materials the language sub- procedure,when people use the C language to program compilation,and that can also show the language of C advantage.For example , PC- The DOS, etc. of WORDSTAR is with this kind of method plait to write.

用C 语言加上一些汇编语言子程序,就更能显示C 语言的优势了,象PC- DOS 、 WORDSTAR等就是用这种方法编写的。

Today, both C and C++ are workhorse languages, with little in the way of sexy new development.

今天,C和C++都是主力语言。

The results showed that the TiN andN films are polycrystalline phase. The properties and structure of TiN films are mainly determined by the N2 mass flow rate, and as the N2 mass flow rate increases, the structure of the TiN films transforms to f.c.c. type leading to better the properties of films. The presence of negative bias voltage can optimize the grain of TiN films, lower the defect proportion and denser the films, which improves the hardness of films. The TiN films conform to the free carrier absorption mechanism and there are many of free electrons with lower N content in TiN films. With the increase of N content, the quantity of free electrons and reflectivity of films decrease, and the plasma frequence shifts to the lower energy, which leads to the regular change of colors of films from silver, yellowy, golden to yellow-red, meanwhile the lightness of films decreases. The goldenN film consists of TiN and ZrN phase, but belongs to a sigle f.c.c. structure with (111) preferred orientation. The Zr-doping dosen't change the position of the valance band, conduction band and forbidden band ofN film, but leads to the presence of new energy levels, which is the reason thatN film remains golden. The transparent hard films with good corrosion resistance and high hardness have been prepared and the further reseachs showed that the grain size of those films is by far smaller than the wave length and the width of forbidden band of those films is very broad, is the reasons that those films are transparent.

研究表明:氮化钛和N 薄膜为多晶态,氮流量决定了氮化钛薄膜的结构和性能,增加氮流量能使氮化钛薄膜的结构向面心立方结构转变,从而得到性能良好的氮化钛薄膜;施加负偏压能优化氮化钛晶粒和减少薄膜中的缺陷,使膜层变得更致密,从而提高薄膜硬度;氮化钛主要遵循自由载流子光吸收,氮含量较少时薄膜中的自由电子数目较多,随着氮含量的增加,薄膜中的自由电子数目不断减少,反射率逐渐降低,等离子体频率向低能端移动,从而使薄膜颜色出现规律变化,由金属色银白色到淡黄、金黄再到红黄,并且薄膜亮度呈下降趋势;金黄色的N 薄膜中存在TiN 和ZrN 的分离相,但其为单一的面心立方结构并具有(111)面择优取向;相对于TiN 薄膜,Zr 掺杂后,并没有使薄膜的导带、价带和禁带发生变化,只是在TiN 禁带内增加了新能级,这也正是掺杂Zr 后,薄膜仍

This study showed that yttria stabilized zirconia can be brazed to 444 stainless steel by in-situ alloying of pure Ni-Ti foils at temperatures around 1000°C. The best brazes were produced when brazing for 60 minutes at 1010°C and 30 minutes at 1030°C.

概要 此文验证了氧化钇稳定的氧化锆在1000°C下可以使用纯镍、钛箔现场配制的合金进行钎焊444不锈钢。1010℃钎焊60分钟,1030℃钎焊30分钟可以达到最好效果。

First of all, we found ZAK will activate ERK and JNK, and the phosphorylation expression levels of down-strean transcription factor including c-Fos, c-Jun and Elk-1were increased.

首先,我们发现过度表现ZAK会活化ERK及JNK分子及其下游调控之转录因子的活性,例如: c-Fos、c-Jun及Elk-1。

The copper atom is in a planar coordination site of [N_2O_2] and it devites from the mean plane by only 0.80 pm. Byπ-πstacking interactions, a alabastrine structure was obtained.(2)Schiff-base complex [Cu(H_2O)]·H_2O, where H3GS is the 3-carboxyl -salicylidene glycine, was synthesized and characterized by elemental analysis, IR spectra and single-crystal analysis. The crystal belongs to monoclinic system, space group P2(1)/c, a=848.46(3)pm, b=681.54(3)pm, c=1967.16(8)pm,β=95.8210(10)°, Z=4, R_1=0.0279, wR_2=0.0724. The copper atom is in a square-pyramidal field with the base

结果表明该晶系属单斜晶系,空间群P2(1)/c,晶胞参数:a=848.46(3)pm,b=681.54(3)pm,c=1967.16(8)pm,α=90°,β=95.8210(10)°,γ=90°,Z=4,R_1=0.0279,wR_2=0.0724,Cu原子位于轻微变形的四方锥场底心,底面被氮原子、酚氧原子、甘氨酸羧基的一个氧原子和一个水分子氧原子占据,而甘氨酸羧基的另一个氧原子占据相邻分子的锥顶,因而形成一维链状结构;合成了单核双聚配合物Na_2[Cu_2_2]·2H_2O ,铜三核配合物Cu_3_2·5H_2O和铜锌异三核配合物ZnCu_2_2·5H_2O,并用元素分析,IR光谱,电子光谱和磁化率测定对配合物的组

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推荐网络例句

This one mode pays close attention to network credence foundation of the businessman very much.

这一模式非常关注商人的网络信用基础。

Cell morphology of bacterial ghost of Pasteurella multocida was observed by scanning electron microscopy and inactivation ratio was estimated by CFU analysi.

扫描电镜观察多杀性巴氏杆菌细菌幽灵和菌落形成单位评价遗传灭活率。

There is no differences of cell proliferation vitality between labeled and unlabeled NSCs.

双标记神经干细胞的增殖、分化活力与未标记神经干细胞相比无改变。