低于正常的

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。

Objective To study the relationship of endangium proliferation and the expression of metalloproteinases and tissue inhibitors of metalloproteinases after angioplasty of iliac artery in rabbits to investigate the probable mechanism of composite Danshen pill in prevention and treatment of restenosisMethods 30 white male rabbits of Japan （the average body weight was 25～30 kg）were randomly divided into 3 groups:normal control group,model group (intima destroyed by balloon and given hypercholesterol diet),CDP group (intima destroyed by balloon and given hypercholesterol diet plus drug CDP 150 mg/d),10 ones each groupResults The results of iliac artery angiogram and the analysis of pathology:there were lumens stenosis,thinner intima,smaller intima area,in CDP group compared with those in model group,and ratio of intima and tunica media thickness and area among each group (P＜001)Immunohistochemistry analysis:the MMP2,MMP9,TIMP1,TIMP2 in model group significantly increased than those in control group (P＜001)The CDP group had lower MMP2 and MMP9 expression,higher TIMP1 and TIMP2 expression,and increased MMP2/TIMP1,MMP9/TIMP2 than model group (P＜001)The thickening of vascular neointima and stenosis degree of vascular lumen were relevant to MMP2 and MMP9 (r=0896,P＜001)Conclusions MMP and TIMP play the very important role in the restenosis process of arteryCDP could inhibit the thickness of vascular neointima after balloon injury,the probable mechanism of which may be inhibiting collagen formation,smooth muscle immigration and decreasing hyperplasia of intima by interfering expression of MMPs and TIMPs

目的 研究兔髂动脉成形术后血管内膜增生和基质金属蛋白酶及其抑制物表达之间的关系，探讨复方丹参滴丸预防再狭窄的可能机制。方法健康成年二级雄性日本大耳白兔30只，平均体重25～30 kg。随机分为3组：正常对照组10只，模型组10只（球囊内膜剥脱加高胆固醇饮食），治疗组10只（球囊内膜剥脱加高胆固醇饮食以及复方丹参滴丸150 mg/d）。结果兔髂动脉造影、血管病理图像分析检测结果：①复方丹参滴丸组较模型组血管造影示管腔直径狭窄、内膜厚度减少、内膜面积减少、内膜厚度和中膜厚度比、面积比，各组之间有显著性差异(P＜001)。②免疫组化分析：模型组MMP2、MMP9、TIMP1、TIMP2表达均高于对照组（P＜001）；复方丹参滴丸组MMP2、MMP9的表达低于模型组，而TIMP1、TIMP2的表达高于模型组；TIMP1/MMP2、TIMP2/MMP9明显增加，差异有显著性（P＜001）。③内膜的增生以及管腔的狭窄程度与MMP2、MMP9有很好的相关性(r=0896，P＜001)。结论 MMP和TIMP在再狭窄形成中起重要作用；复方丹参滴丸能够明显抑制血管损伤后的内膜增生，可能的机制是通过影响金属蛋白酶MMP2、MMP9及其抑制物TIMP1、TIMP2的表达从而抑制胶原的生成、平滑肌的迁移、增生，而减少内膜的增生。

Following chemical activation, blastocysts rate of the treated oocytes was similar to untreated oocytes.8 Following fertilization, however, few oocytes inhibited with CHX developed into morulae/blastocysts, due to a high incidence of polyspermy.9 Cortical granule migration occurred during inhibition, but CHX inhibition impaired CG migration, significantly no oocytes inhibited by CHX completed CG migration after maturation.10 CHX inhibition had no effects onα-microtubles and microfilaments of goat oocytes.

抑制24h转为正常培养24h，不影响卵母细胞的成熟和孤雌激活能力，并且CHX抑制后再成熟的卵母细胞经孤雌激活发育到囊胚的比例与对照组卵母细胞相似。8、体外受精后，CHX抑制后再成熟卵母细胞的多精受精现象显著增加，发育到桑椹胚/囊胚的比例显著低于对照组。9、CHX抑制过程中皮质颗粒仍能发生迁移，但是CHX抑制会对皮质颗粒的迁移造成不可恢复的损伤，使再成熟的卵母细胞内皮质颗粒不能完全迁移。10、CHX抑制对山羊卵母细胞α-微管和微丝都没有影响，无论是抑制后处于生发泡期的卵母细胞，还是抑制后再成熟的卵母细胞，微管和微丝的分布都与对照卵母细胞相似。

Other conclusion include: the trip of transient protection must be locked when the power circuit breaker of transimission line operated,because the trip is incorrect at this time;the features of transient current and its energy is different between the fault and thunder over- voltage in transimission line,and them can be identified;the ratio of high frequency component of current in serise capaciters compensation line , SVC and HVDC system is far little than fault transient current, so all of them do not affect the working of trasient current protection.

研究了在串联补偿电容的输电线路上发生故障时，串联补偿电容的MOV非线性保护电路对暂态保护的影响。串补电容MOV保护电路的非线性电流高频分量含量很小。静止补偿器、高压直流输电系统换流器的非线性特性产生的谐波分量含量极少。仿真计算表明，在与暂态保护判据中高频分量对应的频率范围内，其电流对应的功率谱估计值大大低于线路故障时故障电流的功率谱估计值，因此它们都不会对暂态电流保护的正常工作产生影响。

It has been put forward that there exists single Ball point and double Ball points on the symmetrical connecting-rod curves of equilateral mechanisms.

从鲍尔点的形成原理出发，分析对称连杆曲线上鲍尔点的产生条件，提出等边机构的对称连杆曲线上有单鲍尔点和双鲍尔点。

The factory affiliated to the Group primarily manufactures multiple-purpose pincers, baking kits, knives, scissors, kitchenware, gardening tools and beauty care kits as well as other hardware tools, the annual production value of which reaches US$ 30 million dollars.

集团所属工厂主要生产多用钳、烤具、刀具、剪刀、厨具、花园工具、美容套等五金产品，年生产总值3000万美元，产品价廉物美、选料上乘、质量保证，深受国内外客户的青睐