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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。

In addition, the factory has also passed the ISO certification, ribavirin also adopted the FDA inspection, the product of the production technology of water ahead of the average domestic counterparts, the product of the production capacity are as follows: inosine for 2300 tons of raw materials / year proline API of 350 tons / year of raw materials ribavirin of 500 tons / year, ribofuranose tetraacetate 200 tons / year, valine more than 150 tons / year, product sales leader in the status of the domestic market.

不但如此,本厂还通过了ISO系统认证、利巴韦林还通过了FDA检查,各产品的生产技术水平均领先于国内同行,产品的生产能力分别为:肌苷原料为2300吨/年,脯氨酸原料药为350吨/年,利巴韦林原料为500吨/年、四乙酰核糖200吨/年、缬氨酸150吨以上/年,产品的销售市场占据国内领头羊地位。

Serum immunoglobulin of Amur sturgeon Acipenser schrenckii, Chinese sturgeon Acipenser sinesis and Huso sturgeon Huso dauricus which were healthy and unimmuned, was first time respectively purified by using saturation ammonium sulfate precipitation followed by Sephadex G-200 gel column chromatography. Molecular weight and partial characterization of Ig and heavy chain and light chain of Ig were measured by polyacrylamide gel electrophoresis, SDS polyacrylamide gel electrophoresis, Western blotting and Ouchterlony method.

采用饱和硫酸氨分步沉淀和Sephadex G-200凝胶层析的方法,首次分别纯化制备了健康非免疫状态下中华鲟、史氏鲟和达氏鳇的血清免疫球蛋白,并采用聚丙烯酰胺凝胶电泳、SDS-聚丙烯酰胺凝胶电泳、蛋白免疫印迹及免疫琼扩实验等方法对其Ig及Ig亚单位的分子量和部分特性进行了分析。

Acylamino compounds from AD were synthesized by cleavage of A-ring through NaIO4/KMnO4 oxidation, ring closure by amination, oximation with hydroxylaminechloride in C-17 position, and reduction of oxime in C-17 position and substitution with substituted acylchloride and carboxylic acids.

本文以AD为起始原料,经过NaIO4/KMnO4氧化裂解、胺解环合、羟氨肟化反应,在甾体17位形成肟,并将肟还原成胺,与取代酰氯和有机羧酸反应,合成了15个酰胺化合物。

SA catalyzed the amidation of benzhydryl alcohol in good to excellent yields under solvent-free condition.

加热条件下磺氨酸可以催化磺酰胺和酰胺类化合物与二苯甲醇的取代反应,生成新的取代胺类化合物。

We make chloroacetylaion to N-terminal of Asp, and then convert this compound to GD dipeptide by aminolysis. Followed the lipase -catalyzed peptide synthesis of RGD in aqueous water-miscible organic cosolvent is studied. We set up a new efficient reaction method to synthesize RGD tripeptide. The problem that peptide containing polar amino acid can not be synthesized readily in organic solvent was solved. We offer a new experimental proof for PPL-catalyzed peptide synthesis and make a more general analysis for the effect of some factors in theory.

采用氯乙酰基保护天冬氨酸,然后进行氨解生成GD二肽,利用猪胰脂肪酶催化合成RGD三肽,着重解决了亲水氨基酸/肽片段底物的溶解性与合成产率低的问题,建立了一种新的有效合成RGD三肽的反应模型,并探索解决了极性较强的氨基酸的酶促肽合成的问题,对脂肪酶催化合成肽的影响因素从理论上作了较全面的分析,为肽片段的缩合反应提供了一种新思路。

Aim To establish the method of detecting the Arylamine N-acetyltransferase-1(NAT1) genotype and its distribution of polymorphism,and analyze the correlation between the genotype and the phenotype in a Chinese Hans population.

目的探讨汉族人群中N-乙酰基转移酶1(NAT1)基因多态性的分布特点,以及基因型和表型之间的相关分析,以评价NAT1乙酰化代谢多态性对5氨-基水杨酸等药物疗效的影响。

In the fifth chapter, a series of betulin derivatives including 28-acetoxybetulin, 28-tritylbetulin, 28-benzoylbetulin, 3-0-P-D-glucopyranoside of betulin and some betulinic acid conjugate containing amino acid were synthesized and confirmed by MS and NMR.

第五章分别合成得到了乙酰化白桦酯醇、28-苯甲酰化白桦酯醇、28-三苯甲基化白桦酯醇、3-O-β-D-glucopyranoside of betulin和一系列的白桦酯酸苷氨酸的衍生物,并分别对其进行了结构鉴定。

In the fifth chapter, a series of betulin derivatives including 28-acetoxybetulin, 28-tritylbetulin, 28-benzoylbetulin, 3-O-β-D-glucopyranoside of betulin and some betulinic acid conjugate containing amino acid were synthesized and confirmed by MS and NMR.

第五章分别合成得到了乙酰化白桦酯醇、28-苯甲酰化白桦酯醇、28-三苯甲基化白桦酯醇、3-O-β-D-glucopyranoside of betulin和一系列的白桦酯酸苷氨酸的衍生物,并分别对其进行了结构鉴定。

The paint includes liquid A, which consists of acrylic resin, mixed solvent of xylene, acetone and isophorone, organic silicone solution, superfine silica powder, titanium dioxide powder, methyl-(N-beta-amino ethyl gamma-amino propyl)-dimethoxy siloxane solution, gamma-methyl acrylyloxy propyl tromethoxy siloxane solution and fluorocarbon modified siloxane; and liquid B, which consists of hexamethylene diisocyanate, polydiisocyanate, xylene, and methoxy propyl acetate. When the paint is used, two components are mixed together.

由丙烯酸树脂、二甲苯和丙酮及异佛尔酮混合溶剂、有机硅酮溶液、超细二氧化硅粉、钛白粉、甲基(N-β-氨乙基γ-氨丙基)二甲氧基硅烷溶液、γ-甲基丙烯酰氧基丙基三甲氧基硅烷溶液、氟碳改性硅氧烷溶液组成A液,由六亚甲基二异氰酸酯聚异氰酸酯、二甲苯和甲氧基乙酸丙酯混合溶液组成B液,使用时将两组分混合。

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The split between the two groups can hardly be papered over.

这两个团体间的分歧难以掩饰。

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