Sanfilippo D syndrome: Correction of glucosamine-6-sulphatase deficiency following fibroblast culture in chang's media

The de-O-sulphation of α-linked glucosamine-6-sulphate residues in heparan sulphate requires a specific sulphatase, glucosamine-6-sulphatase, which has been shown to be deficient in tissues of Sanfilippo D, or mucopolysaccharidosis type IIID (MPS IIID), patients. MPS IIID fibroblasts cultured in Basal Eagle's medium supplemented with either fetal calf serum or heat-inactivated fetal calf serum, MDCB or Ultraserg media had residual glucosarnine-6-sulphatase activities towards a heparin-derived trisaccharide substrate, O-(α-N-acetylglucosamine-6-sulphate)-(1→4)-L -O-(α-iduronic acid-2-sulphate)-(1→4)-D -O-2,5-anhydro [1-³H]mannitol-6-sulphate, GlcNAc6S-IdoA2S-anM6S, which were less than 1 per cent of the normal range for fibroblasts cultured in Basal Eagle's medium supplemented with fetal calf serum. However, the glucosamine-6-sulphatase activities of MPS IIID fibroblasts grown in Chang's medium were similar to the activities in normal control fibroblasts which were cultured in Basal Eagle's medium. These results indicate that caution is required for prenatal diagnosis of MPS IIID patients using chorionic villi or amniotic cells cultured in Chang's medium.     

Immediate prenatal diagnosis of Zellweger syndrome by direct measurement of very long chain fatty acids in chorionic villus cells

We report a gas chromatographic-mass spectrometric method which allows the very long chain fatty acids content of trophoblastic tissue to be directly measured in samples collected by biopsy between 8 and 11 weeks of gestation. This method has been successfully applied to the detection of fetal Zellweger syndrome in two pregnant women who had previously delivered affected infants. In one of them, increased concentrations of C26:0 (0.254 versus 0.108±0.035 μ/mg proteins) and C24:0 (1.32 versus 0.815±0.325 μ/mg proteins) in trophoblast indicated that the fetus had Zellweger syndrome, a diagnosis confirmed by pathological findings after abortion. In the second case, the pregnancy was allowed to proceed, on the basis of normal concentrations of very long chain fatty acids in trophoblastic tissue, and its outcome was actually a healthy newborn.     

纳米复合GeO2/TiO2的制备及光催化性能研究

采用改进的Sol-gel方法,制备了一种新型的单分散椭球形微孔结构的纳米GeO2/TiO2复合光催化剂,并用TG-DTA、XRD、XPS、UV-vis、FT-IR、TEM、BET等手段进行了表征.以难生化降解的染料罗丹明B为目标降解物,采用HPLC测定不同光照时间下催化剂对染料降解的情况,考察了催化剂的光催化活性结果表明,改进的Sol-gel法制得的复合光催化剂具有明显的孔结构,而且比表面积大、粒径小、粒径分布窄、分散性好;与适量GeO2复合后能够有效提高TiO2纳米粒子的光催化活性;复合光催化剂的最佳制备条件为GeO2摩尔含量为0.1%,煅烧温度为400℃,所制得的GeO2/TiO2光催化活性比纯TiO2有显著提高.     

Impact of triazophos insecticide on paddy soil environment

ＩｎｔｒｏｄｕｃｔｉｏｎＲｉｃｅｉｓｔｈｅｓｔａｐｌｅｄｉｅｔａｒｏｕｎｄ 3 0 %ｏｆｔｈｅｗｏｒｌｄｐｏｐｕｌａｔｉｏｎａｎｄａｒｏｕｎｄ 60 %ｏｆｔｈｅＡｓｉａｎｐｏｐｕｌａｔｉｏｎ .Ｔｈｉｓｃｒｏｐｉｓｐｒｅｆｅｒｅｎｔｉａｌｌｙｏｒｇｅｎｅｒａｌｌｙｃｕｌｔｉｖａｔｅｄｕｎｄｅｒｓｕｂｍｅｒｇｅｄｓｏｉｌｃｏｎｄｉｔｉｏｎｓｆｏｒｒｅａｓｏｎｓｏｆｂｅｔｔｅｒｙｉｅｌｄｓａｎｄｔｏｐｏｇｒａｐｈｉｃａｌｓｉｔｕａｔｉｏｎｓ(Ｒｅｉｃｈａｒｄｔ,1 997) .Ｔｈｅｉｍｐｏｒｔａｎｃｅｏｆｓｏｉｌｍ…     

Effect of leachate recycling and inoculation on the biochemical characteristics of municipal refuse in landfill bioreactors

Activity development of key groups of enzymes involved in municipal refuse decomposition was measured in laboratory landfill bioreactors with and without leachate recycling and inoculation for about 210 days.The results showed that the enzymes(amylase,protease,cellulase,lipase and pectinase)were present in fresh refuse but at low values and positively affected by leachate recycling and refuse inoculation.The total average of cellulase activity in digesters D3 operated with leachate recycling but no inoculation,D4 and D5 operated with leachate recyling and inoculation was much higher than that in digesters D1 and D2 without leachate recycling and inoculation by 88%-127%,117%-162%and 64%-98%.The total average of protease activity was higher in digester D4 than that in digesters D1,D2,D3and D5 by 63%,39%,24%and 24%,respectively,and the positive effect of leachate recycling and inoculation on protease activity of landfilled refuse mainly was at the first two months.The total average of amylase activity was higher in digesters D3,D4 and D5 than that in digesters D1 and D2 by 83%-132%,96%-148%and 81%-129%.During the early phase of incubation,the stimulatory effect of inoculation on lipase activity was measured,but refuse moisture was the main factor affecting lipase activity of landfilled refuse.The inoculation,initial and continuous inoculation of microorganisms existing in leachate,was the mainly stimulatory factor affecting pectinase activity of landfilled refuse.     

一种新的拓扑指数及其在有机化合物结构/性质相关性研究中的应用

以距离矩阵为基础，提出一种表征化合物分子结构的拓扑指数，并将其用于预报饱和链烃类、醇类和含氮芳香类化合物的性质和活性，获得了满意的结果。     

钙钛矿型烯土复合氧化物及其在汽车尾气净化工程中的应用

本文介绍了钙铁矿型烯土复合氧化物的结构特征、制备方法与催化性能；分析了该类氧化物对汽车尾气环境的适应性，并与现有的贵金属催化剂作了比较．     

镧、铈对厌氧颗粒污泥产甲烷的Hormesis效应及其动力学研究

间歇培养条件下研究了单一稀土元素镧(La3 )和铈(Ce3 )在不同浓度水平下对厌氧颗粒污泥活性及其动力学行为的影响.结果表明,La3 和Ce3 对厌氧颗粒污泥比产甲烷活性有促进作用的浓度范围均为0.01～0.1 mg·L-1,促进作用浓度峰值均为0.05mg·L-1,最大促进百分比分别为10.35%和20.79%;La3 和Ce3 对SMA(比甲烷活性)的半抑制浓度分别为5mg·L-1和1000mg·L-1,La3 较易对污泥产生抑制作用.米-门方程可以用来描述厌氧消化过程中甲烷发酵阶段的动力学行为,稀土元素La3 和Ce3 投加至反应系统后,提高了甲烷发酵过程的动力学常数Vmax和半饱和常数Ks.     

思茅大气环境与气象条件的关系

对思茅地区大气环境监测数据进行统计、分析,与当地天气气候条件进行对比,探究思茅地区大气污染状况与气象条件之间的关系.