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271.
272.
J. L. M. Keulemans I. Sinigerska V. H. Garritsen J. G. M. Huijmans Y. V. Voznyi O. P. van Diggelen W. J. Kleijer 《黑龙江环境通报》2002,22(11):1016-1021
Prenatal diagnosis of the Hunter syndrome (mucopolysaccharidosis type II; MPS II) is preferably achieved by the assay of iduronate-2-sulphate sulphatase (IDS) in uncultured chorionic villi (CV) as this allows early (12th week), rapid (2–3 days) and reliable results. We summarize the results of 174 prenatal analyses in the past 30 years, using various methods such as radiolabelled sulphate incorporation in amniotic fluid (AF) cells, glycosaminoglycan (GAG)-electrophoresis in AF and IDS assay in CV, CV-cells, AF and AF-cells. Twenty-seven fetuses with MPS II were diagnosed after finding clearly abnormal results in pregnancies with a male fetus; very low IDS activity has also been measured in some pregnancies with a (heterozygous) female fetus, emphasizing the need to combine enzyme assay with fetal sex determination. IDS activity has until recently been assessed by a cumbersome radioactive enzyme assay. Here we describe the use of a novel fluorigenic 4-methylumbelliferyl substrate, which allows a sensitive, rapid and convenient assay of IDS activity and reliable early prenatal diagnosis. This novel IDS assay was validated in retrospective analyses of 14 CV, CV-cell, AF and AF-cell samples from affected pregnancies in addition to prospective prenatal diagnosis in eight pregnancies at risk with one MPS II-affected fetus. Copyright © 2002 John Wiley & Sons, Ltd. 相似文献
273.
Toru Sakuma Naruji Sugiyama Takashi Ichiki Masanori Kobayashi Yoshiro Wada Daisuke Nohara 《黑龙江环境通报》1991,11(2):77-82
The urinary acylcarnitine profiles of two mothers whose first children were diagnosed to have glutaric aciduria type 2 (multiple acyl-CoA dehydrogenation deficiency, electron transfer flavoprotein (ETF) deficiency) were analysed in the second pregnancy. Large volumes of tigrylcarnitine and isovalerylcarnitine and a little glutarylcarnitine were detected. Each fetus was also diagnosed to be abnormal by enzyme activity and immunoassay of ETF protein. The acylcarnitines in the mothers' urine disappeared in 1 week after labour or artificial abortion. Acylcarnitines were never detected in the urine of controls. 相似文献
274.
275.
日本在循环社会和生态城市建设上的实践 总被引:7,自引:0,他引:7
文章重点介绍了近年来日本在循环社会和生态城市建设方面的实践,并试图探讨中国今后在生态城市建设上的发展方向。 相似文献
276.
pH5条件下生物制氢反应器的启动及运行特性 总被引:12,自引:2,他引:10
采用连续流搅拌槽式反应器(CSTR),以糖蜜废水为底物,探讨了pH5条件下生物制氢反应器的启动和运行特性.结果表明,保持反应器内pH为5,污泥接种量为6g/L、COD启动负荷为7.0kg/(m3·d)、水力停留时间(HRT)为6h等条件,可在30d内完成产氢发酵菌群的驯化.此时系统氧化还原电位(ORP)稳定在-460mV~-480mV之间,系统呈现明显的混合酸发酵特性.其液相末端发酵产物比例分布相当,乙酸、乙醇、丁酸、丙酸和戊酸含量分别占发酵产物总含量的36%,33%,18%,13%.没有占绝对优势的发酵产物.气相中的氢气含量30%~35%,其最大产氢能力为1.3m3/(m3·d).生物制氢反应器混合酸发酵稳定运行期各种产酸发酵细菌处于均势地位,以球菌和杆菌为主. 相似文献
277.
Conservation of representative facets of geophysical diversity may help conserve biological diversity as the climate changes. We conducted a global classification of terrestrial geophysical diversity and analyzed how land protection varies across geophysical diversity types. Geophysical diversity was classified in terms of soil type, elevation, and biogeographic realm and then compared to the global distribution of protected areas in 2012. We found that 300 (45%) of 672 broad geophysical diversity types currently meet the Convention on Biological Diversity's Aichi Target 11 of 17% terrestrial areal protection, which suggested that efforts to implement geophysical diversity conservation have a substantive basis on which to build. However, current protected areas were heavily biased toward high elevation and low fertility soils. We assessed 3 scenarios of protected area expansion and found that protection focused on threatened species, if fully implemented, would also protect an additional 29% of geophysical diversity types, ecoregional‐focused protection would protect an additional 24%, and a combined scenario would protect an additional 42%. Future efforts need to specifically target low‐elevation sites with productive soils for protection and manage for connectivity among geophysical diversity types. These efforts may be hampered by the sheer number of geophysical diversity facets that the world contains, which makes clear target setting and prioritization an important next step. 相似文献
278.
通过对1981~2012年24例长江上游流域大洪水过程进行普查,发现影响长江上游大洪水强降水过程的天气系统主要为:巴湖槽东移型、贝湖槽稳定型和东北冷槽型。采用统计、诊断及合成分析方法,对3种不同天气类型的大尺度环流背景、主要影响系统及致洪降水发生机理进行研究,并总结其特点及差异。结果表明:3种类型大洪水的洪水特征及降水特征各不相同,大尺度环流背景及天气系统也有所差异。副高及500hPa中高纬环流形势异常是导致强降水发生的直接因素,西南涡、冷暖式切变线及冷空气在不同的类型洪水降水中作用各不相同,异常气旋性环流场、水汽输送方向及水汽通量辐合区与强降水的位置密切相关。 相似文献
279.
三峡生态屏障区农户退耕受偿意愿的调查分析 总被引:4,自引:0,他引:4
运用参与式农户评估方法,对三峡生态屏障区内农户退耕还林的受偿意愿进行了调查.分析表明,退耕还林的生态效益受到普遍认可,但其可持续性令人担忧.1355份有效问卷中,愿意接受补偿的占74%,对未来生计的预期与土地相关性低的农户更愿意退耕.受偿意愿的形成具有明显的社会异质性和区域差异性.受访者性别、耕地面积、家庭年收入、帮扶形式、是否移民对受偿意愿值具有显著影响.屏障区平均补偿意愿为1014元/(亩×a).万州、秭归、渝北的受偿意愿值高于平均水平.建立基于区域的生态补偿标准和多元化的生态补偿方式,可以弥补目前均一型生态补偿标准和方式的不足,促进退耕还林工程和区域的可持续发展. 相似文献
280.
Satoru Isoda 《环境科学学报(英文版)》2013,(S1):S172-S179
Power conversion efficiency of p-i-n type microcrystalline silicon (c-Si:H) solar cells has been analyzed in terms of sequential processes of photo-induced electron transfer. The effect of the excitonic state on the charged carrier generation has been studied compared to a conventional scheme in which only charged carriers are taken into account for the operation of the solar cells. A numerical model has been developed to calculate current-voltage characteristics of solar cells on the basis of two types of charged carrier generation processes (exciton process and charged carrier process). The light trapping effect due to a textured back surface reflector (BSR) was embedded in the numerical model by using the effective medium theory in combination with the matrix method in the field of the electromagnetic theory of light. As an application of this modeling, it was found that the reported data of the power conversion efficiency were not explained by the conventional charged carrier process model and that the combined model of the charged carrier process with the exciton process well explains the performance of the p-i-n type c-Si:H solar cells. In this way, the typical power conversion efficiencies were estimated to be 10.5% for the device (i-layer thickness: 1.8 m) with the BSR (period: 600 nm; height: 250 nm) and 8.6% for the device with the flat reflector under the condition that the fractions of the exciton process and charged carrier process were 60% and 40%, respectively. 相似文献