Prenatal prediction of childhood-onset spinal muscular atrophy (SMA) in Turkish families

Childhood-onset spinal muscular atrophy (SMA) is one of the most common neurodegenerative genetic disorders. SMN1 is the SMA-determining gene deleted or mutated in the majority of SMA cases. There is no effective cure or treatment for this disease yet. Thus, the availability of prenatal testing is important. Here we report prenatal prediction for 68 fetuses in 63 Turkish SMA families using direct deletion analysis of the SMN1 gene by restriction digestion. The genotype of the index case was known in 40 families (Group A) but unknown in the remaining 23 families (Group B). A total of ten fetuses were predicted to be affected. Eight of these fetuses were derived from Group A and two of these fetuses were from Group B families. Two fetuses from the same family in Group A had the SMNhyb1 gene in addition to homozygous deletion of the NAIP gene. One fetus from Group A was homozygously deleted for only exon 8 of the SMN2 gene, and further analysis showed the presence of both the SMN1 and SMNhyb1 genes but not the SMN2 gene. In addition, one carrier with a homozygous deletion of only exon 8 of the SMN1 gene was detected to have a SMNhyb2 gene, which was also found in the fetus. To our knowledge, these are the first prenatal cases with SMNhyb genes. Follow-up studies demonstrated that the prenatal predictions and the phenotype of the fetuses correlated well in 33 type I pregnancies demonstrating that a careful molecular analysis of the SMN genes is very useful in predicting the phenotype of the fetus in families at risk for SMA. Copyright © 2002 John Wiley & Sons, Ltd.     

等离子体与光催化耦合净化空气污染物研究现状

综合探讨了低温等离子体与光催化耦合这一新兴技术净化空气污染物的机理,讨论了影响耦合净化功能的主要因素以及臭氧的去除方法,介绍了该技术在去除挥发性有机物、氮氧化物、除菌等方面的研究现状,提出了其今后的研究方向。     

省域生态环境质量动态评价及差异研究

以2010~2015年30个省域为研究对象，采用“驱动力-压力-状态-影响-响应”框架构建评价指标体系，运用逐层纵横向拉开档次法确定指标权重，运用“时间度”和“熵值”法确定时间权重，构建TOWA-GA混合算子生态环境质量动态评价模型.结果表明：6a来，30个省域生态环境质量总体均值为0.837，整体呈好转趋势，但省域间差异明显.通过Theil指数分析发现，省域间总体差异下降了15.91%，人口密度和人口自然增长率是省域间差异的主要影响因素.     

聚丙烯酰胺-膨润土复合物的制备及其性能研究

采用盐酸对膨润土(BNT)进行酸活化改性,改性BNT再与氯化乙醇胺(EACl)进行阳离子交换反应,引入功能基团,得到胺化产物(ABNT-EACl).利用ABNT-EACl作为还原剂与Ce4 组成氧化还原引发体系,引发了丙烯酰胺(AM)单体的插层聚合得到了改性膨润土/聚丙烯酰胺无机/有机杂化物(ABNT/PAM).采用1H-NMR和FTIR对杂化物进行了结构表征,结果表明,杂化物中存在离子键合作用.通过实验对比研究了不同条件下的杂化物ABNT/PAM与普通絮凝剂处理工业印染废水的脱色效果,发现ABNT/PAM杂化物的效果优于普通絮凝剂.     

Stand-alone wind power system with battery/supercapacitor hybrid energy storage

This paper presents a stand-alone wind power system with battery/supercapacitor hybrid energy storage. A stand-alone wind power system mainly consists of a wind turbine, a permanent magnet synchronous generator, hybrid energy storage devices based on a vanadium redox flow battery and a supercapacitor, an AC/DC converter, two bidirectional DC/DC converters, a DC/AC converter and a variable load. Several control strategies for the stand-alone wind power system are involved such as a maximum power point tracking (MPPT) control, a vanadium redox flow battery charge/discharge control and a supercapacitor charge/discharge control. The proposed MPPT control combines a sliding mode control with an extreme search control to capture maximum wind energy. This strategy avoids the necessity of measuring wind velocity, obtaining models or parameters of the wind turbine and calculating the differentials of the power generated from the wind power system and from the speed of the generator. The battery charge/discharge control maintains a constant DC bus voltage. When the battery charging/discharging current reaches the setting threshold, the charge/discharge control of the supercapacitor is triggered to limit the charging/discharging current of the battery. The simulation results show that the proposed method can rapidly respond to variations in wind velocity and load power.     

基于混合指标值TODIM方法的东营市生态系统健康评价

城市生态系统健康评价是城市可持续发展以及城市生态文明建设的需要。在当今各种"城市病"频发的背景下,研究城市生态系统健康水平,不仅有利于协调城市发展与城市生态系统健康之间的矛盾,而且对于指导城市建设规划编制,监控城市生态环境的可持续发展具有重要的现实意义。鉴于此,论文在原有的城市生态系统健康评价指标的基础上,通过基于混合指标值的TODIM方法评价东营市2007—2013年间的城市生态系统健康状况,并分析活力、组成结构、恢复力、维持生态系统服务功能以及生态系统认知的构成指标对城市生态系统健康状况的影响。为了避免主观判断对城市生态系统健康评价的不确定性,文章采用兼顾客观信息和主观信息的组合权重赋权法确定各个指标的权重;根据东营市城市生态系统健康评价指标的特性和数据特点,建立了混合多属性决策TODIM方法的数学模型;采用扩展的TODIM方法对东营市城市生态系统健康状况进行评价,并分析了各个指标在城市生态发展过程中的作用。研究表明:(1)东营市的城市生态系统健康状况一直向好,由脆弱状态逐渐过渡到亚健康状态,但是生态状况仍然不容乐观,距离健康状态仍有较大的差距;(2)环保投入力度不足、污染物排放量增加、产业结构仍不完善、建成区人口密度过高等成为制约东营市城市生态系统健康发展的突出问题。文章提出了相应的对策建议,以促进城市生态系统的健康发展。     

厌氧-准好氧联合型生物反应器填埋场产气规律的研究

通过将厌氧型生物反应器填埋场（ANBL）和准好氧矿化垃圾生物反应床（SAARB）串联,组成新型的厌氧-准好氧联合型生物反应器填埋场（AN-SABL）,研究其产气速率、产气量以及产气组分的变化规律,以期为填埋气体的收集、利用和处理提供理论依据.实验表明,AN-SABL中的厌氧填埋单元的产气受到了抑制,其中ANBL2号单元和ANBL3号单元的产气率分别为49 L.kg-1和39 L.kg-1,仅占ANBL1号的94.2%和75.0%,但提高回灌频率,能促进厌氧填埋单元的产气,其甲烷含量最大值可达到62.67%;ANBL夏季产气速率和产气量明显高于冬季,并以12 h为周期交替出现产气高峰;此外,AN-SABL能够促进其厌氧单元的硝化和反硝化作用,N2O的含量受季节和填埋场类型影响显著,其变化范围在0.001 7%～4.017 9%之间.ANBL的累积产气量在初始调整阶段呈对数增长,过渡酸化阶段呈线性增长,酸化产甲烷阶段呈指数增长.     

华南沿海某大气背景点黑碳气溶胶污染特征

黑碳气溶胶(BC)因其显著的气候效应和对人类健康的危害而成为研究的热点。研究背景点位BC的浓度水平和来源特征有助于掌握BC在区域尺度上的辐射强迫特性和环境影响。2009年秋季在华南沿海某区域大气背景点进行了为期约40 d的BC在线观测。观测期间BC的平均浓度为(2.34±1.33)μg/m3,与其它区域背景点相比处于较高水平。利用中尺度天气研究和预报系统WRF模拟的高分辨率三维气象场数据驱动HYSPLIT-4反向轨迹模式,模拟观测期间每小时的气团轨迹,将所有轨迹分为来源特征明显的东北沿海、北方内陆、香港方向和东南海面方向四类。当受到东北沿海来源气团影响时,BC的浓度为平均水平的1.4倍,BC和CO有较好的线性相关关系,而其它三类气团影响时的BC浓度都低于平均浓度,且BC和CO的相关关系很差,说明来自东北沿海地区的较近距离区域传输是影响该背景点大气BC的主要源区。     

微压流化式复合生物反应器处理含酚废水效能的研究

通过连续动态试验,对新型微压流化式复合生物反应器(MP-FHBR)处理模拟苯酚废水的效能进行研究.结果表明,MP-FHBR的COD容积负荷可达4.86kg/(m3·d)、苯酚容积负荷可达1.96kg/(m3·d),出水COD低于200mg/L、出水苯酚浓度低于1mg/L,COD和苯酚去除率分别达到90%和99%以上.当苯酚容积负荷达到2.04kg/(m3·d),苯酚在反应器中逐渐累积,反应器内微生物的TTC-比脱氢酶活性下降.当MP-FHBR进水苯酚浓度为800mg/L时,最佳水力停留时间为9～10h.水力负荷对反应器沉淀池的泥水分离效果影响较大,限制了反应器的进水量,所以MP-FHBR主要适用于处理较高浓度的含酚废水.     

复合催化膜生物反应器处理一氧化氮废气研究

采用溶胶-凝胶法以聚砜(PSF)中空纤维膜为载体制备了Fe-Ti O_2/PSF复合催化膜,以此构建新型复合催化膜生物反应器(HCMBR),实现膜催化与硝化反硝化耦合烟气脱硝,进一步提高NO去除能力.采用Fe-Ti O_2/PSF复合催化膜生物反应器(HCMBR)处理一氧化氮废气,实现了180 d长时间高效稳定运行,NO去除效率可达93.2%,去除能力可达167.1g·(m~3·h)~(-1).适宜运行条件为:气体停留时间9 s、自然光光照强度670 lx,p H为6.8~7.2,n C/n N=3.7.Fe-Ti O_2/PSF复合催化膜加入,复合催化膜生物反应器去除NO的效率比膜生物反应器提高了1.4%~13%,在Fe-Ti O_2/PSF复合催化膜附着稳定的生物膜后,复合催化膜生物反应器去除NO的效率比湿式膜催化反应器提高了59.5%~66%.