伍廷芳的司法独立观初探

伍廷芳的司法独立观建构在西方“三权分立”原则之上 ,其主要内涵有三点 :一是反对行政权干涉司法权 ;二是采用律师制、陪审制、公开审判、辩论式诉讼、无罪推定等西方资产阶级文明审判方式和原则 ;三是开办法律学堂 ,实行法官高薪制。     

The peripheral myelin protein gene PMP-22 is contained within the Charcot-Marie-Tooth disease type 1A duplication.

Charcot-Marie-Tooth disease (CMT1) is the most common form of inherited peripheral neuropathy. Although the disease is genetically heterogeneous, it has been demonstrated that the gene defect is the most frequent type (CMT1A) is the result of a partial duplication of band 17p11.2. Recent studies suggested that the peripheral hypomyelination syndrome in the trembler (Tr) mouse, a possible animal model for CMT1 disease, is associated with a point mutation in the peripheral myelin protein-22 gene (pmp-22). Expression of pmp-22 is particularly high in Schwann cells, and the protein is found in peripheral myelin. We now report that the human PMP-22 gene is contained within the CMT1A duplication. We therefore, suggest that increased dosage of the PMP-22 gene may be the cause of CMT1A neuropathy.     

Mutations in a new member of the chromodomain gene family cause CHARGE syndrome

CHARGE syndrome is a common cause of congenital anomalies affecting several tissues in a nonrandom fashion. We report a 2.3-Mb de novo overlapping microdeletion on chromosome 8q12 identified by array comparative genomic hybridization in two individuals with CHARGE syndrome. Sequence analysis of genes located in this region detected mutations in the gene CHD7 in 10 of 17 individuals with CHARGE syndrome without microdeletions, accounting for the disease in most affected individuals.     

Mutations in the polyglutamine binding protein 1 gene cause X-linked mental retardation

We found mutations in the gene PQBP1 in 5 of 29 families with nonsyndromic (MRX) and syndromic (MRXS) forms of X-linked mental retardation (XLMR). Clinical features in affected males include mental retardation, microcephaly, short stature, spastic paraplegia and midline defects. PQBP1 has previously been implicated in the pathogenesis of polyglutamine expansion diseases. Our findings link this gene to XLMR and shed more light on the pathogenesis of this common disorder.     

Mutations in SMAD3 cause a syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis

Thoracic aortic aneurysms and dissections are a main feature of connective tissue disorders, such as Marfan syndrome and Loeys-Dietz syndrome. We delineated a new syndrome presenting with aneurysms, dissections and tortuosity throughout the arterial tree in association with mild craniofacial features and skeletal and cutaneous anomalies. In contrast with other aneurysm syndromes, most of these affected individuals presented with early-onset osteoarthritis. We mapped the genetic locus to chromosome 15q22.2-24.2 and show that the disease is caused by mutations in SMAD3. This gene encodes a member of the TGF-β pathway that is essential for TGF-β signal transmission. SMAD3 mutations lead to increased aortic expression of several key players in the TGF-β pathway, including SMAD3. Molecular diagnosis will allow early and reliable identification of cases and relatives at risk for major cardiovascular complications. Our findings endorse the TGF-β pathway as the primary pharmacological target for the development of new treatments for aortic aneurysms and osteoarthritis.     

基于LNG冷能利用的低温冷库与冷能发电系统的集成

为提高液化天然气(LNG)冷能的利用效率,在对低温冷库利用LNG冷能进行(火用)分析的基础上,以甲烷和乙烷混合物(两者质量比为65∶35)为工质,将利用冷能发电的Rankine循环与利用LNG冷能的冷库制冷过程进行集成,以便在供应冷库所需的冷能不变的情况下,将深冷部分的LNG冷(火用)转换为电能.文中还对影响Rankine循环发电效率的参数进行分析.研究结果表明,集成后的系统在满足冷库所需冷能的基础上,使每吨LNG的冷能还可发电约15.5kW·h,LNG冷(火用)的利用效率从38.5％提高到54.0％.     

东北某污水处理厂各流程出水中污染物时空分布特征

为研究污水处理厂对有机污染物的去除效能,对我国北方某市的污水处理厂进行了1年的调查分析,考察了其各工艺出水中有机污染物的时空分布特征.结果表明:该污水处理厂采用A/O处理工艺处理后,除冬季出水氮磷略高,全年其它时间出水基本满足国家污水一级排放标准.采用气相色谱-质谱联用对有机物的检测分析结果表明,经过二级生物处理后,有机污染物种类有所减少,但仍含有较大量有机污染物,需采取有效的深度处理措施以减少中水排放对受纳水体的影响并保障水源水质的安全性.     

重庆市三峡库区医疗垃圾现状及无害化处理分析

医疗垃圾属于一类危险废物，必须进行无害化处理。文章论述了医疗垃圾的特性和危害性，对重庆市三峡库区目前医疗垃圾管理和处理的现状进行了分析，针对其特点，提出对三峡库区医疗垃圾有效的管理措施和进行分区集中处理，具体分析了医疗垃圾处理技术方案：分类收集、密闭运输、预处理、焚烧、完全填埋等。通过无害化处理后，杜绝医疗垃圾对环境的污染，以保护三峡库区生态环境和水资源。     

Al_(88)Co_4Y_6Er_2非晶合金的晶化动力学行为

采用单辊旋淬法制备了Al88Co4Y6Er2非晶合金,差热分析手段研究了该非晶合金晶化动力学性能.根据约化玻璃转变温度Trg,发现Er(2at%)元素部分取代Al88Co4Y8非晶合金中的Y元素后,得到的Al88Co4Y6Er2非晶合金具有更强的玻璃形成能力.计算得到Al88Co4Y6Er2非晶合金的晶化激活能,初始晶化激活能为381 kJ·mol-1,发现Al88Co4Y6Er2非晶合金具有很好的热稳定性.     

上海地区冷季型草坪主要病害鉴定及防治策略

研究调查了上海地区高羊茅，草地早熟禾，多年生黑麦草，葡匐剪股颖等主要冷季型草坪草病害发生情况。鉴定出８种主要病害，其中早熟禾枯萎病，黑麦草和高羊 在部分草坪上发生严重，翦股颖上没发现严重病害。针对上海地区的具体情况和国际上草坪病害研究的新进展，提出了冷季型草坪草病害的综合防治策略；