Mutations in ISPD cause Walker-Warburg syndrome and defective glycosylation of α-dystroglycan

Walker-Warburg syndrome (WWS) is an autosomal recessive multisystem disorder characterized by complex eye and brain abnormalities with congenital muscular dystrophy (CMD) and aberrant a-dystroglycan glycosylation. Here we report mutations in the ISPD gene (encoding isoprenoid synthase domain containing) as the second most common cause of WWS. Bacterial IspD is a nucleotidyl transferase belonging to a large glycosyltransferase family, but the role of the orthologous protein in chordates is obscure to date, as this phylum does not have the corresponding non-mevalonate isoprenoid biosynthesis pathway. Knockdown of ispd in zebrafish recapitulates the human WWS phenotype with hydrocephalus, reduced eye size, muscle degeneration and hypoglycosylated a-dystroglycan. These results implicate ISPD in a-dystroglycan glycosylation in maintaining sarcolemma integrity in vertebrates.     

新概念无人穿梭艇静水操纵性能

引入一种具有单体半滑行穿浪船型的新概念无人艇——穿梭艇,对其优良的操纵性能进行研究和分析.根据国际海事组织船舶操纵性试验标准,搭建穿梭艇自航模系统,进行标准的自航操纵性试验,获取相关的静水操纵性数据.采用船舶操纵性理论,结合试验数据对穿梭艇的操纵性能进行研究和分析.试验和分析结果表明穿梭艇具有良好的操纵性能和灵活性、独特的操纵性特点和优势.此外,采用数值计算方法,对其独特的操纵性特点进行了理论分析.     

科学饲料管理系统的研究与实现

本文论述了“清真畜产品规范生产与质量认证系统”中“科学饲料管理系统”的设计过程以及研发成果。该系统针对奶牛、肉牛和肉羊等特色动物养殖中饲料合理化和科学化的要求，对异构数据集成整合，即在客户端将各种异构数据转换成XML格式文件，通过Internet网络上传至服务器指定文件夹，并进行字段映射和数据整理，最后将经过整理的数据导入服务器数据库的相关表中，从而建立了宁夏地区特色动物饲料原料成分及配比数据库（知识库），实现了全区共享的饲料配方服务管理平台、事务处理平台等。“科学饲料管理系统”的应用有助于改进奶牛等动物饲料配方的科学性，提高饲料的利用率，达到节本增效的目标，为提高养殖业管理水平与畜产品生产效率提供信息化技术支撑。     

Sensor Fault Tolerant Generic Model Control for Nonlinear Systems

Introduction　　Conventionalfeedbackcontrolforacomplexprocessmayresultinunsatisfactoryperformance,oreveninstability,intheeventofmalfunctionsinactuators,sensorsorothersystemcomponents.Aclosed-loopcontrolsystemwhichtoleratesmalfunctions,whilestillmaintainingdesirableperformanceandstabilityproperties,canbesaidtobefaulttolerant[1].Faulttolerantcontrolenablesincreasedavailabilitybyavoidinginadvertentprocessshutdownsfromsimplefaults,e.g.,ininstrumentationandcontrolloops,thatcouldresultinproductionst…     

Microstructural evaluation and nanohardness of an AlCoCuCrFeNiTi high-entropy alloy

An AlCoCuCrFeNiTi high-entropy alloy(HEA) was prepared by mechanical alloying and sintering to study the effect of Ti addition to the widely studied AlCoCuCrFeNi system. The structural and microstructural characteristics were investigated by X-ray diffraction(XRD), scanning electron microscopy(SEM), and transmission electron microscopy(TEM). The formation of four micrometric phases was detected: a Cu-rich phase with a face-centered cubic(fcc) structure, a body-centered cubic(bcc) solid solution with Cu-rich plate-like precipitates(fcc), an ordered bcc phase, and a tetragonal structure. The XRD patterns corroborate the presence of a mixture of bcc-, fcc-, and tetragonal-structured phases. The Vickers hardness of the alloy under study was more than twice that of the AlCoCuCrFeNi alloy. Nanoindentation tests were performed to evaluate the mechanical response of the individual phases to elucidate the relationship between chemical composition, crystal structure, and mechanical performance of the multiphase microstructure of the AlCoCuCrFeNiTi HEA.     

基于遗传算法的一种粗糙集知识约简算法

知识约简是粗糙集理论研究的重要内容之一,是在保持信息系统分类能力不变的基础上,删除冗余知识.文中从系统的信息表出发,根据可辨别下三角矩阵,利用遗传算法,提出一种基于遗传算法的粗糙集知识约简算法,并通过实验分析说明,这种算法可以解决现有启发式算法无法解决的部分问题.     

Research on 3D Micro-nano Processing Reported on Nature

The latest issue of Nature reported in its Research Highlights ( see " Lithography: Luminous Lizards", Nature, Vol. 451, p868, Feb. 21, 2008) that a group of researchers from the Technical Institute of Physics and Chemistry (TIPC), Chinese Academy of Sciences (CAS) achieved the new progress in 3D microstructures of nanometer composites using the multiphoton nanoprocessing technology.     

A survey of stackelberg differential game models in supply and marketing channels

Erratum to: J Syst Sci Syst Eng DOI: 10.1007/s11518-007-5058-2 The presentation of Table 2 in the original version of this article contained a few typos. The corrected Table 2 is given below.