Genetic deficiency of tartrate-resistant acid phosphatase associated with skeletal dysplasia, cerebral calcifications and autoimmunity

Vertebral and metaphyseal dysplasia, spasticity with cerebral calcifications, and strong predisposition to autoimmune diseases are the hallmarks of the genetic disorder spondyloenchondrodysplasia. We mapped a locus in five consanguineous families to chromosome 19p13 and identified mutations in ACP5, which encodes tartrate-resistant phosphatase (TRAP), in 14 affected individuals and showed that these mutations abolish enzyme function in the serum and cells of affected individuals. Phosphorylated osteopontin, a protein involved in bone reabsorption and in immune regulation, accumulates in serum, urine and cells cultured from TRAP-deficient individuals. Case-derived dendritic cells exhibit an altered cytokine profile and are more potent than matched control cells in stimulating allogeneic T cell proliferation in mixed lymphocyte reactions. These findings shed new light on the role of osteopontin and its regulation by TRAP in the pathogenesis of common autoimmune disorders.     

Mid-infrared images of beta Pictoris and the possible role of planetesimal collisions in the central disk

When viewed in optical starlight scattered by dust, the nearly edge-on debris disk surrounding the A5V star beta Pictoris (distance 19.3 pc; ref. 1) extends farther than 1,450 au from the star. Its large-scale complexity has been well characterized, but the detailed structure of the disk's central approximately 200-au region has remained elusive. This region is of special interest, because planets may have formed there during the star's 10-20-million-year lifetime, perhaps resulting in both the observed tilt of 4.6 degrees relative to the large-scale main disk and the partial clearing of the innermost dust. A peculiarity of the central disk (also possibly related to the presence of planets) is the asymmetry in the brightness of the 'wings', in which the southwestern wing is brighter and more extended at 12 microm than the northeastern wing. Here we present thermal infrared images of the central disk that imply that the brightness asymmetry results from the presence of a bright clump composed of particles that may differ in size from dust elsewhere in the disk. We suggest that this clump results from the collisional grinding of resonantly trapped planetesimals or the cataclysmic break-up of a planetesimal.     

微小卫星星务计算机系统模拟部件可靠性设计

为确保星务计算机系统长期在轨工作的稳定性,针对系统中数模转换器等不同模拟部件的特点,给出相应的可靠性设计方案。在模拟输入通道中采用中位值滤波和递推平均滤波的组合算法,来获取尽可能可靠的输入数据,在模拟输出通道中则采用中值表决电路通用模块实现正确的模拟输出。该方案已在原理样机研制中得到验证和完善。仿真和验证结果表明,本方案在兼顾微小卫星对功耗、质量等苛刻要求的同时,能够很好地满足系统的可靠性要求。     

Aspartate 338 contributes to the cationic specificity and to driver-amino acid coupling in the insect cotransporter KAAT1

To investigate the peculiar ionic specificity of KAAT1, an Na⁺- and K⁺-coupled amino acid cotransporter from Lepidoptera, a detailed analysis of membrane topology predictions was performed, together with sequence comparison with strictly Na⁺-dependent mammalian cotransporters from the same family. The analysis identified aspartate 338, a residue present also in the other cotransporter accepting K⁺ (CAATCH1), but absent in most mammalian transporters that have, instead, an asparagine in the corresponding position. Mutation of D338 in KAAT1 led either to non-functional transporters (D338G, D338C), or to an altered ionic selectivity (D338E, D338N), observable in uptake experiments and in electrophysiological properties. In particular, in D338E, the transport activity, while persisting in the presence of Na⁺, appeared to be completely abolished in the presence of K⁺. D338E also showed uncoupling between transport-associated current and uptake. The opposite mutation in the -aminobutyric acid transporter rGAT-1 (N327D) resulted in complete loss of function. In conclusion, aspartate 338 in KAAT1 appears to be important in allowing K⁺, in addition to Na⁺, to drive the transport mechanism, although other residues in different parts of the protein may also play a role in the complete determination of ionic selectivity.Received 23 September 2003; received after revision 11 November 2003; accepted 25 November 2003     

人因工程在青岛世园会的应用分析

青岛世园会是第一次在山地举办的世园会,如何让游客方便、安全的游览是园区设施设计的目的,本文从色彩的应用、安全标志、说明标志、公共服务设施、无障碍设计等多个方面对世园会的设计进行了分析,解释了世园会按照人因工程原则进行设计的过程和细节,并分析了不足,为公共设施的设计提供了借鉴。     

马纳斯河流域民主参与式的水资源统一管理

本文针对玛纳斯河流域水资源管理存在逐利、水资源被分割管理,以及缺乏民主参与等问题,提出流域实行水资源统一管理,成立利益相关者参与的民主管理监督机构。     

LabVIEW和三维虚拟现实技术在物理实验中的应用研究

虚拟仪器技术主要倾向于数据处理的精确性,三维虚拟技术则倾向于形象的仪器展示.利用两者的优势,我们创造性地实现了三维虚拟现实技术与虚拟仪器技术的完美结合,并以"电子示波器的使用"为例,探讨LabVIEW技术和3D技术在物理实验中的应用.     

正交试验法优选田七花总黄酮的提取工艺

用正交试验法对田七花中的总黄酮提取工艺进行优化.以田七花中的总黄酮含量为考察指标,采用正交试验L9(34)表进行实验,对提取过程中的乙醇浓度、提取时间、提取次数及料液比四个影响因素进行研究.结果显示影响田七花中的总黄酮提取工艺的显著因素是提取次数,最佳提取工艺是:12倍量的80%的乙醇提取3次,每次1 h.