Mutations in the gene encoding the basal body protein RPGRIP1L, a nephrocystin-4 interactor, cause Joubert syndrome

Protein-protein interaction analyses have uncovered a ciliary and basal body protein network that, when disrupted, can result in nephronophthisis (NPHP), Leber congenital amaurosis, Senior-L?ken syndrome (SLSN) or Joubert syndrome (JBTS). However, details of the molecular mechanisms underlying these disorders remain poorly understood. RPGRIP1-like protein (RPGRIP1L) is a homolog of RPGRIP1 (RPGR-interacting protein 1), a ciliary protein defective in Leber congenital amaurosis. We show that RPGRIP1L interacts with nephrocystin-4 and that mutations in the gene encoding nephrocystin-4 (NPHP4) that are known to cause SLSN disrupt this interaction. RPGRIP1L is ubiquitously expressed, and its protein product localizes to basal bodies. Therefore, we analyzed RPGRIP1L as a candidate gene for JBTS and identified loss-of-function mutations in three families with typical JBTS, including the characteristic mid-hindbrain malformation. This work identifies RPGRIP1L as a gene responsible for JBTS and establishes a central role for cilia and basal bodies in the pathophysiology of this disorder.     

适于大斜视角星载SAR系统的改进距离-多普勒算法

分析了当天线斜视角增大时,导致传统距离-多普勒成像算法性能下降的主要误差来源.提出了一种适合大斜视角星载SAR系统的改进距离-多普勒算法,并给出了处理仿真点目标数据得到图像质量的比较.结果表明,在大斜视角情况下,改进算法的性能远大于传统的距离\|多普勒成像算法.     

专业集成电路设计中三次多项式实现的新结构

专业集成电路（ＡｐｐｌｉｃａｔｉｏｎＳｐｅｃｉｆｉｃＩｎｔｅｒｇｒａｔｅｄＣｉｒｃｕｉｔ,ＡＳＩＣ）设计中的三次多项式计算,存在着一类典型的应用问题,即自变量是累积形成的,待自变量完整形成后再行直接计算是低效的。提出一种改进的三次多项式算法,在保证和直接算法相同精度的条件下,以较少的电路元件实现计算功能。同时,本算法从自变量完整形成到给出最终结果的延迟很小。     

A new method for uniform local heating deep in body using ultrasound phased-array system

A new method for targeted heating of deep tissue was developed by using an ultrasound phased-array system which can generate various multiple foci patterns by electronically changing its amplitude or phase pattern. This method involves using a technique of combining switching and rotating of multiple foci patterns to create a uniform temperature over tissue volumes in various size. Using this method, the target tissue deep in the body can be heated to a specified temperature, which gives conditions for thermo-sensitive liposomes release. A simulation study for a 108-element, spherically sectioned array was performed to determine an optimal heating scheme from a set of multiple focus fields which were produced by inputting different combinations of phases and amplitudes. Comparisons of a static multiple foci field, the switched fields and the switched-rotated fields indicated that the technique of combining switching and rotating of multiple foci patterns has advantages of both lowering the peak temperature and evening the temperature distribution. The simulation results also show that the therapeutic heating zones in various size employing the combined method. These results offer significant data for designing thermotherapy equipment for tumor-specific drug release with thermo-sensitive liposomes.     

钢筋混凝土及预应力混凝土梁式桥非线性分析

对钢筋混凝土结构进行非线性分析,可以认识结构在各类荷载作用下的受力性能和破坏机理;可以为结构进行合理的设计和施工提供依据和参考。运用fortran90编制程序计算普通混凝土及预应力混凝土梁式桥弯矩-曲率和荷载-位移关系曲线,模拟其从加载、开裂直到破坏的全过程结构反应,从而对其进行非线性分析,该计算结果与相关试验结果较为吻合,对于梁式桥非线性分析具有足够的精度性和适用性。     

Rapid reductive degradation of azo dyes by a unique structure of amorphous alloys

An Fe-based amorphous alloy was found to significantly enhance the reductive degradation of an azo dye in aqueous solution. The surface-area normalized rate constant was up to 2.0 L m-2 min-1 at room temperature,an order of magnitude larger than any previously reported value. The activation energy of the degradation process was calculated according to the Arrhenius equation,and a much lower value than those previously reported was obtained. Valence band measurement indicated that a depressed valence band maximum and a widened empty band were established in the amorphous ribbon. The unique structure of the amorphous alloy is important-it may reduce the activation energy of the degradation process and enhance the activity of the electrons,thus accelerating the degradation process.     

盾构克泥效浆液成分与渗透扩散研究

克泥效工法常在盾构隧道近接既有建筑物穿越工程中被引进作为控制地层位移的一种辅助工法,克泥效浆液在地层的渗透扩散性对盾壳与地层间环向间隙填充效果有着重要影响,克泥效浆液的流变性能与其微观成分对其流动性有着重要影响。本文基于对克泥效浆液流变性能与微观成分分析,研究了浆液性能变化。进一步结合宾汉姆流体砂土渗流公式与数值计算模型,分析了浆液性能对渗透效果的具体影响,并验证数值模型可靠性与合理性。结果表明:(1)各组克泥效浆液样本在宾汉姆本构下的非线性回归系数均大于0.95,塑性黏度与屈服强度会随着水粉比的减小而增大。(2)克泥效浆液主要成分为石英、斜长石、伊利石、钾长石,随着A液水粉比的增加,克泥效水化产物斜长石增加,而伊利石的含量减小。(3)克泥效浆液会在25s左右占满环向间隙,而后再向地层中发生渗透扩散,地层中浆液体积分数增速随时间而减小,直至增速降为0。(4)随A液水粉比增大、地层渗透系数增加、注浆压力增加,克泥效在地层渗透距离均会增加。理论渗透值与数值模型计算值误差在各工况下的平均误差控制在10%以内。     

公路路基变形破坏及其测试技术研究进展与展望

路基变形是公路病害的主要原因之一,其在公路建设、管理、运营、养护过程中带来的隐患日益凸显。目前以遥感测绘、地球物理探测、多传感器为代表性的相关技术,在路基变形测试中发挥了重要作用。为研究道路工程领域动态和行业发展趋势,梳理近年来道路工程领域理论、方法、技术及装备研究现状,阐述了公路路基变形的主要形式及影响因素,分析了路基变形测试技术类型及其优缺点,进一步讨论了对全周期智慧监测体系的认识及其发展趋势。构建路基变形监测体系是感知路基状态、路基稳定性评估和灾害预警的主要途径。随着人工智能和先进测试技术的快速进步,路基变形测试技术呈现出动态化、透明化、智慧化等新特点。加强公路测试基础理论、技术与装备的研发,形成基于立体空间的多手段路基健康监测体系是未来道路安全运维的重要发展方向。