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.     

乌龟卵壳结构的研究

本文对乌龟卵壳在扫描电镜下的微观结构进行了研究.乌龟卵壳主要由钙质层与壳膜组成.钙质层由霰石状态的碳酸钙晶体组成,可分为许多个壳单位,每个壳单位由同一中心辐射出来的针状晶体构成.卵壳的外表面具有一定的饰纹,有些部位还分布有纵横交错的有机纤维和块状的有机物质.壳膜由多层网状纤维组成,没有内、外壳膜之分.龟卵在孵化过程中不形成气室.同时对孵化前后龟卵壳的结构进行了对比研究.     

四构件带传动机构引导给定点轨迹的研究

在点的轨迹引导中,多采用四连杆机构中的连杆曲线来实现。但这种方法能引导的点数有限(理论上最多为9点),当要求被引导的点数较多或要求引导某一连续函数时,则机构尺寸的综合较困难犤1犦,而且引导精度较低。文章介绍了一种新型机构———将四连杆机构中的其中两个构件用盘状构件和柔性带来替代,组成一四构件带传动机构犤2犦,并通过算例详细叙述了该机构引导给定点轨迹的原理和方法。该机构用于点的轨迹引导,具有结构简单、引导精度高、传力性能好的特点。     

广义塑性力学的硬化理论

对广义塑性力学的硬化理论进行了系统而深入的剖析 ,阐明了硬化模型和硬化定律的基本概念和物理意义及其区别和联系 ,并指出应根据不同的土性、屈服面以及苛载类型恰当地选取硬化模型和硬化定律 ,进一步完善了广义塑性力学的硬化理论     

Temporal difference models describe higher-order learning in humans

The ability to use environmental stimuli to predict impending harm is critical for survival. Such predictions should be available as early as they are reliable. In pavlovian conditioning, chains of successively earlier predictors are studied in terms of higher-order relationships, and have inspired computational theories such as temporal difference learning. However, there is at present no adequate neurobiological account of how this learning occurs. Here, in a functional magnetic resonance imaging (fMRI) study of higher-order aversive conditioning, we describe a key computational strategy that humans use to learn predictions about pain. We show that neural activity in the ventral striatum and the anterior insula displays a marked correspondence to the signals for sequential learning predicted by temporal difference models. This result reveals a flexible aversive learning process ideally suited to the changing and uncertain nature of real-world environments. Taken with existing data on reward learning, our results suggest a critical role for the ventral striatum in integrating complex appetitive and aversive predictions to coordinate behaviour.     

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.     

对数凸度设计在十字万向传动轴中的应用

十字万向传动轴被广泛地应用在各类机械传动中,十字轴轴承是其重要的部件．轴承滚针的凸度设计,决定了滚动体与滚道间的接触压力分布,影响其性能和寿命．根据轴承结构对接触压力分布的影响,在滚针凸度设计时必须考虑滚针倒角半径和越程槽宽度的因素,并应用相应的数值计算方法．结果表明,使用这种方法设计的滚针轴承,其接触压力分布不会出现“边缘效应”,也不会在接触的中心部分出现应力集中。     

Ultraviolet emissions from the magnetic footprints of Io, Ganymede and Europa on Jupiter

Io leaves a magnetic footprint on Jupiter's upper atmosphere that appears as a spot of ultraviolet emission that remains fixed underneath Io as Jupiter rotates. The specific physical mechanisms responsible for generating those emissions are not well understood, but in general the spot seems to arise because of an electromagnetic interaction between Jupiter's magnetic field and the plasma surrounding Io, driving currents of around 1 million amperes down through Jupiter's ionosphere. The other galilean satellites may also leave footprints, and the presence or absence of such footprints should illuminate the underlying physical mechanism by revealing the strengths of the currents linking the satellites to Jupiter. Here we report persistent, faint, far-ultraviolet emission from the jovian footprints of Ganymede and Europa. We also show that Io's magnetic footprint extends well beyond the immediate vicinity of Io's flux-tube interaction with Jupiter, and much farther than predicted theoretically; the emission persists for several hours downstream. We infer from these data that Ganymede and Europa have persistent interactions with Jupiter's magnetic field despite their thin atmospheres.     

Windows NT的安全探讨

从用户的角度首先对windows NT操作系统的安全模式和安全策略进行了介绍.然后就"NT是一种针对网络应用的操作系统"对在网络中访问NT服务器的三种方式的安全性进行了分析;针对性地提出了其在安全性上的不足和解决方案;最后作者提醒大家除注意技术上的安全措施外还应养成良好的安全习惯.