Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease.

A locus segregating with familial Alzheimer's disease (AD) has been mapped to chromosome 21, close to the amyloid precursor protein (APP) gene. Recombinants between the APP gene and the AD locus have been reported which seemed to exclude it as the site of the mutation causing familial AD. But recent genetic analysis of a large number of AD families has demonstrated that the disease is heterogeneous. Families with late-onset AD do not show linkage to chromosome 21 markers. Some families with early-onset AD show linkage to chromosome 21 markers, but some do not. This has led to the suggestion that there is non-allelic genetic heterogeneity even within early onset familial AD. To avoid the problems that heterogeneity poses for genetic analysis, we have examined the cosegregation of AD and markers along the long arm of chromosome 21 in a single family with AD confirmed by autopsy. Here we demonstrate that in this kindred, which shows linkage to chromosome 21 markers, there is a point mutation in the APP gene. This mutation causes an amino-acid substitution (Val----Ile) close to the carboxy terminus of the beta-amyloid peptide. Screening other cases of familial AD revealed a second unrelated family in which this variant occurs. This suggests that some cases of AD could be caused by mutations in the APP gene.     

基于生产链的企业环境绩效测度模型

提出企业绿色度的概念，分析企业绿色度指标设置的目的、原则与方法，并对企业生产链的各个环节设置指标体系，采用层次分析法进行赋权，建立企业绿色度的计量模型，考核企业的环境绩效。     

电厂远程专家分析平台的建立

提出了建立电厂远程专家分析平台的整体方案．合理构建了电厂远程实时运行数据库平台，基于此数据库平台，进行专业应用软件的开发．介绍了远程实时监视、远程经济性分析、远程仿真、远程故障诊断等专业软件的开发思路.     

甲醇-汽油混合燃料对汽油机性能和排放的影响

通过汽油机燃用甲醇-汽油混合燃料（其中甲醇的体积分数为109／6，汽油的体积分数为90％，简称M10燃料）和汽油的对比试验，研究了M10燃料对汽油机性能和排放的影响，并用气相色谱仪测量了尾气中的甲醇和甲醛排放．试验研究表明：汽油机燃用M10燃料对汽油机动力性影响不大，但汽油机有效热效率和燃油经济性提高，排温有所降低；CO和HC排放减少，NOx排放与燃用汽油时相当；甲醇和甲醛排放增加．排气经三效催化转化器后，CO、HC和NOx排放可以被控制在与常规汽油机排放相当的水平，甲醇和甲醛排放可以被控制在接近零排放的水平．     

Chimaeric sounds reveal dichotomies in auditory perception

By Fourier's theorem, signals can be decomposed into a sum of sinusoids of different frequencies. This is especially relevant for hearing, because the inner ear performs a form of mechanical Fourier transform by mapping frequencies along the length of the cochlear partition. An alternative signal decomposition, originated by Hilbert, is to factor a signal into the product of a slowly varying envelope and a rapidly varying fine time structure. Neurons in the auditory brainstem sensitive to these features have been found in mammalian physiological studies. To investigate the relative perceptual importance of envelope and fine structure, we synthesized stimuli that we call 'auditory chimaeras', which have the envelope of one sound and the fine structure of another. Here we show that the envelope is most important for speech reception, and the fine structure is most important for pitch perception and sound localization. When the two features are in conflict, the sound of speech is heard at a location determined by the fine structure, but the words are identified according to the envelope. This finding reveals a possible acoustic basis for the hypothesized 'what' and 'where' pathways in the auditory cortex.     

'Designer atoms' for quantum metrology

Entanglement is recognized as a key resource for quantum computation and quantum cryptography. For quantum metrology, the use of entangled states has been discussed and demonstrated as a means of improving the signal-to-noise ratio. In addition, entangled states have been used in experiments for efficient quantum state detection and for the measurement of scattering lengths. In quantum information processing, manipulation of individual quantum bits allows for the tailored design of specific states that are insensitive to the detrimental influences of an environment. Such 'decoherence-free subspaces' (ref. 10) protect quantum information and yield significantly enhanced coherence times. Here we use a decoherence-free subspace with specifically designed entangled states to demonstrate precision spectroscopy of a pair of trapped Ca+ ions; we obtain the electric quadrupole moment, which is of use for frequency standard applications. We find that entangled states are not only useful for enhancing the signal-to-noise ratio in frequency measurements--a suitably designed pair of atoms also allows clock measurements in the presence of strong technical noise. Our technique makes explicit use of non-locality as an entanglement property and provides an approach for 'designed' quantum metrology.     

幂律流体管内充分发展的对流换热分析

将非牛顿流体的动量方程、能量方程和幂律流体的本构方程相结合 ,建立了幂律流体管内流动和换热充分发展时的对流换热控制方程组 ,并在恒热流和恒壁温边界条件下分别对方程组进行了求解 ,得到了两种不同边界条件下的温度分布和无量纲对流换热系数 (Nu数 )的表达式。结果表明 ,幂律流体的流变指数对流体流动的影响要大于对换热的影响 ;在恒热流边界条件下 ,幂律流体的温度在管内沿轴向呈线性分布 ;而在恒壁温条件下 ,其截面平均温度沿轴向呈指数规律变化。幂律流体的无量纲对流换热系数与幂律流体的流变指数有关 ,并且在两种边界条件下 ,均随着流变指数的增加而减小     

盘式永磁无刷直流发电机

阐述了轴向磁场盘式永磁无刷直流发电机的结构及其特点，提出了气隙磁场的简化模型，并对三维磁场进行了分析，在此基础上探讨了这种电机的设计方法，以最大功率输出为优化目标，导出了几个简单实用的设计公式。６极９相样机的实测数据证实了分析和设计方法的正确性，这种无刷直流发电机结构紧凑，效率高，特别适用于由引擎驱动的发电系统。     

复合材料层压板疲劳寿命预测方法

测定了碳纤维／树脂基T300／QY8911复合材料的3组典型单轴循环应力下的S-N曲线．基于试验数据，建立了多轴循环应力作用下单向板的寿命模型，并通过整合平面应力分析、失效分析和材料性质退化模块模拟多向层压板的疲劳失效过程．这种方法试图基于单向板在确定应力比下的疲劳试验结果，预测同种材料体系的任意铺层形式的多向层压板在复杂循环应力作用下的疲劳寿命．对于以分层破坏为主控因素的层压板，基于层间应力的计算结果，用计算面内累计损伤的方法计算分层损伤，层压板的寿命等于分层扩展寿命和分层后子层板剩余寿命之和．考虑分层扩展后，层压板的寿命预测结果得到明显改善．     

Palaeogene fossil <Emphasis Type="Italic">Populus</Emphasis> leaves from Lanzhou Basin and their palaeoclimatic significance

An angiosperm compression flora is found in Palaeogene from Lanzhou Basin and the cuticular analysis of Populus davidiana Dode in the flora is carefully made. Furthermore, the fossil cuticles are compared with the epidermal structures of extant Populus leaves growing in different environments, i.e. moist, semimoist, and semiarid to arid climatic regions. The present experiments indicate that mature leaves of P. davidiana show leaf size from big to small, leaf cuticles from thick to thin and anticlinal walls of epidermal cells from faintness to clarity along with the increase of lattudes of the plant distributions, the climatic variation from moist to arid, the annual precipitation from more to less and the annual mean temperature from high to low. The fossil P. davidiana differs from the specimens collected from Shandan in semiarid to arid climatic regions but closely resembles the Wushan leaves in a semi-moist climatic area in a lot of features. In a word, the new research may reflect that the flora lives in a semi-moist climatic environment. The present discovery of compression of Paleogene Populus davidiana is of great significance to studying vegetation types, climatic and environmental changes during the primal uplifting of the Qinghai-Tibet Plateau.