Mutations in dynamin 2 cause dominant centronuclear myopathy

Autosomal dominant centronuclear myopathy is a rare congenital myopathy characterized by delayed motor milestones and muscular weakness. In 11 families affected by centronuclear myopathy, we identified recurrent and de novo missense mutations in the gene dynamin 2 (DNM2, 19p13.2), which encodes a protein involved in endocytosis and membrane trafficking, actin assembly and centrosome cohesion. The transfected mutants showed reduced labeling in the centrosome, suggesting that DNM2 mutations might cause centronuclear myopathy by interfering with centrosome function.     

实测模态下三跨整体桥精细化基准有限元模型

以一座新建的整体式桥台桥梁(简称整体桥)为工程背景,建立服务于健康监测的精细化基准动力有限元模型.将设计阶段基于梁格法建立的全桥三维模型与采用梁-壳单元的精细化模型作为初始有限元模型,把环境激励下获取的实测模态信息(自振频率与振型)作为参照,基于参数灵敏度分析修正了上述两种结构模型.结果表明：尽管两种模型修正后都可以获得与实测模态尤其是频率值较好的吻合度,但修正后的梁格模型的模型参数严重偏离其真实的物理意义,难以作为长期监测的基准模型;而修正后的精细化梁-壳单元模型的模型参数物理意义明确,可以作为计入环境因素影响(如温度、湿度等)的结构长期监测的基准模型.该研究还基于动力试验和模型分析结果讨论了整体桥特有的结构-土相互作用的问题.     

A spherical representation of a correlation matrix

It is common practice to perform a principal component analysis (PCA) on a correlation matrix to represent graphically the relations among numerous variables. In such a situation, the variables may be considered as points on the unit hypersphere of an Euclidean space, and PCA provides a sort of best fit of these points within a subspace. Taking into account their particular position, this paper suggests to represent the variables on an optimal three-dimensional unit sphere.

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Résumé Il est classique d'utiliser une analyse en composantes principales pour représenter graphiquement une matrice de corrélation. Dans une telle situation, les variables peuvent être considérées comme des points sur l'hypersphère unité d'un espace Euclidien, et l'analyse en composantes principales permet d'obtenir une bonne approximation de ces points à l'aide d'un sous-espace Euclidien. Prenant en compte une telle situation géométrique, le présent article suggère de représenter les variables sur une sphère tri-dimensionelle optimale.

     

First insights into the biodiversity and biogeography of the Southern Ocean deep sea

Shallow marine benthic communities around Antarctica show high levels of endemism, gigantism, slow growth, longevity and late maturity, as well as adaptive radiations that have generated considerable biodiversity in some taxa. The deeper parts of the Southern Ocean exhibit some unique environmental features, including a very deep continental shelf and a weakly stratified water column, and are the source for much of the deep water in the world ocean. These features suggest that deep-sea faunas around the Antarctic may be related both to adjacent shelf communities and to those in other oceans. Unlike shallow-water Antarctic benthic communities, however, little is known about life in this vast deep-sea region. Here, we report new data from recent sampling expeditions in the deep Weddell Sea and adjacent areas (748-6,348 m water depth) that reveal high levels of new biodiversity; for example, 674 isopods species, of which 585 were new to science. Bathymetric and biogeographic trends varied between taxa. In groups such as the isopods and polychaetes, slope assemblages included species that have invaded from the shelf. In other taxa, the shelf and slope assemblages were more distinct. Abyssal faunas tended to have stronger links to other oceans, particularly the Atlantic, but mainly in taxa with good dispersal capabilities, such as the Foraminifera. The isopods, ostracods and nematodes, which are poor dispersers, include many species currently known only from the Southern Ocean. Our findings challenge suggestions that deep-sea diversity is depressed in the Southern Ocean and provide a basis for exploring the evolutionary significance of the varied biogeographic patterns observed in this remote environment.     

波形钢腹板组合连续箱梁有效翼缘宽度计算初探

针对目前规范中缺少有关波形钢腹板组合连续梁桥有效翼缘宽度的相关规定,提出一种翼缘有效宽度计算方法,以某大跨度波形钢腹板预应力混凝土组合连续箱梁桥为背景,对其有效翼缘宽度计算进行初步研究,研究结果表明:在自重和集中荷载作用下,跨中混凝上内衬边缘的剪力滞效应显著,翼缘板的有效翼缘宽度系数分别达到0.87和0.7左右,其它部位剪力滞效应不明显;而预应力荷载作用下,波形钢腹板组合连续箱梁的各截面处的剪力滞效应均不明显,可以忽略不计,最后通过有限元计算结果与国内外规范对比发现,波形钢腹板箱梁跨中部分有效翼缘宽度与混凝土箱梁基本一致,设计计算时可参照普通混凝土箱梁;内衬边缘截面的剪力滞效应介于普通混凝土箱梁与钢箱梁之间,其有效翼缘宽度的计算也应介于二者之间。