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171.
172.
Ataxia-ocular apraxia 2 (AOA2) was recently identified as a new autosomal recessive ataxia. We have now identified causative mutations in 15 families, which allows us to clinically define this entity by onset between 10 and 22 years, cerebellar atrophy, axonal sensorimotor neuropathy, oculomotor apraxia and elevated alpha-fetoprotein (AFP). Ten of the fifteen mutations cause premature termination of a large DEAxQ-box helicase, the human ortholog of yeast Sen1p, involved in RNA maturation and termination.  相似文献   
173.
Raşa M  Philipse AP 《Nature》2004,429(6994):857-860
The determination of molecular masses from barometric sedimentation profiles, a main topic in ultracentrifugal analysis, is thought to be quantitatively correct for non-interacting particles. Whereas this expectation is justified for uncharged colloids or macromolecules at low volume fractions, early ultracentrifugation studies on charged particles had already indicated that the obtained masses might be much too low. More recently, expanded sedimentation profiles have been observed for charged particles, sometimes inflated by orders of magnitude relative to the barometric prediction, which highlights a shortcoming in our understanding of centrifugation of even very dilute charged species. Theory and simulations, anticipated by various authors, now propose that strongly non-barometric sedimentation profiles might be caused by an internal macroscopic electric field that, even for non-interacting particles, significantly decreases the buoyant particle mass. The existence of this field and its intriguing consequences still lack experimental verification. Here we report ultracentrifugation experiments on charged colloidal silica spheres, showing both the existence of such a macroscopic electric field and its drastic effects on the sedimentation profiles of very dilute dispersions at low ionic strength.  相似文献   
174.
This issue published a research article by Yang et al. of Peking University “Significant divergence of sex-re-lated non-coding RNA expression patterns among closely related species in Drosophila”. In a genomic  相似文献   
175.
曹毅  ab  秦友蕾  陈海  刘凯  周辉  ab 《上海交通大学学报》2016,50(5):702-709
为综合得到完全各向同性解耦并联机器人构型,基于GF集理论提出了一种简单而有效的构型综合方法.阐述GF集的基本概念、运算法则,转动特征存在条件;给出机构输入运动副选择原则和分支设计准则,确保了并联机构运动各向同性及解耦性;根据该构型综合原理,实现了3T1R的4自由度完全各向同性解耦并联机构型综合,得到了大量新构型;针对所综合的一种新型并联机构,运用螺旋理论求解其机构的自由度并推导其雅克比矩阵,验证了该机构的完全各向同性,也进一步证明了该构型综合方法的有效性.  相似文献   
176.
限宽水域中船舶平面运动机构试验及水动力导数数值模拟   总被引:1,自引:0,他引:1  
为探究有限宽度水域中大型船舶的水动力特性,基于计算流体力学(CFD)技术对限宽水域中的平面运动机构(PMM)试验进行数值模拟.在对有限水域宽度的PMM试验的动态模拟上,开发了混合动网格技术,数值计算结果与循环水槽PMM试验结果对比证明采用该方法有效.继而模拟不同宽度水域中的PMM试验,分析船舶水动力随运动速度变化的关系.结果表明,限宽水域中水动力随运动速度变化的非线性特征更为显著,船舶操纵加速度导数增大,位置导数减小,旋转导数的绝对值有所增加,二阶非线性导数值变化较大.  相似文献   
177.
针对广义最小二乘算法在WGS-84坐标系中存在收敛速度慢及收敛性能不稳定的问题,提出了一种改进的广义最小二乘算法.该算法以收敛步数作为最小二乘算法中的量测精度加权因子,使得量测精度随着步数的变化而变化,从而实现了收敛步数的减少和收敛之后稳定性的改善,提高了目标跟踪定位的性能.仿真结果表明,改进的广义最小二乘算法优于广义最小二乘算法,进一步验证了该算法的有效性.  相似文献   
178.
针对Zhang细化算法的不足提出改进算法。首先,根据提取的骨架特点设计细化模板,将骨架进行细化并且保持骨架的连通性和满足单像素,便于后续处理;其次,利用像素找出骨架的分支点,骨架减去分支点形成多个不连通区域,根据骨架特点选择不同的阈值,去除小于该阈值的连通分支,从而去除骨架毛刺,该步骤高效地除去了多余的噪音和毛刺,优化了骨架的视觉效果。仿真实验结果表明用改进算法提取图像骨架比Zhang细化算法得到的骨架效果更好,不但能够根据阈值的选择来除掉长短不一的毛刺,且不会破坏物体结构中重要的骨架。  相似文献   
179.
The tumour-suppressor pathway formed by the alternative reading frame protein of the Cdkn2a locus (Arf) and by p53 (also called Trp53) plays a central part in the detection and elimination of cellular damage, and this constitutes the basis of its potent cancer protection activity. Similar to cancer, ageing also results from the accumulation of damage and, therefore, we have reasoned that Arf/p53 could have anti-ageing activity by alleviating the load of age-associated damage. Here we show that genetically manipulated mice with increased, but otherwise normally regulated, levels of Arf and p53 present strong cancer resistance and have decreased levels of ageing-associated damage. These observations extend the protective role of Arf/p53 to ageing, revealing a previously unknown anti-ageing mechanism and providing a rationale for the co-evolution of cancer resistance and longevity.  相似文献   
180.
In this paper, a serial time-encoded amplified microscopy (STEAM) by employing a multiwavelength laser as the light source is proposed and experimentally demonstrated. This system achieves ultrafast optical imaging with a tunable frame rate. The measuring range depends on the spectrum width of the multiwavelength laser. Through tuning the speed of the modulating signal, the frame rate ranges from 100 to 250 MHz. In addition, the spatial resolution can be improved by increasing the group velocity dispersion and reducing the wavelength spacing. Finally, with the development of photonic integrate circuits (PIC), the multiwavelength laser source has the potential for integration on a photonic chip and thus the size of the proposed STEAM could be reduced in the future.  相似文献   
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