Ciliary proteins Fap43 and Fap44 interact with each other and are essential for proper cilia and flagella beating

Cilia beating is powered by the inner and outer dynein arms (IDAs and ODAs). These multi-subunit macrocomplexes are arranged in two rows on each outer doublet along the entire cilium length, except its distal end. To generate cilia beating, the activity of ODAs and IDAs must be strictly regulated locally by interactions with the dynein arm-associated structures within each ciliary unit and coordinated globally in time and space between doublets and along the axoneme. Here, we provide evidence of a novel ciliary complex composed of two conserved WD-repeat proteins, Fap43p and Fap44p. This complex is adjacent to another WD-repeat protein, Fap57p, and most likely the two-headed inner dynein arm, IDA I1. Loss of either protein results in altered waveform, beat stroke and reduced swimming speed. The ciliary localization of Fap43p and Fap44p is interdependent in the ciliate Tetrahymena thermophila.     

Heritable germline epimutation of MSH2 in a family with hereditary nonpolyposis colorectal cancer

Epimutations in the germline, such as methylation of the MLH1 gene, may contribute to hereditary cancer syndrome in human, but their transmission to offspring has never been documented. Here we report a family with inheritance, in three successive generations, of germline allele-specific and mosaic hypermethylation of the MSH2 gene, without evidence of DNA mismatch repair gene mutation. Three siblings carrying the germline methylation developed early-onset colorectal or endometrial cancers, all with microsatellite instability and MSH2 protein loss. Clonal bisulfite sequencing and pyrosequencing showed different methylation levels in different somatic tissues, with the highest level recorded in rectal mucosa and colon cancer tissue, and the lowest in blood leukocytes. This mosaic state of germline methylation with different tissue distribution could act as the first hit and provide a mechanism for genetic disease inheritance that may deviate from the mendelian pattern and be overlooked in conventional leukocyte-based genetic diagnosis strategy.     

Control of a key transition from prostrate to erect growth in rice domestication

The transition from the prostrate growth of ancestral wild rice (O. rufipogon Griff.) to the erect growth of Oryza sativa cultivars was one of the most critical events in rice domestication. This evolutionary step importantly improved plant architecture and increased grain yield. Here we find that prostrate growth of wild rice from Yuanjiang County in China is controlled by a semi-dominant gene, PROG1 (PROSTRATE GROWTH 1), on chromosome 7 that encodes a single Cys(2)-His(2) zinc-finger protein. prog1 variants identified in O. sativa disrupt the prog1 function and inactivate prog1 expression, leading to erect growth, greater grain number and higher grain yield in cultivated rice. Sequence comparison shows that 182 varieties of cultivated rice, including 87 indica and 95 japonica cultivars from 17 countries, carry identical mutations in the prog1 coding region that may have become fixed during rice domestication.     

灵敏度分析在惯导平台误差参数辨识中的应用

为了解决传统的惯导平台测漂方案下,惯导平台上陀螺安装误差角的系统级辨识精度不高的问题,对平台漂移的非线性模型进行了灵敏度分析,找出了影响陀螺安装误差角辨识精度的主要因素,进而提出了转台相对地面存在角速率条件下的改进辨识方案.仿真结果表明,在改进的辨识方案下误差角的辨识精度获得了显著提高.     

金融系统的资源配置功能与经济增长

建立内生增长模型研究金融系统资源配置功能如何促进经济增长 .结论 :通过提高“创新平均成功概率”,增加“创新投资比例”、“储蓄转化比例”和储蓄率 ,金融系统能够促进经济快速稳定增长 .另外本文还得到产出 -资本比“阶跃”增长图象.     

南黄海现代沉积环境及动力沉积体系

基于南黄海600多个表层沉积物样品的研究和对前人大量沉积学资料及水文学资料的分析, 阐述了南黄海现代沉积环境和动力沉积体系特征, 对存在广泛争议的泥质沉积物形成机制进行了探讨. 南黄海陆架可分为弱动力(低能)沉积环境和强动力(高能)环境, 低能沉积环境进一步划分为气旋型和反气旋型涡旋两种环境;高能环境则分为高能沉积环境和高能侵蚀环境. 陆架低能环境发育了泥质沉积体系. 在南黄海中部, 受海域中尺度气旋型涡旋(冷涡)的控制, 发育了冷涡泥质沉积体系, 而在南黄海东南部受反气旋型涡旋(暖涡)控制则形成了反气旋型涡旋泥质沉积体系(暖涡沉积). 二者在粒度、沉积速率、沉积厚度和矿物学特征上有明显区别. 陆架高能沉积环境发育了砂质沉积, 集中分布在南黄海的西部、南部及东北部海区, 在高能侵蚀环境区分布着大量砂岩砾石. 高能沉积环境中原有的细粒物质(黏土、细粉砂等)逐渐被悬浮、搬运至低能区沉积. 还建立了气旋型涡旋和反气旋型涡旋泥质沉积作用模式, 对南黄海泥质沉积体系的成因给出了系统的解释.     

不确定切换系统的动态输出反馈鲁棒H∞控制

研究一类由任意有限多个不确定子系统组成的切换系统的动态输出反馈鲁棒H∞控制问题.利用共同Lyapunov函数方法和凸组合技术,给出由矩阵不等式表示的控制器存在的充分条件,并设计了相应的子控制器和切换规则.采用消元法,将该矩阵不等式转化为一组线性矩阵不等式(LMIs).最后给出一个数值仿真实例证明结论的有效性.研究结果表明:通过切换,闭环系统在整个状态空间上的每个点都满足鲁棒H∞性能,而并不要求每个子系统在整个状态空间上都满足鲁棒H∞性能,甚至也不要求其渐进稳定.     

Mutations in NMNAT1 cause Leber congenital amaurosis and identify a new disease pathway for retinal degeneration

Leber congenital amaurosis (LCA) is a blinding retinal disease that presents within the first year after birth. Using exome sequencing, we identified mutations in the nicotinamide adenine dinucleotide (NAD) synthase gene NMNAT1 encoding nicotinamide mononucleotide adenylyltransferase 1 in eight families with LCA, including the family in which LCA was originally linked to the LCA9 locus. Notably, all individuals with NMNAT1 mutations also have macular colobomas, which are severe degenerative entities of the central retina (fovea) devoid of tissue and photoreceptors. Functional assays of the proteins encoded by the mutant alleles identified in our study showed that the mutations reduce the enzymatic activity of NMNAT1 in NAD biosynthesis and affect protein folding. Of note, recent characterization of the slow Wallerian degeneration (Wld(s)) mouse model, in which prolonged axonal survival after injury is observed, identified NMNAT1 as a neuroprotective protein when ectopically expressed. Our findings identify a new disease mechanism underlying LCA and provide the first link between endogenous NMNAT1 dysfunction and a human nervous system disorder.     

关联交叉口短时交通流可预测性分析及组合预测算法

文中首先根据交叉口交通流参数时间序列的相关性对关联交叉口进行定义,给出关联交叉口短时交通流可预测分析的几个定量指标,以及交通流时间序列最大Lya-punov指数的计算方法;然后提出在短时交通流时间序列的可预测分析基础上,选取一组预测模型并建立基于RBF网络的非线性组合预测模型,提出了关联交叉口短时交通流的组合预测算法;最后对实测短时交通流进行仿真试验,结果表明组合预测方法相对于单项预测方法具有更好的预测性能.     

Characterization and Preparation of Bimetallic Nanoparticles

1 Results Bimetallic particles in the nanometer size range are of substantial interest due to their vast applications in catalysis[1].The synthesis of bimetallic nanoparticles with definite size with a well-control over their nanostructure remains a challenging problem.Thus there exists a great demand for both synthesis and atomic level characterization of nanostructure of bimetallic nanoparticles (NPs).With the recent advent of high-intensity tunable sources of X-rays,now available at synchrotron radia...