A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy

Stargardt-like macular dystrophy (STGD3, MIM 600110) and autosomal dominant macular dystrophy (adMD) are inherited forms of macular degeneration characterized by decreased visual acuity, macular atrophy and extensive fundus flecks. Genetic mapping data suggest that mutations in a single gene may be responsible for both conditions, already known to bear clinical resemblance. Here we limit the minimum genetic region for STGD3 and adMD to a 0.6-cM interval by recombination breakpoint mapping and identify a single 5-bp deletion within the protein-coding region of a new retinal photoreceptor-specific gene, ELOVL4, in all affected members of STGD3 and adMD families. Bioinformatic analysis of ELOVL4 revealed that it has homology to a group of yeast proteins that function in the biosynthesis of very long chain fatty acids. Our results are therefore the first to implicate the biosynthesis of fatty acids in the pathogenesis of inherited macular degeneration.     

Relationship between El Niño /South Oscillation (ENSO) and population outbreaks of some lemmings and voles in Europe

Ecologists have been puzzled by population cycles of lemmings and voles for the over 70 years. At present, our understanding and explanation to this phenomenon remain controversial. Recently, El Niño/South Oscillation has attracted attention of ecologists on its links with population outbreaks of terrestrial animals. This paper aims to investigate the statistical relationship between outbreaks of microtine rodents and ENSO events by scanning available literature. During 1862–1894, outbreaks of Norway lemmings in Norway tended to occur in the Southern Oscillation Index (SOI) peak years or 1 year after the SOI peak years with an approximate significance level (p = 0.057). During 1885–1931, outbreaks of voles in France tended to occur 1 year before the SOI peak years (p = 0.01). During 1946–1993, outbreaks of lemmings and voles in North Finland tended to occur 1 year before the SOI peak years with a significant level (p = 0.022); the peaks of population abundance corresponded well to the SOI trough years (equal to 1 year before the SOI peak years). Outbreaks of common voles in Poland during 1946–1975 tended to occur in the SOI peak years or 1 year before the SOI peak years (p = 0.011), and also tended to occur 1 year before the SOI peak years (p = 0.030). It was also found that the rodent outbreaks in Norway and France, rodent outbreaks in Finland and Poland synchronized well. It was suggested that the ENSO-related climate or food were key factors in causing outbreaks of microtine rodents in Europe.     

Interaction of reelin signaling and Lis1 in brain development

Loss-of-function mutations in RELN (encoding reelin) or PAFAH1B1 (encoding LIS1) cause lissencephaly, a human neuronal migration disorder. In the mouse, homozygous mutations in Reln result in the reeler phenotype, characterized by ataxia and disrupted cortical layers. Pafah1b1(+/-) mice have hippocampal layering defects, whereas homozygous mutants are embryonic lethal. Reln encodes an extracellular protein that regulates layer formation by interacting with VLDLR and ApoER2 (Lrp8) receptors, thereby phosphorylating the Dab1 signaling molecule. Lis1 associates with microtubules and modulates neuronal migration. We investigated interactions between the reelin signaling pathway and Lis1 in brain development. Compound mutant mice with disruptions in the Reln pathway and heterozygous Pafah1b1 mutations had a higher incidence of hydrocephalus and enhanced cortical and hippocampal layering defects. Dab1 and Lis1 bound in a reelin-induced phosphorylation-dependent manner. These data indicate genetic and biochemical interaction between the reelin signaling pathway and Lis1.     

关于本性矩阵的注记

该文给出了欧氏空间Rn的子空间的本性矩阵在与子空间的一一对应关系,以及子空间的直和、正交子空间等方面的一些结果.     

中国近代教育家对大学生的赠言

蔡元培、胡适、竺可桢等几位中国近代著名教育家,在给大学毕业生的赠言中,叮嘱他们出校后不要抛弃学问,要贡献祖国、服务社会,同时也阐述了这样做的原因及要求。教育家们的这些赠言给当前正在求学与就业的大学生提供了许多有益的启示。     

H∞ control for stochastic systems with time-delay

The H∞-control problem of stochastic systems with time-delay is considered. The sufficient conditions are obtained, under which there are always state-feedback control and dynamic output-feedback control so that the resulting closed-loop system is internaly stable and L2 input-output stable in the sense of expectation. Furthermore, the explicit formulas of both kinds of controls are derived. An example is included to illustrate the correctness of theoretic results.     

《材料力学》CAI课件的设计思路及特点

针对《材料力学》传统教学中存在的问题,采用先进的多媒体CAI课件制作手段研制了《材料力学》多媒体CAI课件。文中介绍了《材料力学》多媒体CAI课件的设计思路及特点。并对下一步的研制工作提出展望。     

高等教育大众化阶段地方综合性大学精英教育的发展

大众教育和精英教育是高等教育发展的两个方向。地方综合性大学一方面要发展大众教育,另一方面要有选择地保留精英教育,参与拔尖创新人才的培养。文章对精英教育重要性、可行性进行了分析,对存在的问题和措施进行了探讨。     

Construction of Resilient Functions

Based on the relationship Between nonlinearity and resiliency of a multi-output function, we present a method for constructing nonintersecting linear codes from packing design. Through these linear codes, we obtain n-variable, moutput, t-resilient functions with very high nonlinearity. Their nonlinearities are currently the best results for most of cases.