Trak1 mutation disrupts GABA(A) receptor homeostasis in hypertonic mice

Hypertonia, which results from motor pathway defects in the central nervous system (CNS), is observed in numerous neurological conditions, including cerebral palsy, stroke, spinal cord injury, stiff-person syndrome, spastic paraplegia, dystonia and Parkinson disease. Mice with mutation in the hypertonic (hyrt) gene exhibit severe hypertonia as their primary symptom. Here we show that hyrt mutant mice have much lower levels of gamma-aminobutyric acid type A (GABA(A)) receptors in their CNS, particularly the lower motor neurons, than do wild-type mice, indicating that the hypertonicity of the mutants is likely to be caused by deficits in GABA-mediated motor neuron inhibition. We cloned the responsible gene, trafficking protein, kinesin binding 1 (Trak1), and showed that its protein product interacts with GABA(A) receptors. Our data implicate Trak1 as a crucial regulator of GABA(A) receptor homeostasis and underscore the importance of hyrt mice as a model for studying the molecular etiology of hypertonia associated with human neurological diseases.     

水轮发电机组变结构变参数控制

本文阐述了水轮机变结构变参数调速控制的基本问题,提出了相应的控制策略,并论证该控制策略的稳定性、最优性。仿真结果表明本文提出的策略控制效果优良。     

水轮机调速器智能化自完善控制策略

本文提出了智能化自完善控制策略,通过采用“知识库”存贮最佳的知识与经验,根据受控对象当前运行条件及状态,运用相应的分析逻辑及推理功能,实时地调整控制器结构及参数,以实现逐步改善控制系统性能的目的.文中将所论述的控制策略用于水轮机调速器开发研究.     

Heterozygous deletion of the linked genes ZIC1 and ZIC4 is involved in Dandy-Walker malformation

Dandy-Walker malformation (DWM; OMIM #220200) is a common but poorly understood congenital cerebellar malformation in humans. Through physical mapping of 3q2 interstitial deletions in several individuals with DWM, we defined the first critical region associated with DWM, encompassing two adjacent Zinc finger in cerebellum genes, ZIC1 and ZIC4. Mice with a heterozygous deletion of these two linked genes have a phenotype that closely resembles DWM, providing a mouse model for this malformation.     

Effect of light adaptation on electrical responses of the retinas of four species of bats.

The levels of light adaptation at which the retinas of 4 species of microchiropteran bats became unable to generate electroretinograms were progressively ordered. The order correlated well with light preferences based on activity patterns of the 4 species. These results suggest that the ability of the retina to function in ambient light may govern some natural behaviors of these bats.     

On-line Tool Wear Classification in Unmanned-machining Environments

One of the most important features of the modern ma ch ining system in an “unmanned“ factory is to change tools that have been subjec ted to wear and damage. An integrated tool condition monitoring system composed of multi-sensors, signal processing devices and intelligent decision making pla ns is a necessary requirement for automatic manufacturing processes. An intellig ent tool wear monitoring system will be introduced in this paper. The system is equipped with power consumption, vibration, AE and cutt...     

Polyreactivity increases the apparent affinity of anti-HIV antibodies by heteroligation

During immune responses, antibodies are selected for their ability to bind to foreign antigens with high affinity, in part by their ability to undergo homotypic bivalent binding. However, this type of binding is not always possible. For example, the small number of gp140 glycoprotein spikes displayed on the surface of the human immunodeficiency virus (HIV) disfavours homotypic bivalent antibody binding. Here we show that during the human antibody response to HIV, somatic mutations that increase antibody affinity also increase breadth and neutralizing potency. Surprisingly, the responding naive and memory B cells produce polyreactive antibodies, which are capable of bivalent heteroligation between one high-affinity anti-HIV-gp140 combining site and a second low-affinity site on another molecular structure on HIV. Although cross-reactivity to self-antigens or polyreactivity is strongly selected against during B-cell development, it is a common serologic feature of certain infections in humans, including HIV, Epstein-Barr virus and hepatitis C virus. Seventy-five per cent of the 134 monoclonal anti-HIV-gp140 antibodies cloned from six patients with high titres of neutralizing antibodies are polyreactive. Despite the low affinity of the polyreactive combining site, heteroligation demonstrably increases the apparent affinity of polyreactive antibodies to HIV.     

船舶强度分析中的几个问题

提出在分析现代船舶强度问题时值得重视的几个方面：①整船强度分析;②船体总体翘曲;③疲劳强度;④破舱后总纵强度．采用大连理工大学开发的通用程序ＪＩＦＥＸ９５对２７１６箱集装箱船进行了整船强度分析;作为算例,用薄壁梁有限元对一条３５７箱集装箱船进行了计算;在假定应力范围长期分布服从Ｗｅｉｂｕｌ分布时,形状参数可取为ξ＝１．２１５６－０．００１３３Ｌ．对如何计算热点应力也进行了讨论,并用影响数法计算了破舱后的最大静水弯矩和剪力,它是事半功倍的．推荐了破舱强度分析的准则．     

The three-dimensional structure of a DNA duplex containing looped-out bases

Unpaired bases in DNA have been assigned a possible role in the mechanism of frameshift mutagenesis in sequences with repeated base pairs. They also occur in quasipalindromic DNA sequences, which have been implicated in mutagenesis where there are no repeated base pairs, through the formation of single-stranded hairpin loops. The conformation of unpaired bases in DNA has been the subject of numerous thermodynamic as well as high resolution NMR (nuclear magnetic resonance) studies (reviewed in ref. 4). The NMR studies in solution have shown that the duplex of the tridecamer DNA fragment d(CGCAGAATTCGCG) remains intact, and that the unpaired adenosines are stacked into the duplex. Having crystallized this oligonucleotide and determined its structure, we find its conformation in the crystal is close to that of a B-DNA duplex, with the two additional adenosines looped out from the double helix and causing little disruption of the rest of the structure.