Mutations in WDR62, encoding a centrosome-associated protein, cause microcephaly with simplified gyri and abnormal cortical architecture

Genes associated with human microcephaly, a condition characterized by a small brain, include critical regulators of proliferation, cell fate and DNA repair. We describe a syndrome of congenital microcephaly and diverse defects in cerebral cortical architecture. Genome-wide linkage analysis in two families identified a 7.5-Mb locus on chromosome 19q13.12 containing 148 genes. Targeted high throughput sequence analysis of linked genes in each family yielded > 4,000 DNA variants and implicated a single gene, WDR62, as harboring potentially deleterious changes. We subsequently identified additional WDR62 mutations in four other families. Magnetic resonance imaging and postmortem brain analysis supports important roles for WDR62 in the proliferation and migration of neuronal precursors. WDR62 is a WD40 repeat-containing protein expressed in neuronal precursors as well as in postmitotic neurons in the developing brain and localizes to the spindle poles of dividing cells. The diverse phenotypes of WDR62 suggest it has central roles in many aspects of cerebral cortical development.     

Single-molecule analysis of Mss116-mediated group II intron folding

DEAD-box helicases are conserved enzymes involved in nearly all aspects of RNA metabolism, but their mechanisms of action remain unclear. Here, we investigated the mechanism of the DEAD-box protein Mss116 on its natural substrate, the group II intron ai5γ. Group II introns are structurally complex catalytic RNAs considered evolutionarily related to the eukaryotic spliceosome, and an interesting paradigm for large RNA folding. We used single-molecule fluorescence to monitor the effect of Mss116 on folding dynamics of a minimal active construct, ai5γ-D135. The data show that Mss116 stimulates dynamic sampling between states along the folding pathway, an effect previously observed only with high Mg(2+) concentrations. Furthermore, the data indicate that Mss116 promotes folding through discrete ATP-independent and ATP-dependent steps. We propose that Mss116 stimulates group II intron folding through a multi-step process that involves electrostatic stabilization of early intermediates and ATP hydrolysis during the final stages of native state assembly.     

湿型砂三性能自动快速测定方法及其测定仪

本文研究一种湿型砂三性能自动快速测定方法及其测定仪。该方法是直接测定紧实率的试样的透气压力和湿压强度,代入由试验建立的二元线性回归方程求得标准工艺试样的透气性和湿压强度。该仪器采用用移传感器测定紧实率,用压力传感器测定湿压强度,用气压传感器测定透气压力。仪器的动作程序、检测信号的采集、数据处理及打印险测结果等均由微机控制,自动运行。本方法简单,快速和准确。该仪器采用气压传动,结构简单,制造容易。     

广东省南海膨润土的性能试验研究

广东省南海膨润土矿是近年发现的大型钙基膨润土矿，是广东省首个被综合开发利用的膨润土矿。本文对南海天然钙基膨润土及人工钠基膨润土进行了矿物组成、化学组成的分析研究，对铸造工艺性能进行了试验研究．经试验及生产应用表明，南海膨润土系列产品能满足铸造生产不同的要求。     

Reflective Practices in Community Development: a Grounded Analysis

Systemic Practice and Action Research - Reflective practices (RPs) are recognized as fundamental for the conception, development, implementation and improvement of community-based development in...     

Natural history and ecological niche modelling of coastal Atyphella Olliff Larvae (Lampyridae: Luciolinae) in Vanuatu

ABSTRACT

The genus Atyphella Olliff includes several coastal species with larvae that were collected on coastal rock within the intertidal zone. Recent fieldwork in Vanuatu has expanded the distribution of these insects and begun to provide insight into how they are able to survive in such a unique environment. An ecological niche model is produced using Maxent to predict additional locations of coastal Atyphella in the South Pacific. Larval instars for the two species of Atyphella in Vanuatu are estimated using protergum shield width measurements. Additionally, submersion tests were performed on larvae to determine survivability in saltwater and freshwater environments. These data provide evidence for previously unknown aspects of their natural history that strongly suggest the species are multivoltine and spend a large portion of their larval stage in the intertidal zone.     

Macrophage migration inhibitory factor (MIF) modulates trophic signaling through interaction with serine protease HTRA1

Macrophage migration inhibitory factor (MIF), a small conserved protein, is abundant in the immune- and central nervous system (CNS). MIF has several receptors and binding partners that can modulate its action on a cellular level. It is upregulated in neurodegenerative diseases and cancer although its function is far from clear. Here, we report the finding of a new binding partner to MIF, the serine protease HTRA1. This enzyme cleaves several growth factors, extracellular matrix molecules and is implicated in some of the same diseases as MIF. We show that the function of the binding between MIF and HTRA1 is to inhibit the proteolytic activity of HTRA1, modulating the availability of molecules that can change cell growth and differentiation. MIF is therefore the first endogenous inhibitor ever found for HTRA1. It was found that both molecules were present in astrocytes and that the functional binding has the ability to modulate astrocytic activities important in development and disease of the CNS.