A recurrent mutation in MED12 leading to R961W causes Opitz-Kaveggia syndrome

Opitz-Kaveggia syndrome (also known as FG syndrome) is an X-linked disorder characterized by mental retardation, relative macrocephaly, hypotonia and constipation. We report here that the original family for whom the condition is named and five other families have a recurrent mutation (2881C>T, leading to R961W) in MED12 (also called TRAP230 or HOPA), a gene located at Xq13 that functions as a thyroid receptor-associated protein in the Mediator complex.     

Pineal-related changes in cyclic AMP levels of rat medial basal hypothalamus

Summary Pinealectomy (Px) in adult male rats resulted in increased cyclic AMP accumulation by medial basal hypothalamic (MBH) explants 3 and 7 days after surgery. 24 h after superior cervical ganglionectomy (Gx) an augmented MBH cyclic AMP accumulation was observed. The effects of Px and Gx were additive, as revealed in animals subjected to Gx 3 days after Px.This study was supported by grants from the Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET) (N^o 6638) and from the Fundación Alberto J. Roemmers, Argentina.     

Chemotaxis is not a special case of haptotaxis

Summary Serum peptides containing classical anaphylatoxin (CAT) produce marked chemotactic orientation of human neutrophil granulocytes without modifying cell attachment to the substratum. Furthermore gradients of adhesion produced with gammaglobulins fail to induce morphological orientation of neutrophils. The results suggest that chemotaxis is not a special case of haptotaxis.The excellent technical assistance of Miss M. Schuster, Mrs A. Damschen and Mr M. Arnold is gratefully acknowledged. This work was supported by the Swiss National Science Foundation and the Sandoz-Stiftung zur Förderung der Medizinisch-Biologischen Forschung.     

Biosynthesis ofα- andβ-ecdysone by the CrayfishOrconectes limosus in vivo and by its Y-Organs in vitro

Summary - and -ecdysone were synthesized from labelled cholesterol by premolt crayfish in vivo and by their Y-organs in vitro.     

Lower Cambrian yolk-pyramid embryos from Southern Shaanxi, China

TheCambrianexplosioniswidelyacceptedasthesuddenappearanceofnumerousbilateriananimalphylaatornearthebeginningofCambriantime[1,2 ] .The 5 30 million year oldMaotianshanShalefaunacontainstheoldestgoodwhole bodyfossilsofbilateri ans,documentinganincreasingnumberofpresent dayanimalphyla[3,4 ] orsubphyla[5] (evenincludingvertebrates[6～ 8] aswell)knownfromLowerCambri an .ThebeginningoftheCambrianperiodisdatedat5 43millionyearsago ,whenthefirstlargeandelabo ratefossilburrowstogetherwiththemicrosco…     

一种新数值工具在车辆气动声学开发中的应用

为了优化车辆开发过程的有效性，数值气动声学分析法的实施和应用对汽车制造商来说变得越来越重要。提出了一种结合延迟分离涡流模拟和基于Lighthill方程及声学扰动方程的有限元模型混合数值工具，应用OpenFOAM和Actran软件实现了基于声学有限元法的近场声源区提取及声学压力脉动计算，并尝试应用于车顶扰流板及后视镜区域的气动噪声计算及分析。在兰博基尼Urus车型上，研究了不同车顶扰流板设计的气动声学行为，将仿真结果与斯图加特大学气动声学全尺寸风洞的实验结果进行比较，发现了令人信服的相关性。此外，探讨了扰流板上的主要噪声产生机制，研究了汽车表面压力波动水平随几何形状改变的变化情况。     

The Role of Survey Data in Nowcasting Euro Area GDP Growth

This paper evaluates the impact of new releases of financial, real activity and survey data on nowcasting euro area gross domestic product (GDP). We show that all three data categories positively impact on the accuracy of GDP nowcasts, whereby the effect is largest in the case of real activity data. When treating variables as if they were all published at the same time and without any time lag, financial series lose all their significance, while survey data remain an important ingredient for the nowcasting exercise. The subsequent analysis shows that the sectoral coverage of survey data, which is broader than that of timely available real activity data, as well as their information content stemming from questions focusing on agents' expectations, are the main sources of the ‘genuine’ predictive power of survey data. When the forecast period is restricted to the 2008–09 financial crisis, the main change is an enhanced forecasting role for financial data. Copyright © 2015 John Wiley & Sons, Ltd.     

Ca<Superscript>2+</Superscript> signals,cell membrane disintegration,and activation of TMEM16F during necroptosis

Activated receptor-interacting protein kinase 3 (RIPK3) and mixed lineage kinase domain like (MLKL) are essential components of the necroptotic pathway. Phosphorylated MLKL (pMLKL) is thought to induce membrane leakage, leading to cell swelling and disintegration of the cell membrane. However, the molecular identity of the necroptotic membrane pore remains unclear, and the role of pMLKL for membrane permeabilization is currently disputed. We observed earlier that the phospholipid scramblase and ion channel TMEM16F/anoctamin 6 cause large membrane currents, cell swelling, and cell death when activated by a strong increase in intracellular Ca²⁺. We, therefore, asked whether TMEM16F is also central to necroptotic cell death and other cellular events during necroptosis. Necroptosis was induced by TNFα, smac mimetic, and Z-VAD (TSZ) in NIH3T3 fibroblasts and the four additional cell lines HT₂₉, 16HBE, H441, and L929. Time-dependent changes in intracellular Ca²⁺, cell morphology, and membrane currents were recorded. TSZ induced a small and only transient oscillatory rise in intracellular Ca²⁺, which was paralleled by the activation of outwardly rectifying Cl^? currents, which were typical for TMEM16F/ANO6. Ca²⁺ oscillations were due to Ca²⁺ release from endoplasmic reticulum, and were independent of extracellular Ca²⁺. The initial TSZ-induced cell swelling was followed by cell shrinkage. Using typical channel blockers and siRNA-knockdown, the Cl^? currents were shown to be due to the activation of ANO6. However, the knockdown of ANO6 or inhibitors of ANO6 did not inhibit necroptotic cell death. The present data demonstrate the activation of ANO6 during necroptosis, which, however, is not essential for cell death.     

Control of cerebral size and thickness

The mammalian neocortex is a sheet of cells covering the cerebrum that provides the structural basis for the perception of sensory inputs, motor output responses, cognitive function, and mental capacity of primates. Recent discoveries promote the concept that increased cortical surface size and thickness in phylogenetically advanced species is a result of an increased generation of neurons, a process that underlies higher cognitive and intellectual performance in higher primates and humans. Here, we review some of the advances in the field, focusing on the diversity of neocortical progenitors in different species and the cellular mechanisms of neurogenesis. We discuss recent views on intrinsic and extrinsic molecular determinants, including the role of epigenetic chromatin modifiers and microRNA, in the control of neuronal output in developing cortex and in the establishment of normal cortical architecture.