In Silico Analysis of the Molecular-Level Impact of SMPD1 Variants on Niemann-Pick Disease Severity

Sphingomyelin phosphodiesterase (SMPD1) is a key enzyme in the sphingolipid metabolism. Genetic SMPD1 variants have been related to the Niemann-Pick lysosomal storage disorder, which has different degrees of phenotypic severity ranging from severe symptomatology involving the central nervous system (type A) to milder ones (type B). They have also been linked to neurodegenerative disorders such as Parkinson and Alzheimer. In this paper, we leveraged structural, evolutionary and stability information on SMPD1 to predict and analyze the impact of variants at the molecular level. We developed the SMPD1-ZooM algorithm, which is able to predict with good accuracy whether variants cause Niemann-Pick disease and its phenotypic severity; the predictor is freely available for download. We performed a large-scale analysis of all possible SMPD1 variants, which led us to identify protein regions that are either robust or fragile with respect to amino acid variations, and show the importance of aromatic-involving interactions in SMPD1 function and stability. Our study also revealed a good correlation between SMPD1-ZooM scores and in vitro loss of SMPD1 activity. The understanding of the molecular effects of SMPD1 variants is of crucial importance to improve genetic screening of SMPD1-related disorders and to develop personalized treatments that restore SMPD1 functionality.     

Characterization of a plasma polymer coating from an organophosphorus silane deposited at atmospheric pressure for fire-retardant purposes

Protective coatings from diethylphosphatoethyltriethoxysilane (DEPETS) have been deposited on different polymer substrates in a plasma discharge operated at atmospheric pressure. Plasma polymer chemistry and structure were characterized by means of Fourier transform infrared spectroscopy (FTIR), laser desorption ionization-mass spectrometry (LDI-MS), nuclear magnetic resonance (NMR) and scanning electron microscopy (SEM). A chemical structure of the plasma polymer has been proposed based on the coating molecular characterization. Coatings were deposited on polycarbonate (PC) and polyamide 6 (PA6) substrates. The flame retardant properties of coated substrate samples were assessed using cone calorimetry and compared to those of bare substrates. A significant increase in the time to ignition (TTI), up to +143%, was recorded after coating deposition due to the formation of a high-performance barrier layer at the surface of both polymer substrates.     

石油化工生产火灾危险性分析

针对石油化工生产的特点,从物料特性、上艺装置、工艺控制参数、防火防爆安全装置、作业性质和消防安全设施等几方面综合分析其火灾危险性,并提出相应的防范措施,为消防安全评价提供依据。     

基于多传感器数据融合方法的火灾模拟实验炉温度采集系统

基于多传感器算术平均值与分批估计的数据融合方法在火灾模拟实验炉上温度数据采集中的应用，用VB6．0开发了相应的实际应用系统。     

新型磷系膨胀阻燃剂

介绍了一种新型磷酸盐,通过试验分析了其作为膨胀型阻燃剂对聚烯烃及热固性塑料的阻燃机理与阻燃效果;红磷 酚类体系在高温聚合物中有较好阻燃效果     

Retrospective mapping of burnt areas in Central Siberia using a modification of the normalised difference water index

The boreal forest contains almost half the total carbon pool of world forest ecosystems, and so has a very significant role in global biogeochemical cycles. The flux of greenhouse gases in and out of these forests is influenced strongly by disturbances such as diseases, logging and predominantly fire. It is important to quantify these disturbances to enable the modelling of major greenhouse gases. However, because of the remoteness and vastness of the boreal forest, little data is available on the type, extent, frequency and severity of these disturbances in Siberia. For burnt areas, two of the more responsive wavelengths are the short wave infra-red (SWIR) and the near infra-red (NIR). These produce a vegetation index, the normalised difference SWIR (NDSWIR) capable of detecting retrospective disturbances. Here we combine the NDSWIR from MODIS imagery acquired in the summer of 2003 with thermal anomaly data from 1992 to 2003 to detect and date areas which burnt at some point between 1992 and 2003. The semi-automated method is called SWIR and Thermal ANomalies for Detecting Disturbances (STANDD) and is complemented by an Normalised Difference Vegetation Index (NDVI) differencing method using MODIS 2002 and 2003 imagery to ensure reliable detection of area burnt in the year of image acquisition (i.e. 2003). The area of this study covers approximately 3 million km² stretching from Lake Baikal in the south to the Laptev Sea in the north, above the Arctic Circle. Landsat ETM+ images were used to validate the shape and areal extent of the burnt areas resulting in an 81% overall accuracy with a kappa coefficient of agreement of 0.63.     

水喷雾灭火系统在电信枢纽工程燃气锅炉房的应用

分析了燃气锅炉的火灾危险性和水喷雾用于燃气锅炉消防的灭火机理,提出了水喷雾灭火系统的设计参数的选择、系统的控制方法以及在设计中可能涉及到的问题和注意事项,并结合工程实例进行了探讨。     

Post-Event Rumination and Negative Self-Appraisal in Social Phobia Before and After Treatment.

This study investigated the relationship between self-appraisals of performance, symptom severity and post-event rumination in social phobia, and evaluated the effect of treatment on these variables. A socially phobic group and a nonanxious control group performed an impromptu speech and were told that their performance would be evaluated. Participants appraised their performance immediately after the speech and 1 week later, and the frequency of post-event rumination during the week following the speech was assessed. The socially phobic group maintained the negative appraisals of their speech over the week, whereas the nonclinical group showed increased positivity about their performance The socially phobic group also engaged in more negative rumination than controls. Treatment improved perceptions of performance and reduced negative rumination. These results are discussed in the light of cognitive models of social phobia. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Stability of formal thought disorder and referential communication disturbances in schizophrenia.

This study examined the degree to which different types of communication disturbances in the speech of 48 schizophrenia patients and 28 controls were variable and state related versus stable and traitlike. Clinically rated formal thought disorder and 5 types of referential disturbance showed substantial stability within participants over time. The sixth type of referential disturbance, the vague reference, was not stable over time. Formal thought disorder was associated with the severity of core psychotic symptoms in patients, whereas referential disturbances showed little or no association with positive or negative symptom severity. Furthermore, changes in psychotic symptoms over time were accompanied by corresponding changes in formal thought disorder but not referential disturbances. These results support the idea that some types of referential disturbances are traitlike and may be reflective of vulnerability as well as manifest illness. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

静电火灾的产生及其防治

文章详细分析了静电产生的机理，指出积聚静电电荷的多少与材料的电阻率和介电常数有关。讨论了静电火灾发生的条件，指出静电火灾的发生具有隐蔽性、复杂性和突发性。但只要对其规律进行深入地研究，可以找到有效地防止静电火灾发生的办法。