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11.
Von Hippel-Lindau disease (VHL) is an autosomal dominant tumour syndrome caused by germline mutations of the VHL tumour suppressor gene located on chromosome 3p25-26. In VHL tumours may occur in 14 different target organs, including the eye. Retinal angiomas are considered the first manifestation of VHL disease in 43% of cases, and the cumulative probability of developing a retinal angioma in one or both eyes rises during each decade of life, reaching 80% for patients over 80 years old. Since 1976 patients with VHL at the University Hospital of Utrecht and their at-risk relatives have been screened periodically by a multidisciplinary team. Long-term follow-up ophthalmological data were analysed with special attention to natural course and results of treatment. In addition, we looked for a genotype-phenotype correlation. Retinal angiomas were found in all families. In one large family with a missense mutation (V170D) of the VHL gene, in which the complete spectrum of visceral- and central nervous system (CNS) features of VHL is present, macular, parapapillary, optic disc and ora serrata angiomas were also found. In general, however, a clear-cut genotype-phenotype correlation could not be found. Only early detection and treatment of peripheral retinal angiomas can be expected to decrease the percentage of patients with decreased visual acuity. Therefore, early detection and treatment of these tumours is of paramount importance. Ophthalmological screening of patients and persons at risk should start as early as possible. In patients with apparently sporadic retinal angiomas it is advisable to perform germline DNA analysis, since the risk of developing VHL is high, especially if the angiomas are bilateral, or unilateral and multifocal, if the patient is young, or if there is a family history suggestive of VHL.  相似文献   
12.
Most metal carbides or slicides may be synthesized at room temperature, by ball milling mixtures of elemental powders for some tens of hours with a vibratory mill. Both stable and metastable compounds containing a high density of defects can be obtained. In general, phases stable at low temperatures are synthesized. This observation allows us to confirm recent estimations for the maximum temperature (600 K) attained in these powders during mechanical alloying. Exceptions are found for some MSi2 suicides with M = titanium, iron or molybdenum for which both low- and high-temperature phases are formed.  相似文献   
13.
Smart surveillance systems are increasingly being used to detect potentially dangerous situations. To do so, the common and easier way is to model normal human behaviors and consider as abnormal any new strange behavior in the scene. In this article, Dominant Sets is adapted to model most frequent behaviors and to detect any unknown event to trigger an alarm. It is proved that after an unsupervised training, Dominant Sets can robustly detect abnormal behaviors. The method is tested in several different cases and compared to other usual clusterization methods such as KNN, mixture of Gaussians or Fuzzy \(K\) -Means to confirm its robustness and performance. The overall performance of abnormal behavior detection based on Dominant Sets is better, being the error ratio at least \(1.5\) points lower than the others.  相似文献   
14.
Plants are commonly attacked by more than one species of herbivore, potentially causing the induction of multiple, and possibly competing, plant defense systems. In the present paper, we determined the interaction between feeding by the phloem feeder silverleaf whitefly (SWF), Bemisia tabaci Gennadius (B-biotype = B. argentifolii Bellows and Perring), and the leaf-chewing beet armyworm (BAW), Spodoptera exigua Hübner, with regard to the induction of volatile compounds from cotton plants. Compared to undamaged control plants, infestation with SWF did not induce volatile emissions or affect the number and density of pigment glands that store volatile and nonvolatile terpenoid compounds, whereas infestation by BAW strongly induced plant volatile emission. When challenged by the two insect herbivores simultaneously, volatile emission was significantly less than for plants infested with only BAW. Our results suggest that tritrophic level interactions between cotton, BAW, and natural enemies of BAW, that are known to be mediated by plant volatile emissions, may be perturbed by simultaneous infestation by SWF. Possible mechanisms by which the presence of whiteflies may attenuate volatile emissions from caterpillar-damaged cotton plants are discussed.  相似文献   
15.
A large part of pharmaceutical manufacturing involves the use of particulate materials. It is well known that both particle size and shape affect the physical characteristics of tablets. An image processing and analysis algorithm based on the invariant image moment technique was developed in this work to determine the particle shape by comparing features (moments) extracted from templates to those extracted from each of the objects in the image. First it determines the particle shape (rectangle, circle, etc.) and then calculates its specific dimensions (diameter, aspect ratio).The statistical validation of the vision system obtained a repeatability of 0.0012 in and 0.5% relative standard deviation and accuracy within 0.1 to 0.9% of the average value considered as true value. Also the pattern recognition technique indicated high precision and accuracy for images containing particles with some level of contact between them. The shape recognition of microcrystalline cellulose (MCC) indicated that particles of equant and acicular shape as defined by USP are predominant. The results suggest that image processing and analysis would be a suitable tool for pharmaceutical process analytical technologies (PAT) and process optimization.  相似文献   
16.
This study documents the feasibility of switching to an aprotic medium in sugar receptor research. The solvent change offers additional insights into mechanistic details of receptor--carbohydrate ligand interactions. If a receptor retained binding capacity in an aprotic medium, solvent-exchangeable protons of the ligand would not undergo transfer and could act as additional sensors, thus improving the level of reliability in conformational analysis. To probe this possibility, we first focused on hevein, the smallest lectin found in nature. The NMR-spectroscopic measurements verified complexation, albeit with progressively reduced affinity by more than 1.5 orders of magnitude, in mixtures of up to 50% dimethyl sulfoxide (DMSO). Since hevein lacks the compact beta-strand arrangement of other sugar receptors, such a structural motif may confer enhanced resistance to solvent exchange. Two settings of solid-phase activity assays proved this assumption for three types of alpha- and/or beta-galactoside-binding proteins, that is, a human immunoglobulin G (IgG) subfraction, the mistletoe lectin, and a member of the galectin family of animal lectins. Computer-assisted calculations and NMR experiments also revealed no conspicuous impact of the solvent on the conformational properties of the tested ligands. To define all possible nuclear Overhauser effect (NOE) contacts in a certain conformation and to predict involvement of exchangeable protons, we established a new screening protocol applicable during a given molecular dynamics (MD) trajectory and calculated population densities of distinct contacts. Experimentally, transferred NOE (tr-NOE) experiments with IgG molecules and the disaccharide Gal'alpha1-3Galbeta1-R in DMSO as solvent disclosed that such an additional crosspeak, that is, Gal'OH2--GalOH4, was even detectable for the bound ligand under conditions in which spin diffusion effects are suppressed. Further measurements with the plant lectin and galectins confirmed line broadening of ligand signals and gave access to characteristic crosspeaks in the aprotic solvent and its mixtures with water. Our combined biochemical, computational, and NMR-spectroscopical strategy is expected to contribute notably to the precise elucidation of the geometry of ligands bound to compactly folded sugar receptors and of the role of water molecules in protein--ligand (carbohydrate) recognition, with relevance to areas beyond the glycosciences.  相似文献   
17.
Summary The complex 5-(C5H5)2Ti (1-OC(O)C6H5)2 was synthesized and activated with MA0 for ethylene polymerization. The resultant polymer is UHMW-PE with a viscosimetric molecular weight of Mv = 6.8 x 106 to 0.7 x 106 g/mol dependent on the temperature of the reaction. The effects of the Al/Ti molar ratio and temperature of the reaction on the catalytic activity and polymer properties were studied.  相似文献   
18.
19.
随着能源成本的增加,水泥行业不断寻求利用石油焦炭等替代燃料,但这些替代燃料的使用增加了物料在预热器、窑、冷却机中的阻塞和堆积。对物料的堵塞和堆积,常用的清除方法会带来许多弊端。利用空气炮防止物料的阻塞和堆积比较有效,可以从熟料质量、熟料产量、耐火材料寿命、能源、安全性等方面达到降低成本的目的。马丁公司在预热器和冷却器的改造方面与水泥行业有非常紧密的合作,墨西哥水泥厂是这种合作关系成功的典范。从1996年至今,墨西哥水泥厂已经安装了大约2900个马丁公司的BIG BLASTER空气炮,每年因为安装空气炮带来的收益达27万美元。基于此,UCC万吨熟料生产线也不例外地选用于马丁公司的空气炮。  相似文献   
20.
Abstract. Since the seminal paper by Dickey and Fuller in 1979, unit‐root tests have conditioned the standard approaches to analysing time series with strong serial dependence in mean behaviour, the focus being placed on the detection of eventual unit roots in an autoregressive model fitted to the series. In this paper, we propose a completely different method to test for the type of long‐wave patterns observed not only in unit‐root time series but also in series following more complex data‐generating mechanisms. To this end, our testing device analyses the unit‐root persistence exhibited by the data while imposing very few constraints on the generating mechanism. We call our device the range unit‐root (RUR) test since it is constructed from the running ranges of the series from which we derive its limit distribution. These nonparametric statistics endow the test with a number of desirable properties, the invariance to monotonic transformations of the series and the robustness to the presence of important parameter shifts. Moreover, the RUR test outperforms the power of standard unit‐root tests on near‐unit‐root stationary time series; it is invariant with respect to the innovations distribution and asymptotically immune to noise. An extension of the RUR test, called the forward–backward range unit‐root (FB‐RUR) improves the check in the presence of additive outliers. Finally, we illustrate the performances of both range tests and their discrepancies with the Dickey–Fuller unit‐root test on exchange rate series.  相似文献   
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