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1.
Two forms of glutathione synthetase deficiency have been described. While one form is mild, causing hemolytic anemia, the other more severe form causes 5-oxo-prolinuria with secondary neurological involvement. Despite the existence of two deficiency phenotypes, Southern blots hybridized with a glutathione synthetase cDNA suggest that there is a single glutathione synthetase gene in the human genome. Analysis of somatic cell hybrids showed the human glutathione synthetase gene (GSS) to be located on chromosome 20, and this assignment has been refined to subband 20q11.2 using in situ hybridization.  相似文献   
2.
We report on a new patient with d-transposition of the great arteries who was found to have deletion of 22q11.2. He had minor facial anomalies, normal T- and B-cell subsets, and transient hypocalcemia. Similar to rare previous reports, our patient's extracardiac manifestations were relatively mild.  相似文献   
3.
Ionic liquids as electrolytes   总被引:5,自引:0,他引:5  
Salts having a low melting point are liquid at room temperature, or even below, and form a new class of liquids usually called room temperature ionic liquids (RTIL). Information about RTILs can be found in the literature with such key words as: room temperature molten salt, low-temperature molten salt, ambient-temperature molten salt, liquid organic salt or simply ionic liquid. Their physicochemical properties are the same as high temperature ionic liquids, but the practical aspects of their maintenance or handling are different enough to merit a distinction. The class of ionic liquids, based on tetraalkylammonium cation and chloroaluminate anion, has been extensively studied since late 1970s of the XX century, following the works of Osteryoung. Systematic research on the application of chloroaluminate ionic liquids as solvents was performed in 1980s. However, ionic liquids based on aluminium halides are moisture sensitive. During the last decade an increasing number of new ionic liquids have been prepared and used as solvents. The general aim of this paper was to review the physical and chemical properties of RTILs from the point of view of their possible application as electrolytes in electrochemical processes and devices. The following points are discussed: melting and freezing, conductivity, viscosity, temperature dependence of conductivity, transport and transference numbers, electrochemical stability, possible application in aluminium electroplating, lithium batteries and in electrochemical capacitors.  相似文献   
4.
The paper delivers the benchmark results for the Michell cantilevers constructed within a half strip, for selected values of the σ T /σ C ratio, σ T , σ C being the admissible stresses in tension and compression, respectively.  相似文献   
5.
The present study investigated the changes in the content of steroids and triterpenoids in C. officinalis hairy root cultures and plants exposed to cadmium stress. The observed effects included the content and composition of analyzed groups of compounds, particularly the proportions among individual sterols (e.g., stigmasterol-to-sitosterol ratio), their ester and glycoside conjugates. The total sterol content increased in roots (by 30%) and hairy root culture (by 44%), whereas it decreased in shoots (by 15%); moreover, these effects were inversely correlated with Cd-induced growth suppression. Metabolic alterations of sterols and their forms seemed to play a greater role in the response to Cd stress in roots than in shoots. The symptoms of the competition between general metabolites (sterols) and specialized metabolites (triterpenoids) were also observed, i.e., the increase of the sterol biosynthesis parallel to the decrease of the triterpenoid content in C. officinalis plant roots and hairy root culture, and the inverse phenomenon in shoots. The similarity of the metabolic modifications observed in the present study on C. officinalis plant roots and hairy roots confirmed the possibility of application of plant in vitro cultures in initial studies for physiological research on plant response to environmental stresses.  相似文献   
6.
This work concerns the investigation of porous polybenzoxazines based on the non-toxic renewable diphenolic acid. The approach described relies on the in situ generation of foaming agent (CO2) during the thermal curing. For this purpose, the previously synthesized benzoxazine monomer from diphenolic acid was thermally polymerized at different temperatures. As the beginning of decarboxylation is about 200 °C, we selected five foaming temperatures (Tf) ranging from 190 to 230 °C. The influence of the foaming temperature on the cellular structure and its dependency on final properties is discussed.  相似文献   
7.
The reaction of mixtures of renewable diphenolic acid (DPA) and its methylesterbenzoxazine derivative (MDP-Bz) has been studied. The DPA was introduced to lower the high temperature needed to complete the curing of the pure benzoxazine. In this way, samples with different DPA/MDP-Bz ratio (0, 2, 5, 10 and 25% of DPA) were investigated. Moreover, high performance flame retardant thermosetting resins with phosphorus were prepared through the mixture of MDP-Bz and a DPA-phosphazene derivative (DPA-PPZ). The curing behavior of these materials was studied by differential scanning calorimetry (DSC). Finally, the properties of the materials were evaluated by termogravimetric analysis (TGA), dynamic mechanical analysis (DMTA), tensile measurements, limiting oxygen index (LOI) and UL-94 Burn Test.  相似文献   
8.
The dialysis patient is prone to elevations in the calcium phosphorus product and hyperparathyroidism, which contributes to valvular and vascular calcification. We present the case of a young lady on chronic dialysis that developed mitral calcification complicated by severe mitral stenosis, caseous calcification and retinal embolization. She subsequently required mitral valve replacement.  相似文献   
9.
Scissors mechanisms are commonly used in safety engineering during the construction of temporary structures, owing to their inherent advantages of foldability, transformability, and reusability. We effectively utilized these scissors mechanism features to develop a lightweight, deployable emergency Mobile Bridge (MB) based on optimization, and control of the folding structure. Here, we discuss the problems of optimal reinforcement layout for the MB by formulating and solving three optimization problems, namely: (a) the load capacity maximization problem, (b) the weight minimization problem, and (c) coupling the load capacity maximization problem and the weight minimization problem. The potential benefits resulting from the application of reinforcement were evaluated using a combination of finite element analysis and an optimization algorithm based on the differential evolution method. The results demonstrate the significant positive influence of the additional reinforcing members. In particular, the limit load was increased by over 10 times, while the weight was decreased to approximately half. The proposed methodology enabled the development of a substantially improved version of the MB characterized by a higher load capacity and lower weight in comparison to the initial bridge design.  相似文献   
10.
Polyethylene (PE) was modified and prepared as double-layer polyethylene/polycaprolactone (PE/PCL) film. Magnetite and casein were added to the PCL-coating film to improve barrier properties and prevent destruction of basic structure of primary polymer PE. Significant improvements were observed with regards to mechanical (tensile strength, elongation at break) and thermal properties, while barrier (O2 permeability) properties were slightly improved. Overall migration values into acetic acid were lower (from 1 to 4.6 mg/dm2) than the upper limit set by the legislation. Specific migration of iron in PE/PCL-Fe samples is also below (µg/L) specific migration limit value set by the legislation (mg/kg).  相似文献   
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