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91.
Galactocerebrosidase (GALC) is responsible for the lysosomal catabolism of certain galactolipids, including galactosylceramide and psychosine. Patients with GALC deficiency have an autosomal recessive disorder known as globoid cell leukodystrophy (GLD) or Krabbe disease. Storage of undegraded glycolipids results in defective myelin and the characteristic globoid cells observed on pathological examination of the central and peripheral nervous systems. Most patients have the infantile form of GLD, although older individuals are also diagnosed. Recently the human, mouse, and canine GALC genes were cloned, and mutations causing GLD have been identified. We now describe the construction of a vector containing human GALC cDNA (MFG-GALC), and the transduction of cultured skin fibroblasts from molecularly characterized Krabbe disease patients, as well as rat brain astrocytes and human CD34(+) hematopoietic cells, using retrovirus produced by the psi-CRIP amphotropic packaging cell line. The transduced fibroblasts showed extremely high GALC activity (up to 20,000 times pretreatment levels, about 100 times normal). GALC was secreted into the media and was taken up by untransduced fibroblasts from the same or a different patient. Mannose-6-phosphate receptor-mediated uptake was only partially responsible for the efficient transfer of GALC to neighboring cells. Additional studies confirmed the presence of normal GALC cDNA and mRNA in the transduced cells. The GALC produced by the transduced cells and donated to neighboring untransduced cells was localized to lysosomes as demonstrated by the normal metabolism of [14C]stearic acid-labeled galactosylceramide produced from endocytosed [14C]sulfatide.  相似文献   
92.
Increasing demands for up-to-date road network and the availability of very-high-resolution (VHR) satellite images as well as the popularity of high-speed computers provide motivation and preliminary materials for researchers to propose more advanced approaches in order to increase the automation and robustness of road extraction strategies. In this article, road characteristics are modelled via object-based image analysis (OBIA). Object-based information is embedded as heuristic information in the ant colony optimization (ACO) algorithm for handling the road network extraction problem. A new neighbourhood definition in object space is introduced, which affects the transition rule in order to decrease the road gaps. Furthermore, an innovative desirability function for ACO is designed, which extracts the road objects, competently. The experimental results demonstrate the efficiency of the proposed algorithm for road extraction from VHR images. Moreover, the results of two state-of-the-art methods are compared with the proposed algorithm.  相似文献   
93.
Sheath blight disease in rice has caused major crop losses worldwide. Managing the causal agent of disease Rhizoctonia solani Kühn is difficult because of its broad host range and formation of sclerotia which can survive in harsh environmental conditions; therefore developing innovative disease management methods without application of hazardous chemicals has been considered as the main concern to maintain sustainable agriculture. This presented research has revealed the negative impact of silver nanoparticles (SNPs) on R. solani and disease progress both in vitro and in vivo. The adverse effects of the SNPs on R. solani are significantly dependent on the quantity of SNPs, sprayed at different concentrations in vitro. The highest inhibition level against sclerotia formation and mycelia growth are 92 and 85%, respectively, at a SNPs concentration of 50 ppm. In vivo glasshouse experiments also showed that SNPs at the same concentration favourably affects both the fresh and dry weight of rice plants with a remarkable suppressive effect on the lesion development in leaves.Inspec keywords: antibacterial activity, silver, nanoparticles, nanomedicine, plant diseases, cropsOther keywords: in vitro antifungal properties, in vivo antifungal properties, silver nanoparticles, rice sheath blight disease, major crop losses, causal agent, Rhizoctonia solani Kühn, sclerotia, harsh environmental conditions, disease management methods, hazardous chemicals, sustainable agriculture, negative impact, inhibition level, mycelia growth, in vivo glasshouse experiments, rice plants, suppressive effect, lesion development, leaves  相似文献   
94.
95.
The effects of the deep cryogenic heat treatment on the microstructural changes,wear resistance,and hardness of carburized DIN 1.7131 grade steel were investigated.Results show that cryogenic heat treatment reduced the retained austenite and increased the carbide amount.In addition,after the cryogenic heat treatment,carbide shows a more uniform distribution,as compared to the conventionally treated ones.It was also clarified that the hardness of the cryogenically treated samples was improved,but the relative improvement decreases with the distance as the surface increases.It has been shown that the wear resistance improves due to the cryogenic heat treatment,and the predominant wear mechanism is a combination of the adhesive and tribo-chemical wear.  相似文献   
96.
Natural phosphate (NP) and synthetic fluorapatite phosphate (SFAP) were proposed as stable, inexpensive, readily available and recyclable catalysts for the condensation of 1,2-diamines with 1,2-dicarbonyls in methanol to afford quinoxaline at room temperature. NP provided as high as 92–99% yield for quinoxalines in short reaction times (i.e., 1–45 min), while SFAP created quinoxalines with 87–97% yield in 60–120 min. From the chemical analyses, X-ray fluoresecency, X-ray diffraction, energy dispersive X-ray and Fourier-transform infrared spectroscopy methods, two main phases (CaO, P2O5) appeared in NP together with other low content phases (SiO2, Fe2O3). Compared to other phases, apatite (CaO and P2O5 as Ca10(PO4)6) played a major role in the catalytic activity of NP. SFAP with similar Ca/P atomic ratio showed a relatively lower catalytic activity than NP for the condensation of 1,2-diamine with 1,2-dicarbonyl in methanol at ambient temperature. To investigate the recyclability of catalysts, the surface properties of NP and 6-recycled NP were investigated using scanning electron microscopy, energy dispersive X-ray and Brunauer–Emmett–Teller and Barrett–Joyner–Halenda methods. Some differences were observed in NP and 6-recycled NP’s particle size, surface area, the volume and size of pores, and the content of elements; nevertheless, the use–reuse process did not noticeably change the catalytic property of NP.  相似文献   
97.
Experimental and numerical studies were conducted on the performance and capacity of structured packings for mixture separation with very low separation factor. Different formations and conditions of the innovative structured packing PACK‐2100 with high surface area were studied to evaluate the main characteristics such as dry pressure drop. In addition, numerical simulation was performed to describe the details of the flow structure in these modified structured packings. Three‐dimensional computational fluid dynamics modeling of PACK‐2100 allowed for comparing the pressure drop in both laminar and turbulent flow regime. The obtained experimental and numerical data demonstrate the potential contribution of the packing to high‐efficiency systems with low separation factor.  相似文献   
98.
A new aromatic diamine, 2,3‐bis(4‐(4‐amino‐2‐(trifluoromethyl) phenoxy)phenyl)naphtho[2,3‐f]quinoxaline‐7,12‐dione, was synthesized and fully characterized by using FTIR, 1H and 13C NMR, DEPT technique, and elemental analysis. A series of novel fluorescent anthraquinone‐quinoxaline containing polyamides (PAs) with inherent viscosities of 0.39–0.62 dL/g was prepared by direct polycondensation of the diamine with various dicarboxylic acids. These PAs were readily soluble in many polar aprotic organic solvents and could be solution‐cast into tough and flexible films. The PAs exhibited glass transition temperatures (Tg)s between 230 and 323°C, and 10% weight loss temperatures in the range of 362–433°C in N2. All of the PAs have fluorescence emission in solution and in solid state with maxima around 452–510 nm and with the quantum yields in the range of 6–17%. Also, cyclic voltammetry (CV) method was used to study the electrochemical oxidation behavior of these polymers at the surface of a modified multiwalled carbon nanotube (MWCNT)s glassy electrode. © 2012 Wiley Periodicals, Inc. J. Appl. Polym. Sci., 2013  相似文献   
99.
An efficient method was developed for the preparation of three tetraorganodistannoxane complexes, (C2H5)8Sn4Cl2(CH3O)2O2 (1), (CH3)8Sn4(C3H4N3S)2(CH3O)2O2 (2) and (C4H9)8Sn4(C3H4N3S)2(CH3O)2O2 (3). All prepared complexes were characterized by infrared, 1H, 13C and 119Sn NMR spectroscopes and elemental analysis. The molecular structure of a representative complex (1) was determined by single-crystal X-ray diffraction. Results showed that 1 is a tetranuclear, centrosymmetric dimeric, and contains two endo-cyclic five-coordinated tin atoms and two exo-cyclic five-coordinated tin atoms. Compound 1 lies about a center of inversion and the tetranuclear molecule features a three-ring-staircase Sn4O4 core. The asymmetric unit of 1 contains two independent Sn(IV) atoms and a 2D infinite rigid chain structure forms via C–H···Cl interactions between tetranuclear units.  相似文献   
100.
A new dimethyltin(IV) complex, {[Me2Sn(O2CNC9H6)]2O}2 (1), was prepared by reaction of dimethyltin(IV) dichloride with the quinoline-2-carboxylic acid and characterized by elemental analysis, IR, 1H-, 13C-, 119Sn-NMR spectroscopes. The structure of 1 was determined by single-crystal X-ray diffraction. The results showed that 1 is a tetranuclear, centrosymmetric dimeric, and contains two endo-cyclic five-coordinated and two exo-cyclic six-coordinated tin atoms and a N-atom of the 2-quinaldic carboxylate ligand coordinated to exo-cyclic tin. Complex 1 was utilized as a precursor for SnO2 nanoparticles by direct thermal decomposition at 500 °C in air. The nano-structure of SnO2 was characterized by scanning electron microscopy and X-ray powder diffraction. The SnO2 core showed a band gap of ~4 eV determined from the UV/visible absorption spectrum. The SnO2 nanoparticles show stable photoluminescence (PL) with an emission centered at 557 nm.  相似文献   
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