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The ratio of C26:0/C22:0 fatty acids in patient lipids is widely accepted as a critical clinical criterion of peroxisomal diseases, such as Zellweger syndrome and X-linked adrenoleukodystrophy (X-ALD). However, phospholipid molecular species with very long chain fatty acids (VLCFA) have not been precisely characterized. In the present study, the structures of such molecules in fibroblasts of Zellweger syndrome and X-ALD were examined using LC–ESI–MS/MS analysis. In fibroblasts from Zellweger patients, a large number of VLCFA-containing molecular species were detected in several phospholipid classes as well as neutral lipids, including triacylglycerol and cholesteryl esters. Among these lipids, phosphatidylcholine showed the most diversity in the structures of VLCFA-containing molecular species. Some VLCFA possessed longer carbon chains and/or larger number of double bonds than C26:0-fatty acid (FA). Similar VLCFA were also found in other phospholipid classes, such as phosphatidylethanolamine and phosphatidylserine. In addition, VLCFA-containing phospholipid species showed some differences among fibroblasts from Zellweger patients. It appears that phospholipids with VLCFA, with or without double bonds, as well as C26:0-FA might affect cellular functions, thus leading to the pathogenesis of peroxisomal diseases, such as Zellweger syndrome and X-ALD.  相似文献   
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Graphitic microtexture in carbon nanospheres (CNSs, average diameter = 500–1000 nm) formed by a heat-treatment between 2000 °C and 2900 °C was investigated with XRD, Raman spectrum, SEM and TEM. By the heat-treatment up to 2000 °C, CNS particles in any size were polyhedronized. In the microtexture of polyhedronized CNS particles thus obtained, graphitic stacking structure of aromatic layers was well developed in the polyhedron face regions, while an aggregation of column-like crystallite was confirmed in the polyhedron ridge region. It was also shown that some ridges of CNSs with the average diameter over 700 nm were collapsed by the heat-treatment at 2900 °C. These collapses include an exfoliation of aromatic layers in which the torn layers have hairpin-like microtexture at their ends, and a crack of layer stacks with adjacent layer stacks. According to a direct examination with TEM, it was clarified that a ratio of ridges with either type of damage to a total number of ridges is obviously higher in CNS particles with larger diameter. This phenomenon was discussed in relation to the high crystallinity in the polyhedron face regions and the aggregation of structural defects in the ridge regions.  相似文献   
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Examined the perinatal development of spontaneous body movements and reflexes in Slc:ICR, C3H/He, and BALB/c mice. Mice were observed from Day 18 (a day before normal parturition) to Day 21.5 of gestation. The developmental changes of spontaneous body movements and reflexes occurred quickly. Strain differences in behavioral development were detected in the duration of spontaneous body movements but not in the occurrence of reflexes. Hybrid mice were more active than their parental strains with regard to duration of spontaneous body movements. The rooting reflex and crossed extensor reflex in hybrid mice occurred more frequently on Day 18 than in their parental strains. These results indicate that the perinatal period is characterized by quick qualitative and quantitative changes in behavioral development and by the appearance of these genetic differences. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   
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Thermal conditions and indoor concentrations of aldehydes, volatile organic compounds (VOCs), and NO2 were investigated in 19 occupied temporary houses in 15 temporary housing estates constructed in Minamisoma City, Fukushima, Japan. The data were collected in winter, spring, and summer in January to July 2012. Thermal conditions in temporary log houses in the summer were more comfortable than those in pre‐fabricated houses. In the winter, the indoor temperature was uncomfortably low in all of the houses, particularly the temporary log houses. Indoor air concentrations for most aldehydes and VOCs were much lower than the indoor guidelines, except for those of p‐dichlorobenzene, acetaldehyde, and total VOCs. The indoor p‐dichlorobenzene concentrations exceeded the guideline (240 μg/m3) in 18% of the temporary houses, and the 10?3 cancer risk level (91 μg/m3) was exceeded in winter in 21% due to use of moth repellents by the occupants. Indoor acetaldehyde concentrations exceeded the guideline (48 μg/m3) in about half of the temporary houses, likely originating from the wooden building materials. Indoor NO2 concentrations in the temporary houses were significantly higher in houses where combustion heating appliances were used (0.17 ± 0.11 ppm) than in those where they were not used (0.0094 ± 0.0065 ppm).  相似文献   
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An intelligent shRNA expression device (iRed) contains the minimum essential components needed for shRNA production in cells, and could be a novel tool to regulate target genes. However, general delivery carriers consisting of cationic polymers/lipids could impede function of a newly generated shRNA via electrostatic interaction in the cytoplasm. Recently, we found that faint electric treatment (fET) of cells enhanced delivery of siRNA and functional nucleic acids into the cytoplasm in the absence of delivery carriers. Here, we examined fET of cells stably expressing luciferase in the presence of iRed encoding anti-luciferase shRNA. Transfection of lipofectamine 2000 (LFN)/iRed lipoplexes showed an RNAi effect, but fET-mediated iRed transfection did not, likely because of the endosomal localization of iRed after delivery. However, fET in the presence of lysosomotropic agent chloroquine significantly improved the RNAi effect of iRed/fET to levels that were higher than those for the LFN/iRed lipoplexes. Furthermore, the amount of lipid droplets in adipocytes significantly decreased following fET with iRed against resistin in the presence of chloroquine. Thus, iRed could be a useful tool to regulate target genes following fET-mediated cytoplasmic delivery with endosomal escape devices.  相似文献   
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