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排序方式: 共有685条查询结果,搜索用时 15 毫秒
21.
Genetic Creutzfeldt–Jakob disease (gCJD) is a subtype of genetic prion diseases (gPrDs) caused by the accumulation of mutated pathological prion proteins (PrPSc). gCJD has a phenotypic similarity with sporadic CJD (sCJD). In Japan, gCJD with a Val to Ile substitution at codon 180 (V180I-gCJD) is the most frequent gPrD, while the mutation is extremely rare in countries other than Japan and Korea. In this article, we aim to review previously elucidated clinical and biochemical features of V180I-gCJD, expecting to advance the understanding of this unique subtype in gCJD. Compared to classical sCJD, specific clinical features of V180I-gCJD include older age at onset, a relatively slow progression of dementia, and a lower positivity for developing myoclonus, cerebellar, pyramidal signs, and visual disturbance. Diffuse edematous ribboning hyperintensity of the cerebral cortex, without occipital lobes in diffusion-weighted magnetic resonance imaging, is also specific. Laboratory data reveal the low positivity of PrPSc in the cerebrospinal fluid and periodic sharp wave complexes on an electroencephalogram. Most patients with V180I-gCJD have been reported to have no family history, probably due to the older age at onset, and clinical and biochemical features indicate the specific phenotype associated with the prion protein gene mutation. 相似文献
22.
Methyl formate synthesis during growth on methanol by methylotrophic yeasts has been considered to play a role in formaldehyde detoxification. An enzyme that catalyses methyl formate synthesis was purified from methylotrophic yeasts, and was suggested to belong to a family of alcohol dehydrogenases (ADHs). In this study we report the gene cloning and gene disruption analysis of three ADH-encoding genes in the methylotrophic yeast Candida boidinii (CbADH1, CbADH2 and CbADH3) in order to clarify the physiological role of methyl formate synthesis. From the primary structures of these three genes, CbAdh1 was shown to be cytosolic and CbAdh2 and CbAdh3 were mitochondrial enzymes. Gene products of CbADH1, CbADH2 and CbADH3 expressed in Escherichia coli showed both ADH- and methyl formate-synthesizing activities. The results of gene-disruption analyses suggested that methyl formate synthesis was mainly catalysed by a cytosolic ADH (CbAdh1), and this enzyme contributed to formaldehyde detoxification through glutathione-independent formaldehyde oxidation during growth on methanol by methylotrophic yeasts. 相似文献
23.
A pressure and wind tunnel test was conducted to obtain the basic data for wind resistance design of a mechanically anchored waterproofing membrane system. The test specimen was a flat roof with the following dimensions: 2.4 m in width, 3 m in length and 0.29 m in height. The waterproofing material was polyvinyl chloride sheet reinforced with polyester fiber (PVC sheets). In the pressure test, because the applied pressure was equivalent to the pressure on the entire surface area of the roof, the billowing heights of the PVC sheet around the fastener had almost the same maximum values; therefore, the axial force at the fastener was also similar to the pressure induced by a compressor, and no lateral forces were measured. On the other hand, in the wind tunnel test, the strain of the PVC sheet around the fastener at windward side was larger than that of the leeward side. The lateral force was 70% of the axial force at a mean wind speed of 38.6 m/s. Therefore, it was clear that the characteristics of the mechanically anchored waterproofing membrane system in the pressure test and the wind tunnel test were different. 相似文献
24.
Masaki Saito Marina Hirano Tomohiro Izumi Yu Mori Kentaro Ito Yurika Saitoh Nobuo Terada Takeya Sato Jun Sukegawa 《International journal of molecular sciences》2022,23(4)
The primary cilium is a hair-like immotile organelle with specific membrane receptors, including the receptor of Hedgehog signaling, smoothened. The cilium organized in preosteoblasts promotes differentiation of the cells into osteoblasts (osteoblast differentiation) by mediating Hedgehog signaling to achieve bone formation. Notably, 4.1G is a plasma membrane-associated cytoskeletal protein that plays essential roles in various tissues, including the peripheral nervous system, testis, and retina. However, its function in the bone remains unexplored. In this study, we identified 4.1G expression in the bone. We found that, in the 4.1G-knockout mice, calcium deposits and primary cilium formation were suppressed in the trabecular bone, which is preosteoblast-rich region of the newborn tibia, indicating that 4.1G is a prerequisite for osteoblast differentiation by organizing the primary cilia in preosteoblasts. Next, we found that the primary cilium was elongated in the differentiating mouse preosteoblast cell line MC3T3-E1, whereas the knockdown of 4.1G suppressed its elongation. Moreover, 4.1G-knockdown suppressed the induction of the cilia-mediated Hedgehog signaling and subsequent osteoblast differentiation. These results demonstrate a new regulatory mechanism of 4.1G in bone formation that promotes the primary ciliogenesis in the differentiating preosteoblasts and induction of cilia-mediated osteoblast differentiation, resulting in bone formation at the newborn stage. 相似文献
25.
Reuben Wu Karen A. Kozielski Patrick G. Hartley Eric F. May John Boxall Nobuo Maeda 《American Institute of Chemical Engineers》2013,59(7):2640-2646
Induction time distributions for gas hydrate formation were measured for gas mixtures of methane + propane at pressures up to 11.3 MPa using a high‐pressure automated lag time apparatus (HP‐ALTA). Measurements were made at subcooling temperatures between 4.3 and 13.5 K and, while isothermal induction times between 0 and 15,000 s were observed, the median isothermal induction times for the distributions ranged from 100 to 4000 s. A hyperbolic relationship between median induction time and subcooling was used to correlate the data. A graphical interpretation is presented that relates the two types of data that can be acquired by using the HP‐ALTA in one of two modes to study hydrate formation: induction time distributions at constant subcooling and formation temperature distributions observed during linear cooling ramps. The equivalence of these two modes provides a robust method for studying the variation of formation phenomena in different hydrate systems. © 2013 American Institute of Chemical Engineers AIChE J, 59: 2640–2646, 2013 相似文献
26.
Several Fe-Cr-Ni austenitic stainless steels (Cr wt.%: 13–25, Ni wt.%: 15) were oxidized in steam for 1000 hr at 500–900°C. The oxide scales were examined and categorized with respect to the chromium concentration and the grain size of the base metal. Experiments showed three conditions for the critical bulk Cr concentration and the oxidation temperature at which the oxidation behavior changed drastically. Metallographic examination showed that two of these three conditions resulted from the internal-external transition of Cr2O3 either on the metal surface or along the grain boundaries of the base metal. Attempts were made to interpret these conditions from the available oxidation theories. Atkinson's treatment was employed with some modification to incorporate the grain-boundary diffusion of Cr in the base metal. The calculation basically explained the internal-external transition for the oxidation of these steels. 相似文献
27.
天津以及周边近代化遗产的思考 总被引:1,自引:0,他引:1
小序2006年9月我们从日本来到天津大学任教,我们在天津工作的主要目标是:(1)成立文化遗产的保护研究中心;(2)参与中国的文化遗产保护研究和规划设计;(3)从事文化遗产学的教学。我们关注传统建筑的保护研究,同时也深感近代遗产研究是一个薄弱环节。 相似文献
28.
从北洋水师大沽船坞保护到天津滨海新区总体规划 总被引:1,自引:0,他引:1
北洋水师大沽船坞100年前曾经在直隶创造了中国近代工业的先驱性业绩,如今对这个遗址的保护集中反映了现阶段中国对工业遗产的认识和保护情况。此外,在塘沽海河沿岸还分布着28个不同等级的文物,如何从滨海新区总体规划的角度考虑文化遗产群是值得深思的问题。文章首先记录了大沽船坞的保护过程,分析保护第一线的若干特征;其次,将文物调查数据和中心商务区进行比较,指出文物所在地和中心商务区高度重合的问题;第三,分析以往塘沽总体规划的历程和滨海新区文化定位的重要性。文章的结论是,在世界创意城市的背景下,不仅应该考虑城市的经济定位,更应该从建设之初就考虑其文化定位。 相似文献
29.
To characterize the highly nonlinear hysteretic behavior of high damping rubber (HDR) bearings, a previously developed mathematical hysteresis model, rather than a simplified bilinear hysteresis model, is adopted in this study. Unilateral and bilateral seismic simulation tests of a scaled-down multistory structure isolated with HDR bearings subjected to three recorded earthquakes were conducted. Based on the unilateral test results, different sets of model parameters can be identified. The fidelity of the adopted hysteresis model is investigated by comparing the analytical predictions using all sets of identified parameters with results measured from the tests. The significant influence ascribed to different ground motion characteristics such as the effects of near-field and far-field earthquakes on modeling the hysteretic behavior of HDR bearings is observed. Besides, a feasibility study on applying the identified parameters of the adopted hysteresis model to predict the bilateral seismic responses is performed. Even though the prediction accuracy of the seismic responses is satisfactory in two orthogonally horizontal directions, the bilateral hysteretic modeling of HDR bearings requires further intensive studies considering the coupling effect under bilateral excitations. 相似文献
30.
Nobuo Otsuka 《Corrosion Science》2011,(6):2269-2276
Twenty-three tube deposits taken from seven heat-recovery boilers of municipal solid waste incinerators were examined by chemical analyses and X-ray diffraction. These deposits were measured by Differential Scanning Calorimeter (DSC) in N2 to investigate their melting characteristics. Sixteen deposits were used to evaluate their corrosiveness to carbon steel by high-temperature corrosion test conducted at 400 °C for 20 h in 1500 ppm HCl – 300 ppm SO2 – 7.5%O2 – 7.5%CO2 – 20%H2O – N2. Total heat of endothermic reactions of the deposits taking place between 200 and 400 °C can be related to the corrosion rate of carbon steel at 400 °C. Corrosion initiated at temperatures when the deposits started to melt, became severe when fused salt constituents increased, and alleviated when the majority of the deposits became fused. The corrosion can be interpreted as fused salt corrosion caused by chloride and sulfate salts. 相似文献