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1.
Leucine-rich repeat kinase 2 (LRRK2) is a major causative gene of late-onset familial Parkinson’s disease (PD). The suppression of kinase activity is believed to confer neuroprotection, as most pathogenic variants of LRRK2 associated with PD exhibit increased kinase activity. We herein report a novel LRRK2 variant—p.G2294R—located in the WD40 domain, detected through targeted gene-panel screening in a patient with familial PD. The proband showed late-onset Parkinsonism with dysautonomia and a good response to levodopa, without cognitive decline or psychosis. Cultured cell experiments revealed that p.G2294R is highly destabilized at the protein level. The LRRK2 p.G2294R protein expression was upregulated in the patient’s peripheral blood lymphocytes. However, macrophages differentiated from the same peripheral blood showed decreased LRRK2 protein levels. Moreover, our experiment indicated reduced phagocytic activity in the pathogenic yeasts and α-synuclein fibrils. This PD case presents an example wherein the decrease in LRRK2 activity did not act in a neuroprotective manner. Further investigations are needed in order to elucidate the relationship between LRRK2 expression in the central nervous system and the pathogenesis caused by altered LRRK2 activity.  相似文献   
2.
This study presents systematic packaging design tools integrating functional and environmental consequences on product life cycle. To design packaging for sustainability, the trade-offs between functional and environmental aspects of packaging throughout the product life cycle should be considered. However, it is difficult for packaging designers to understand the overall trade-offs because the extent of the design consequences on the entire life cycle of packaging and its contents is unclear. We developed two tools for packaging design: the Life Cycle Association Matrix (LCAM) and the Function Network Diagram (FND). The following three steps, based on literature reviews and interviews with industrial experts, were applied. Firstly, we listed the product functions and design variables related to the functions as the attributes allocated to the product life cycle. Secondly, the attributes were connected appropriately based on causal relationships. Lastly, we identified the factors to support decision making in the packaging design procedure. As a result, the LCAM depicts the design consequences on the life cycle, and the FND determines the stakeholders affected by the design consequences. Two case studies were demonstrated to analyze the trade-offs by using our tools. In the case studies, a liquid laundry detergent bottle and a milk carton were redesigned. The tools identified the design consequences and stakeholders affected by the redesign of the usability and protective function for the detergent and milk cases, respectively. The results showed the significance of understanding the design consequences on the product life cycle by integrating the functional and environmental aspects.  相似文献   
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Abstract— We studied the influence of annealing in air on doped europium in BaMgAl10O17 by performing x‐ray absorption fine‐structure measurements. We determined the oxidation of doped divalent europium by annealing in air at over 500°C. The interatomic distance between the europium and the surrounding oxygen atoms was compressed by oxidation. It also appears that the oxidation process of europium is determined by the diffusion of oxygen into BaMgAl10O17.  相似文献   
5.
Photoinduced Formation of Small Gold Particles in Silica Gels   总被引:2,自引:0,他引:2  
AuCl4 ions in sol-gel-derived silica gels (Au/silica gels) were photoreduced by ultraviolet light irradiation at room temperature. In the presence of ethylene glycol in the silica gels, small Au particles were effectively formed by the irradiation. When the storage temperature of the gels was increased in the dark after the irradiation, the surface plas-mon resonance absorption peak of the Au particles was shifted to longer wavelengths, indicating that the Au particles became large. At a storage temperature below 9°C, Au particles could not be observed in the silica gels. The apparent activation energy of Au particle formation was estimated to be 17. 3 kcal·tmol−1.  相似文献   
6.
High-resolution positive and negative photoresists were used to fabricate ?/4-phase-shifted corrugations by holographic exposure. To avoid mixing these photoresists, cyclised polyisoprene was used as a midlayer. The shape and depth of the corrugations on the positive and negative photoresist regions were almost the same. Single-mode operation at the Bragg wavelength was confirmed in lasers with the corrugation.  相似文献   
7.
This paper presents an application of digital signal processing to data acquired by the radio imaging method (RIM) that was adopted to measure moisture distribution inside the human body. RIM was originally developed for the mining industry; we are applying the method to a biomedical measurement because of its simplicity, economy, and safety. When a two‐dimensional image was constructed from the measured data, the method provided insufficient resolution because the wavelength of the measurement medium, a weak electromagnetic wave in a VHF band, was longer than human tissues. We built and measured a phantom, a model simulating the human body, consisting of two water tanks representing large internal organs. A digital equalizer was applied to the measured values as a weight function, and images were reconstructed that corresponded to the original shape of the two water tanks. As a result, a two‐dimensional image containing two individual peaks corresponding to the original two small water tanks was constructed. The result suggests the method was applicable to biomedical measurement by the assistance of digital signal processing. This technique may be applicable to home‐based medical care and other situations in which safety, simplicity, and economy are important.  相似文献   
8.
We studied morphology of GaAs surfaces and the transport properties of two-dimensional electron gas (2DEG) on vicinal (111)B planes. Multi-atomic steps (MASs) are found on the vicinal (111)B facet grown by molecular beam epitaxy, which will affect electron transport on the facet. We also studied how the morphology of GaAs epilayers on vicinal (111)B substrates depends on growth conditions, especially on the As4 flux. The uniformity of MASs on the substrates have been improved and smooth surfaces were obtained when the GaAs was grown with high As4 flux, providing step periodicity of 20 nm. The channel resistance of the 2DEG perpendicular to the MASs is reduced drastically with this smooth morphology. These findings are valuable not only for fabricating quantum devices on the (111)B facets but also those on the vicinal (111)B substrates.  相似文献   
9.
This paper presents advanced techniques to determine all independent elastic-stiffness coefficients C/sub ij/, the associated internal friction Q/sub ij//sup -1/, and piezoelectric coefficients e/sub ij/ of monocrystal langasite (La/sub 3/Ga/sub 5/SiO/sub 14/) using a single rectangular parallelepiped specimen. Langasite's crystal structure belongs to the trigonal system with point group 32, and thus possesses six independent C/sub ij/, two e/sub ij/, and two dielectric coefficients /spl epsiv//sub ij/. All of the elastic and piezoelectric coefficients affect the mechanical resonance frequencies of the solid specimen, and measuring them very accurately permits one to determine the C/sub ij/ and e/sub ij/ with known density, dimensions, and e/sub ij/. We developed a piezoelectric tripod to support the specimen upward and measured the free-vibration resonance frequencies with minimum load from its own weight. This weak and stable acoustic coupling ensures accuracy of the frequency measurement better than 10/sup -5/, enough to determine the coefficients reliably. Our C/sub ij/ fall in the range of results measured with previous (conventional) methods. Our e/sub 11/ is smaller than the reported values by 1.2-13%, and e/sub 14/ is larger by 44-97%. For the internal friction measurement, we used a solenoid coil to vibrate the specimen without any contact. The longitudinal-wave internal friction considerably exceeds the shear-wave internal friction, which can be explained by phonon-phonon interactions.  相似文献   
10.
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