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排序方式: 共有1291条查询结果,搜索用时 46 毫秒
1.
Jun Ogata Kentaro Hirao Kenya Nishioka Arisa Hayashida Yuanzhe Li Hiroyo Yoshino Soichiro Shimizu Nobutaka Hattori Yuzuru Imai 《International journal of molecular sciences》2021,22(7)
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.
3.
Electrical Breakdown Properties and Space Charge Formation in High Temperature Region in Ultraviolet Ray Irradiated PVC 下载免费PDF全文
Polyvinyl chloride (PVC) is the most popular insulating material for electric wiring instruments. However, an exothermic reaction above 150 °C may cause deterioration of the insulating properties of PVC. Therefore, it is important to clarify the heat degradation in PVC, not only to investigate the ignition of electrical wiring products but also to use electrical products safely. It is known that ultraviolet (UV) irradiation causes chemical deterioration of PVC and an increase in its conductivity. Generally, it has been thought that the electrical breakdown properties, electrical conduction, and insulating performance are affected by space charge accumulation in an insulating material. A high temperature pulsed electroacoustic (PEA) system usable up to 250 °C has been developed, and the PEA system can measure the space charge distribution and conduction current in the high temperature range simultaneously. In this investigation, the space charge distribution and conduction current were measured up to electrical breakdown in a non‐UV irradiated sample (normal PVC) and in 353 nm and 253 nm UV‐irradiated PVC samples in the range from room temperature to 200 °C in a DC electric field. In the short wavelength UV irradiated PVC sample (253 nm, 300 h), a deterioration of breakdown strength at 90 °C to 150 °C and negative packet‐like charges were observed at 60 °C and 100 °C, a positive charge accumulated in front of both the anode and cathode above 90 °C, and a higher electric field near the cathode side because the positive charge of the cathode side was greater. 相似文献
4.
Taro Shimonosono Yoshihiro Hirata Mubin Changgan Syohei Kamei Rina Tokaiya Soichiro Sameshima Toshifumi Yoshidome Katsuhiko Yamaji 《Ceramics International》2018,44(8):8904-8912
This paper reports the performance of porous Gd-doped ceria (GDC) electrochemical cells with Co metal in both electrodes (cell No. 1) and with Ni metal in the cathode and Co metal in the anode (cell No. 2) for CO2 decomposition, CH4 decomposition, and the dry reforming reaction of a biogas with CO2 gas (CH4 + CO2 → 2H2 + 2CO) or with O2 gas in air (3CH4 +?1.875CO2 +?1.314O2 → 6H2 +?4.875CO +?0.7515O2). GDC cell No. 1 produced H2 gas at formation rates of 0.055 and 0.33?mL-H2/(min?m2-electrode) per 1?mL-supplied gas/(min?m2-electrode) at 600?°C and 800?°C, respectively, by the reforming of the biogas with CO2 gas. Similarly, cell No. 2 produced H2 gas at formation rates of 0.40?mL-H2/(min?m2) per 1?mL-supplied gas/(min?m2) at 800?°C from a mixture of biogas and CO2 gas. The dry reforming of a real biogas with CO2 or O2 gas at 800?°C proceeded thermodynamically over the Co or Ni metal catalyst in the cathode of the porous GDC cell. Faraday's law controlled the dry reforming rate of the biogas at 600?°C in cell No. 2. This paper also clarifies the influence of carbon deposition, which originates from CH4 pyrolysis (CH4 → C + 2H2) and disproportionation of CO gas (2CO → C + CO2), on the cell performance during dry reforming. The dry reforming of a biogas with O2 molecules from air exhibits high durability because of the oxidation of the deposited carbon by supplied air. 相似文献
5.
Hideo Sawada Hiroshi Kakehi Masashi Koizumi Yoshihiro Katoh Masashi Miura 《Journal of Materials Science》2007,42(17):7147-7153
Fluoroalkyl end-capped N-(1,1-dimethyl-3-oxobutyl)acrylamide oligomer [RF-(DOBAA)
n
-RF] reacted with tetraethoxysilane (TEOS) and silica nanoparticles in the presence of low-molecular weight biocides such as
hibitane, hinokitiol, and hinokioil under alkaline conditions to afford RF-(DOBAA)
n
-RF/silica nanocomposites-encapsulated these biocides in excellent to moderate isolated yields. Fluoroalkyl end-capped N,N-dimethylacrylamide oligomer [RF-(DMAA)
n
-RF] and acrylic acid oligomer [RF-(ACA)
n
-RF]/silica nanocomposites-encapsulated hibitane were obtained under similar conditions. Dynamic light scattering measurements
showed that the size of these fluorinated nanocomposites-encapsulated biocides thus obtained is nanometer size-controlled.
Additionally, these fluorinated nanocomposites were shown to have a good dispersibility and stability in methanol and water.
Of particular interest, these fluorinated nanocomposites-encapsulated biocides were found to have a good antibacterial activity
against Staphylococcus aureus, and these nanocomposites were applied to the surface modification of traditional organic polymers such as poly(methyl methacrylate). 相似文献
6.
7.
Y Ohe ER Podack KJ Olsen Y Miyahara K Miura H Saito Y Koishihara Y Ohsugi T Ohira K Nishio 《Canadian Metallurgical Quarterly》1993,67(5):939-944
HuIL-6 cDNA, cloned into a neomycin resistant conferring expression vector, BMGNeo, was transfected into Lewis Lung Carcinoma (LLC) cells. LLC cells (5 x 10(6) ml-1) transfected with IL-6 cDNA (LLC-IL6) secreted IL-6 into the culture supernatant at a concentration of 9.9 ng ml-1 within 48 h. When 1,000,000 of untransfected LLC, BMGNeo vector transfected LLC (LLC-Neo) or LLC-IL6 cells were transplanted into C57BL/6 mice subcutaneously, the mean +/- s.d. of survival times of these mice were 33.3 +/- 9.7, 34.3 +/- 7.1 and 17.0 +/- 3.1 days, respectively. The survival time of LLC-IL6 cells transplanted mice was significantly shorter than that of LLC (P < 0.01) or LLC-Neo (P < 0.01) cells transplanted mice without a measurable difference of tumour size. Plasma concentration of IL-6 steadily increased in LLC-IL6 transplanted mice. Body weight and serum albumin were significantly lower in LLC-IL6 transplanted mice than in LLC transplanted mice. Mouse IL-1 alpha and mouse TNF-alpha were not detected in the plasma of LLC-IL6 transplanted mice. These data suggested that secretion of IL-6 from LLC cells was unable to alter net tumour growth rate but rather caused a state similar to cachexia without detectable increase of IL-1 alpha and TNF-alpha in the plasma. This state may be responsible for the shortened survival of LLC-IL6 tumour-bearing mice. 相似文献
8.
Koji Yamada Noboru Miura Chihiro Hamaguchi Norihiko Kamata 《Solid-state electronics》1989,32(12):1113-1117
Hot carrier magnetophonon resonances of n-type Si, short channel InP and p-type InSb were investigated in pulsed high magnetic fields up to 40 T. Using a recently developed high resolution technique in pulsed high fields, many new features of the hot carrier-phonon interactions in high magnetic fields were found. 相似文献
9.
A mica-based machinable glass-ceramic has been converted to a glass-ceramic containing cordiertie and body-centered cubic chondrodite through heat treatment at temperatures higher than that used for the crystallization of mica. The machinability is lost, microhardness increases, and the thermal expansion coefficient decreases with the change of the crystalline phase from mica to cordierite and chondrodite. 相似文献
10.
Ikeda S. Ohta H. Hideo Miura Hagiwara Y. 《Semiconductor Manufacturing, IEEE Transactions on》2003,16(4):696-703
An ideal fabrication process is designed to minimize mechanical stress in semiconductor devices and to improve device reliability. Mechanical stress levels were predicted by in-house simulations supported by a thin-film database. These stress levels were correlated with stress-induced defects by TEM analysis supported by fail bit addressing on matured megabit SRAMs. Amorphous-doped silicon film with various annealing temperatures were used for the gate electrode to change the mechanical stress in devices and to get the direct relationship between predicted stress levels and stress related defects. The authors describe brief guidelines for suppressing dislocations in the small geometry shallow-trench isolation process utilizing this system. Polysilicon thickness in the W-polycide gate electrode is designed to minimize mechanical stress in the gate oxide and to suppress the gate oxide failure in probe and class tests. Moreover, critical stress generates dislocations during post source/drain ion implantation anneal obtained by a ball indentation method. This indicated that lower temperature anneal is effective in suppressing the dislocations. A two-step anneal was introduced to suppress dislocations and to enable higher ion activation. 相似文献