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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.
In the fiber Bragg grating sensing using a superluminescent diode as the light source and an arrayed waveguide grating as the wavelength interrogator, the wavelength measurement error due to the spectral modulation of the superluminescent diode is theoretically formulated and quantitatively analyzed by computer simulation. It is shown that the wavelength measurement errors produced with the use of real superluminescent diodes are in very good agreement with the theoretical results. In order to reduce the wavelength measurement error due to the spectral modulation of superluminescent diode, two different types of remedies are proposed that utilize the temperature switching of superluminescent diode and that adjust the bandwidths of fiber Bragg grating and arrayed waveguide grating, and their efficacies are experimentally demonstrated together with good quantitative agreement with the theoretical calculation.  相似文献   
3.
We have investigated the current pulse width dependence on current-driven magnetization reversal in double-barrier structures using GaMnAs-based magnetic tunneling junctions (MTJ) in order to clarify the origin of low threshold current density for current-driven magnetization reversal. Comparing with the case of single-barrier MTJ, the pulse-width dependence reveals that threshold current density is reduced by double-barrier MTJ. We confirmed that the threshold current density in the order of 104 A/cm2 is estimated considering the effect of current pulse width.  相似文献   
4.
Foliar application of cytozyme to 30-day-old black-gram plants resulted 48% increase of dry matter accumulation. The increase in fresh and dry weights of total plants was largely due to enhanced CO2 assimilation rates which were associated with increased RuBP carboxylase activities. The photochemical characteristics in the isolated chloroplasts exhibited an increase of 32, 28 and 40%, measured as the photoreduction of DCPIP, FeCN and NADP, respectively. Cytozyme treatment also resulted an increase in the chlorophyll content in leaves.  相似文献   
5.
To objectively evaluate the parenchymal echo pattern of cirrhotic liver and chronic hepatitis, the authors applied an image analyzing system (IAS) using a neural network. Autopsy specimens in a water tank (n=13) were used to examine the relationship between the diameter of the regenerative nodule and the coarse score (CS) calculated by IAS. CS was significantly correlated with the diameter of the regenerative nodule (p<0.0001, r=0.966). CS is considered to be useful for evaluating the coarseness of the parenchymal echo pattern  相似文献   
6.
Silica particles dispersed in liquid crystals exhibit a novel behavior such as a unique migrating behavior and alignment. These characteristics are found to depend on the size of the nanoparticles, the surface state of nanoparticles, the liquid crystal (LC) phases, the amplitude and the frequency of applied voltage. These are discussed in terms of anisotropic particle-particle and particle-LC molecules interactions in the anisotropic environment of liquid crystal matrix  相似文献   
7.
The reliability of InP-based HEMTs is studied, focussing on how it is affected by the doped layer material and gate recess structure. Bias-and-temperature stress tests reveal that fluorine-induced donor passivation in the recess region, formed adjacent to the gate electrode, causes the source resistance (Rs) to increase at large drain bias voltages. The increase in Rs can be prevented by using InP or InAlP as the carrier supply layer material instead of InAlAs. On the other hand, the increase in the drain resistance (Rd) does not depend on the material of the carrier supply layer, which suggests that a mechanism different from that in the case of Rs should be considered. It is also found that a deep gate recess suppresses the increase in Rd after long-term stressing.  相似文献   
8.
Yoshino  M. Inoue  K. 《Electronics letters》1994,30(23):1956-1957
The authors demonstrate that an injection locking technique suppresses the chirp in wavelength conversion using a laser diode. Waveform patterns of a 2.5 Gbit/s intensity-modulated signal after 128 km transmission through a normal dispersion singlemode fibre were improved by the injection locking technique  相似文献   
9.
The absorption spectrum of poly(3-alkylthiophene) gel changes drastically in association with a volume instability upon changing the solvent composition and also the temperature. In the expanded state in chloroform and at high temperature, the photoluminescence is much enhanced compared with the shrunken state in ethanol and at low temperature. This gel chromism and anomalous luminescence are discussed in terms of the change of effective conjugation length induced by the steric hindrance and the dynamics of the photoexcited species.  相似文献   
10.
The mean molal activity coefficients of aqueous scandium chloride, nitrate, bromide and perchlorate solutions were determined at 25.0° for dilute to saturated concentrations, together with the activities of water. In the dilute solutions of scandium halides, the activity coefficients were obtained from electromotive force measurements on galvanic cells, and the osmotic coefficients of all four solutions above 0.1 mol kg−1 were determined from the isopiestic measurements. Least-squares equations were fitted to these coefficients, which were then used to calculate the mean molal activity coefficients and water activities. The relationships between these results and the corresponding activity data for other rare earth salts, and to the cation hydration and ionic interactions, are discussed. The results on scandium perchlorate solutions suggested that the inner-sphere hydration number of tervalent scandium ion may be seven.  相似文献   
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