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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.
Since the introduction of bender element tests to soil testing, the reliability of the estimated travel time has been the most serious problem. The author has previously shown a potential solution whereby removing the response of the bender element subsystem from the whole response could dramatically improve the accuracy of the travel time estimation. In order to lay the foundation for estimating the response of the bender element subsystem, this paper examines the correlation between the displacements of the element and the induced feedback signals by employing a self-monitoring element. The response of the self-monitoring element is modeled as a transfer function involving two internal transfer functions that relate the input signals to the displacements and the displacements to the feedback signals, respectively. Using a laser displacement sensor, the displacements are directly measured through the entire surface and reveal the three-dimensional bending motion of the element oscillating in both longitudinal and width directions. The feedback signals are similar to, but inconsistent with, the tip displacements, and an attempt is made to correct the feedback signals. Finally, a conclusion is given on the potential for estimating the response of the bender element subsystem using the self-monitoring element.  相似文献   
3.
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   
4.
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   
5.
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   
6.
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   
7.
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   
8.
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   
9.
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   
10.
Y. Ogino 《Polymer》2006,47(15):5669-5677
Crystallization of polyethylene (PE) blends of low and high molecular weight components under shear flow was studied using time-resolved depolarized light scattering (DPLS), focusing on effects of the high molecular weight component on the shish-kebab structure formation. Anisotropic two-dimensional scattering pattern due to shish-like structure formation was observed above a certain concentration of the high molecular weight PE. The threshold was about 2.5-3 times larger than the chain overlap concentration, suggesting an important role of entanglements of the high molecular weight component. On the basis of these results a gel-spinning-like mechanism for the shish-like structure formation has been proposed. The DPLS results also implied that the shish-like structure was mainly formed from the high molecular weight PE. This was confirmed by small-angle neutron scattering (SANS) and small-angle X-ray scattering (SAXS) measurements on an elongated PE blend of low molecular weight deuterated PE and high molecular weight hydrogenated PE (3 wt%).  相似文献   
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