A Novel LRRK2 Variant p.G2294R in the WD40 Domain Identified in Familial Parkinson’s Disease Affects LRRK2 Protein Levels

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.     

Effect of light source spectral modulation on wavelength interrogation in fiber Bragg grating sensors and its reduction

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.     

An automatic torque ripple compensation system for direct drive motors

A serious problem in motion control is the occurrence of torque ripple. Since a direct drive (DD) motor with a magnet rotor uses a rare-earth magnet, the torque ripple is more serious than that of conventional servomotors. This paper presents an auto-compensation of torque ripple using a software-implemented torque observer. The frequency and amplitude of torque ripple can be obtained in the controller by using the estimated torque from an observer, and the autonomous generation of a compensating current component for a torque ripple is possible. The proposed algorithm has been verified by experiments.     

Stabilization of bioethanol recovery with silicone rubber‐coated ethanol‐permselective silicalite membranes by controlling the pH of acidic feed solution

In order to produce highly concentrated bioethanol by pervaporation using an ethanol‐permselective silicalite membrane, techniques to suppress adsorption of succinic acid, which is a chief by‐product of ethanol fermentation and causes the deterioration in pervaporation performance, onto the silicalite crystals was investigated. The amount adsorbed increased as the pH of the aqueous succinic acid solution decreased. The pervaporation performance also decreased with decreasing pH when the ternary mixtures of ethanol/water/succinic acid were separated. Using silicalite membranes individually coated with two types of silicone rubber, pervaporation performance was significantly improved in the pH range of 5 to 7, when compared with that of non‐coated silicalite membranes in ternary mixtures of ethanol/water/succinic acid. Moreover, when using a silicalite membrane double‐coated with the two types of silicone rubber, pervaporation performance was stabilized at lower pH values. In the separation of bioethanol by pervaporation using the double‐coated silicalite membrane, removal of accumulated substances having an ultraviolet absorption maximum at approximately 260 nm from the fermentation broth proved to be vital for efficient pervaporation. Copyright © 2005 Society of Chemical Industry     

Performance prediction of a hybrid‐excitation synchronous machine with axially arranged excitation poles and permanent‐magnet poles

This paper presents a method of predicting the steady‐state performance of a new hybrid‐excitation synchronous machine (HESM) theoretically. The field pole of this HESM is axially divided into two parts; one is an excitation part and the other a permanent‐magnet (PM) part. A nonlinear equivalent circuit, which can include the saliency of the rotor and the magnetic saturation due to the iron core, is derived. Based on this equivalent circuit, the steady‐state performance of the HESM is calculated, and the results are confirmed through experiments. © 2004 Wiley Periodicals, Inc. Electr Eng Jpn, 150(2): 43–49, 2005; Published online in Wiley InterScience ( www.interscience.wiley.com ). DOI 10.1002/eej.20033     

Pulse-Width Dependence in Current-Driven Magnetization Reversal Using GaMnAs-Based Double-Barrier Magnetic Tunnel Junction

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.     

Suppression of adjuvant arthritis in rats by oral administration of type II collagen in combination with type I interferon

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.     

An image analyzing system using an artificial neural network forevaluating the parenchymal echo pattern of cirrhotic liver and chronichepatitis

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     

Structure and dynamics of suspensions of silica particles in liquidcrystals under a low frequency AC applied voltage

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     

Bias-stress-induced increase in parasitic resistance of InP-based InAlAs/InGaAs HEMTs

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 (R_s) to increase at large drain bias voltages. The increase in R_s 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 (R_d) does not depend on the material of the carrier supply layer, which suggests that a mechanism different from that in the case of R_s should be considered. It is also found that a deep gate recess suppresses the increase in R_d after long-term stressing.