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.     

Stabilization of SnO2 ultrafine particles by additives

In order to stabilize ultrafine particles of SnO₂ which is essential to obtain high gas sensitivity, a systematic investigation was undertaken regarding the stabilizing effects of 5 at% impregnated foreign additives, consisting of oxides or polyoxy compounds of 31 metals and 3 non-metals. The data of specific surface area, SA, as well as SnO₂ crystallite size, D, evaluated from X-ray diffraction showed that the additives could be classified into several groups according to the effectiveness. The most effective group, consisting of P-Ba, Sm, Ba, P, Mo, W, Ca, Sr, Cr and In, could keepD less than 10 nm even after calcination at 900°C, whereas pure SnO₂ underwent grain growth to haveD of 13 and 27 nm at 600 and 900°C, respectively. Electron microscopy revealed that neck sizes, X, between crystallites were fairly proportional toD(X/D = 0.80). A simple analysis of SA and D data based on a monosized sphere model suggested that each crystallite was coordinated with 3–4.5 neighbours through the necks. The existing state and stabilizing mechanism of additives are discussed in conjunction with the electrical resistance of porously sintered elements.     

A CMOS 1/spl times/-16/spl times/ speed DVD write channel IC

Optical recording demands a meticulous write strategy to control the laser beam power and regulate the phase change layer temperature tightly. The width, height, and delay of a string of short pulses applied to the laser diode need to be adjusted in fine steps, and the writing speed varies widely per applications. A multi-phase phase-locked loop (PLL) tracks a wide range of clock frequencies, and provides a low-jitter time base for write pulses. With two enabling circuit concepts, PLL loop filter voltage folding/unfolding and switch-in of parallel MOS resistors in delay cells, it is possible to operate a PLL to cover a frequency range spanning over three octaves with one VCO. A 10-stage differential VCO is phase-locked to the input channel clock ranging from 26 to 420 MHz (1/spl times/-16/spl times/ DVD speed), and its 20-phase outputs are used to generate write pulses. The pulsewidth and delay are programmed with 120 /spl plusmn/ 40 ps time resolution. The prototype chip fabricated in 0.35 /spl mu/m CMOS occupies 3.5/spl times/3.3 mm/sup 2/, and consumes 294 mW at 3.3 V.     

Development and field test of the articulated mobile robot T2 Snake-4 for plant disaster prevention

Abstract

In this work, we develop an articulated mobile robot that can move in narrow spaces, climb stairs, gather information, and operate valves for plant disaster prevention. The robot can adopt a tall position using a folding arm and gather information using sensors mounted on the arm. In addition, this paper presents a stair climbing method using a single backward wave. This method enables the robot to climb stairs that have a short tread. The developed robot system is tested in a field test at the World Robot Summit 2018, and the lessons learned in the field test are discussed.     

Receptor function of small semiconductor crystals with clean and electron-traps dispersed surfaces

Theoretical exploration has been conducted to formulate the receptor function and response of a semiconductor gas sensor using thin plate-like crystals. The receptor function can be formulated over a wide range of depletion from conventional one (regional depletion) to new type one (volume depletion) by focusing attention on how the surface density of adsorbed oxygen ions (O⁻) is related with chemical moiety outside the crystals and physical moiety inside. The responses to gases thus derived are inversely proportional to the thickness of the plates used in the range of volume depletion. Electron-traps, if dispersed on the crystals, are shown to exert drastic effects in promoting sensitivity to gases.     

Calibration of multiple kinects with little overlap regions

When using multiple Kinects, there must be enough distances among neighboring Kinects to avoid spoiled range data caused by the interference of their infrared speckle patterns. In the arrangement, their overlapped regions are too small to apply existing calibration methods using correspondences between their observations straightforwardly. Therefore, we propose a method to calibrate Kinects without large overlapped regions. In our method, first, we add extra RGB cameras in an environment to compensate overlapped regions. Thanks to them, we can estimate their camera parameters by obtaining correspondences between color images. Next, for accurate calibration, which considers range data as well as color images of Kinects, we optimize the estimated parameters by minimizing both the errors of correspondences between color images and those of range data of planar regions, which exist in a general environment such as walls and floors. Although our method consists of conventional techniques, its combination is optimized to achieve the calibration. © 2015 Institute of Electrical Engineers of Japan. Published by John Wiley & Sons, Inc.     

Small-angle X-ray scattering studies on melting and recrystallization behaviors of poly(oxyethylene) crystallites in poly(d,l-lactide)/poly(oxyethylene) blends

Direct determination of the discrete distribution for crystalline lamellar thickness has been performed for poly(d,l-lactic acid)/poly(oxyethylene) (PDLLA/PEG) blends by conducting small-angle X-ray scattering (SAXS) measurements using synchrotron radiation. The PDLLA used was an random (racemic) copolymer of bio-based poly(l-lactic acid) (PLLA) and poly(d-lactic acid) (PDLA) with the lactide monomer ratio of l:d = 50:50. It is known that PLA is miscible with PEG in the amorphous state. In the current paper, we report comprehensive results on structural analyses of PDLLA/PEG blends in the course of heating and cooling process using SAXS to elucidate the change in the thickness distribution of the lamellae. As a consequence, it was found that the distribution of the lamellar thickness moves toward the larger value (in other words, lamellar thickening) as temperature approaches the melting point. Typically, the thickness distribution was dispersed in the range of 10–20 nm at room temperature and it changed toward 40 nm in the vicinity of the melting temperature. To the best of our knowledge, this is the first report of direct determination of the discrete distribution for the crystalline lamellar thickness and their in-situ changes in the course of the lamellar thickening process. As a result, the lamellar thickening was found to occur at much lower temperature for the blend samples with 10% and 20% of PDLLA contents as compared to the PEG 100% sample. This phenomenon can be ascribed to the melting point depression owing to the miscibility between PEG and PDLLA. Thereby, thinner lamellae were melted and thicker ones appeared at much lower temperature for the blends than for the PEG 100% sample. As for the average repeating distance (long period) of the lamellar stacks, an abrupt increase similar to the critical divergence was observed (from 25 nm to 50 nm) in the heating process. Not only for the melting behavior but also in the course of recrystallization, change in the lamellar-thickness distribution was uncovered, which shows strong hysteresis depending on what temperature the sample was cooled down from.     

In vitro formation and thermal transition of novel hybrid fibrils from type I fish scale collagen and type I porcine collagen

Novel type I collagen hybrid fibrils were fabricated by neutralizing a mixture of type I fish scale collagen solution and type I porcine collagen solution with a phosphate buffer saline at 28 °C. Their structure was discussed in terms of the volume ratio of fish/porcine collagen solution. Scanning electron and atomic force micrographs showed that the diameter of collagen fibrils derived from the collagen mixture was larger than those derived from each collagen, and all resultant fibrils exhibited a typical D-periodic unit of ∼67 nm, irrespective of volume ratio of both collagens. Differential scanning calorimetry revealed only one endothermic peak for the fibrils derived from collagen mixture or from each collagen solution, indicating that the resultant collagen fibrils were hybrids of type I fish scale collagen and type I porcine collagen.     

Contribution of electron tunneling transport in semiconductor gas sensor

It was found that thin film devices derived from SnO₂ sols by spin-coating method showed unique thermal behavior of electric resistance in air involving a temperature region where resistance was independent of temperature. The temperature independent resistance region extended up to 400 °C, replacing a region of temperature-conventionally dependent resistance, as film thickness increased. Such unique behavior of resistance was observed also for a brush-coated device but not for screen-coated thick film devices or disk-type device, suggesting that the absence of mechanical forces applied during device fabrication favored the occurrence of the unique behavior. It was shown that the unique behavior could be well accounted for by postulating a combination of electron tunneling transport and conventional migration transport. Calculation of tunneling probability based on a simple model allowed estimating that electron tunneling transport can take place between oxide grains with a probability of 0.01 or larger if a gap in between is narrower than 0.01 nm.