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.     

Hydrogen production through dry reforming of biogas using a porous electrochemical cell: Effects of a cobalt catalyst in the electrode and mixing of air with biogas

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.     

Wideband and compact folded loop antenna

A simple and successful design method that yields a wideband and compact antenna without a ground plane is proposed. The antenna, referred to as the folded loop antenna, can, with the right parameters, achieve wideband characteristics. Calculated and measured results agree well and more than 50% bandwidth (return loss /spl les/-10 dB) is obtained.     

A 322 MHz random-cycle embedded DRAM with high-accuracy sensing and tuning

A 16 Mb embedded DRAM macro in a fully CMOS logic compatible 90 nm process with a low noise core architecture and a high-accuracy post-fabrication tuning scheme has been developed. Based on the proposed techniques, 61% improvement of the sensing accuracy is realized. Even with the smallest 5 fF/cell capacitance, a 322 MHz random-cycle access while 32 ms data retention time which contributes to save the data retention power down to 60 /spl mu/W are achieved.     

The As-prepared surfaces of C-axis-oriented Nd1Ba2Cu3Oy thin films characterized using scanning probe microscopes

Recently, superconducting Nd₁Ba₂Cu₃O_y (Ndl23) thin films with high superconducting transition temperature (T _c) have been successfully fabricated at our institute employing the standard laser ablation method. In this paper, we report the results of surface characterization of the Nd123 thin films using an ultrahigh vacuum scanning tunneling microscope/spectroscopy (UHV-STM/STS) and an atomic force microscope (AFM) system operated in air. Clear spiral pattern is observed on the surfaces of Nd123 thin films by STM and AFM, suggesting that films are formed by two-dimensional island growth mode. Contour plots of the spirals show that the step heights of the spirals are not always the integer or half-integer number of thec-axis parameter of the structure. This implies that the surface natural termination layer of the films may not be unique. This result is supported byI-V STS measurements. The surface morphology of the Nd123 thin films is compared with that of thec-axis-oriented Y₁Ba₂Cu₃S_y thin films. Surface atomic images of the as-prepared Nd123 thin films are obtained employing both STM and AFM. STS measurements show that most of the surfaces are semiconductive. The results of STS measurements together with the fact that we are able to see the surface atomic images using scanning probe microscopes suggest that exposure to air does not cause serious degradation to the as-prepared surfaces of Nd123 thin films.     

Heat Capacity of 3He Solid Film on Graphite in Magnetic Fields

Heat capacities of a submonolayer ³He solid film adsorbed on a graphite surface are measured in magnetic fields up to 400 Oe. The measured heat capacity shifts to higher temperatures with an increasing magnetic field. The amplitudes of the shifts are twenty times larger than the magnitude of the Zeeman energy, which is anomalously large. With regard to the origin of these large shifts, the reduction of frustrations and the weakening of the competition between multiple spin exchange interactions are discussed.     

Fabrication of GaAs quantum wire structure using metal organic molecular beam epitaxy

GaAs quantum wires (100*20 nm/sup 2/) buried in AlAs layers have been successfully fabricated using metal organic molecular beam epitaxy (MOMBE) for the first time. The underlying growth mechanism is that, under appropriate As/sub 4/ pressure in MOMBE, GaAs preferentially grows only on the sidewalls of the patterned     

Growth condition of iodine-doped n+-CdTe layers in metal-organic vapor phase epitaxy

Iodine doping of CdTe layers grown on (100) GaAs by metal-organic vapor phase epitaxy (MOVPE) was studied using diethyltelluride (DETe) and diisopropyltelluride (DiPTe) as tellurium precursors and ethyliodine (EI) as a dopant. Electron densities of doped layers increased gradually with decreasing the growth temperature from 425°C to 325°C. Doped layers grown with DETe had higher electron densities than those grown with DiPTe. When the hot-wall temperature was increased from 200°C to 250°C at the growth temperature of 325°C, doped layers grown with DETe showed an increase of the electron density from 3.7×10¹⁶ cm⁻³ to 2.6×10¹⁸ cm⁻³. On the other hand, such an increase of the electron density was not observed for layers grown with DiPTe. The mechanisms for different doping properties for DETe and DiPTe were studied on the basis of the growth characteristics for these precursors. Higher thermal stability of DETe than that of DiPTe was considered to cause the difference of doping properties. With increasing the hot-wall temperature from 200°C to 250°C, the effective ratio of Cd to Te species on the growth surface became larger for layers grown with DETe than those grown with DiPTe. This was considered to decrease the compensation of doped iodine and to increase the electron density of layers grown with DETe. The effective ratio of Cd to Te species on the growth surface also increased with decreasing growth temperature. This was considered to increase the electron density with decreasing growth temperature.     

Wavelength-selective fused fiber couplers utilizing fielddifference between core and cladding modes

It is shown that wavelength-selective fused taper couplers can be made by using the large difference in field distribution, between core and cladding modes. It is evident from the calculation results that fibers with a high-index difference and a thin outside diameter are necessary to fabricate the couplers. Fused taper couplers are made of thin fibers with a cladding diameter of 66.0 μm and an index difference of 0.84%. The splitting ratio of the coupler obtained is more than 20 dB in the 1.53-μm region and less than -20 dB in the 0.8 to 1.0-μm region. The loss is less than 0.2 dB and 0.4 dB in the 1.53-μm and the 0.8 to 1.0-μm regions, respectively     

Preparation of porous carbons from thermoplastic precursors and their performance for electric double layer capacitors

Porous carbons with high surface area were successfully prepared from thermoplastic precursors, such as poly(vinyl alcohol) (PVA), hydroxyl propyl cellulose and poly(ethylene terephthalate), by the carbonization of a mixture with MgO at 900 °C in an inert atmosphere. After carbonization the MgO was dissolved out using a diluted sulfuric acid and the carbons formed were isolated. The mixing of the PVA carbon precursor with the MgO precursors (reagent grade MgO, magnesium acetate or citrate) was done either in powder form or in an aqueous solution. The BET surface area of the carbons obtained via solution mixing could reach a very high value, such as 2000 m²/g, without any activation process. The pore structure of the resultant carbons was found to depend strongly on the mixing method; the carbons prepared via solution mixing were rich in mesopores, but those produced via powder mixing were rich in micropores. The size of mesopores was found to be almost the same as that of the MgO particles, suggesting a way of controlling the mesopore size in the resultant carbons. Measurement of capacitance was carried out in 1 mol/L H₂SO₄ electrolyte. The porous carbon with a BET surface area of 1900 m²/g prepared at 900 °C through solution mixing of Mg acetate with PVA showed a fairly high EDLC capacitance, about 250 F/g with a current density of 20 mA/g and 210 F/g with 1000 mA/g. The rate performance was closely related to the mesoporous surface area.