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.     

Preparation of soluble TiCl3 catalysts by reduction of TiCl4 with Grignard reagents and their use for copolymerization of ethylene with propylene

Summary Preparations of soluble TiCl₃ catalysts by reduction of TiCl₄ with some types of Grignard reagents were carried out in halogenated hydrocarbon solvents by using appropriate ethers as donor. The soluble TiCl₃·MgX₂·ether complex catalysts and triisobutylaluminum as co-catalyst showed high activities for the copolymerization of ethylene with propylene. It was first found that the soluble TiCl₃·MgX₂·ether complex catalysts enhance the activities for the copolymerizations in the same manner as solid titanium catalysts supported on MgCl₂ which show high activities for homopolymerizations of olefin monomers. The copolymers obtained possessed low crystallinities. Also, the copolymers seem to contain microblock sequences and have outstandingly high tensile strength and elongation at break compared to copolymers by the conventional VOCl₃/Al(Et)_1.5Cl_1.5 catalyst system.     

Structural characterization of 1,2-polybutadiene by 13C-n.m.r. spectroscopy: 2. Sequence distribution of 1,2-unit in hydrogenated polybutadienes

The configurational sequences of 1,2-unit in a series of hydrogenated syndiotactic 1,2-polybutadienes were determined in relation to the sequence distribution of 1,2- and 1,4-units on the basis of the ¹³C-n.m.r. signal assignment of the methyl carbons. The observed triad distributions of 1,2- and 1,4-units and configurational distributions of the 1,2-unit are in good agreement with the calculated distributions from transition probabilities. The number average sequence lengths of the 1,2-unit and the 1,2-unit in the racemic addition are 5.7 to 8.0 and 4.1 to 5.4, respectively. From the extrapolation of the plot of the number average sequence length against the crystallinity of the starting 1,2-polybutadienes, the minimum sequence length for crystallization is estimated to be 3.7 for the 1,2-unit in the racemic addition.     

Design and implementation of a vehicle interface protocol using an IEEE 1394 network

A wide variety of in-vehicle devices such as camera sensors, navigation systems, telematics and communication equipments have been incorporated into a vehicle to realize Intelligent Transport Systems (ITS) applications. Because an efficient standardized network is required, ITS Data Bus (IDB) has been discussed to carry high-speed multimedia data for audio, video and other real-time ITS applications. For connecting devices in a standardized manner, the IDB network has architecture with a gateway called vehicle interface which is located between automaker’s proprietary network and the standardized IDB network. IEEE 1394 (also known as iLink or FireWire), which can transport multimedia data for consumer electronics, is a good candidate for IDB network. In this paper, we analyze the issues for existing AV/C protocol (application layer protocol over IEEE 1394) to comprise the IDB network. In addition, we designed and implemented the vehicle interface protocol as a higher layer of IEEE 1394 to address the AV/C protocol issues for realizing the whole IDB network architecture.     

Efflorescence control in geopolymer binders based on natural pozzolan

This paper addresses methods to reduce efflorescence in a geopolymer binder based on a pumice-type natural pozzolanic material from Taftan, Iran. Geopolymer pastes samples are analyzed for compressive strength and efflorescence formation after curing at 95% humidity for 28 days. To reduce the extent of efflorescence, Al-rich mineral admixtures such as metakaolin, ground granulated blast-furnace slag, and three types of calcium aluminate cements are incorporated into the dry binder at a range of concentrations. Hydrothermal curing at elevated temperatures also shows a positive effect in efflorescence reduction. Calcium aluminate cements show the greatest effect in efflorescence reduction, which is attributed to their dissolution in alkaline media releasing high amounts of alumina into the aluminosilicate geopolymer gel. These results confirm that it is possible to develop a more reliable geopolymer binder with improved properties either by adding a suitable amount of active alumina to precursors such as natural pozzolan, or by manipulating the curing conditions to enhance alumina release from less-reactive precursor phases.     

Synthesis of polyamide-hydroxyapatite nanocomposites

Simultaneous one-pot syntheses of PA66 and HAp were carried out by extracting H₂O and CO₂ from PA66 monomers and HAp raw materials, respectively, resulting in the formation of a polyamide (PA) 66-hydroxyapatite (HAp) nanocomposite. During the process, a spherical nano-sized HAp particle was precipitated following dissolution of micro-sized CaHPO₄・2H₂O. The PA66 monomers were subsequently adsorbed onto the generated HAp product. Some of the adsorbed PA66 monomers formed a bound polymer on HAp, and an increase in the adhesiveness of the PA66-HAp interface was observed as the polymerization progressed. During this process, the synthesis of a nanocomposite from a micro-sized raw material and creation of an autonomous strong interface between the matrix and filler was achieved. In addition, the shape of the resultant HAp was controllable and could be modified to needle shape by the addition of F⁻ and Mg²⁺ ions to the raw material. HAp could also be changed to plate shape via octa-calcium phosphate (OCP). Notably, during the synthesis, the filler shape of the nanocomposite could be controlled to 0D (particle), 1D (needle), and 2D (plate).     

Steam Reforming of Methanol on Copper Catalysts Supported on Large-Surface-Area ZnAl2O3

Steam reforming of methanol on various supported Cu catalysts was examined. Supports strongly affected catalyst activity and, among the catalysts tested, Cu catalyst supported on large-surface-area ZnAl₂O₄ showed the highest activity, which, to the best of our knowledge, was higher than those for the supported catalysts reported so far. For supported Cu catalysts, two species were observed. One was a dispersed Cu species having strong interaction between Cu and support, and the other was an isolated Cu species. The activity of the former species strongly depended on supports.     

Next-generation PON-part III: system specifications for XP-PON

With the knowledge that XG-PON is of primary interest to service providers as their preferred next generation optical access system, the investigation next turns to determining its technical specifications. This article presents the current thinking on the XG-PON systems. It presents a rough outline of the system design for the wavelength plan, the power budget, the protocol, and the management and service model. This work is likely to be the basis for standardization in upcoming ITU-T recommendations.