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.     

Advanced Surface of Fibrous Activated Carbon Immobilized with FeO/TiO2 for Photocatalytic Evolution of Hydrogen under Visible Light

FeO-doped TiO₂ nanoparticle photocatalysts were immobilized onto the surface of fibrous activated carbon (ACF) via a sol-gel process. As an adsorbent and photocatalyst, FeO-TiO₂ on immobilized ACFs (FeO-TiO₂/ACF) greatly improved the photocatalysis rate of hydrogen production as compared with pure TiO₂ and ACF-TiO₂ under UV irradiation and visible light. The addition of ACFs surface significantly reduced the photogenerated pairs of electrons-hole recombination, thereby promoting the photocatalysis action of doped photo-metal oxides of FeO-TiO₂. Co-doping of FeO onto the lattice of the TiO₂ approach can improve the absorption activity of visible light through photo-metal oxide of TiO₂ and further enhance hydrogen production under visible light. The photocatalytic fabrics (FeO-TiO₂/ACF) were effortlessly split out from the experimental solution for re-utilization and exhibited high stability even after five complete regeneration cycles.     

智能动态网络资源分配系统

通过对解决GSM移动通信网络热点区域话务拥塞问题的研究，提出了利用可控功分器实现智能动态网络资源的分配，解决不同种类的话务拥塞问题，并着重讨论了该系统目前的成功运用及前景。     

High-gain hybrid amplifier consisting of cascaded fluoride-based TDFA and silica-based EDFA in 1458-1540 nm wavelength region

A hybrid optical fibre amplifier is described that consists of a fluoride-based thulium-doped fibre amplifier and a silica-based erbium-doped fibre amplifier connected in a cascade. The amplifier has a gain of more than 25 dB and a noise figure of less than 9 dB over a wide wavelength region of 1458-1540 nm.     

Surgical treatment for primary cardiac malignant lymphoma

A 60-year-old woman who complained of palpitation was diagnosed as ventricular tachycardia on electrocardiography and admitted to our hospital. The ultrasonic cardiography showed cardiac tumor in right ventricle and right atrium. Due to the obstruction of the right ventricle inflow by the tumor, we immediately performed resection of cardiac tumor and repair of right ventricle wall under cardiopulmonary bypass. The tumor was diagnosed as malignant lymphoma by pathological examination for surgical specimen of tumor. After operation her general condition was good, but residual cardiac lymphoma developed large size. We performed radiation therapy for cardiac lymphoma. Therefore the lymphoma was reduced to minimum size. Six months after operation metastatic malignant lymphoma appeared at whole body. So we performed chemotherapy for reduction of systemic malignant lymphoma. At first the chemotherapy was very effective. But metastasis spread rapidly and effectiveness of chemotherapy reduced. Thirteen months after operation she died for respiratory distress, probably due to metastatic brain tumor.     

SQL的现状及其研究与发展

一、SQL简介 如果说七十年代关系数据库还处于研制实验阶段,那么到了八十年代大量关系数据库商用产品的面市几乎占据了整个数据库市场,其中的原因在于它们使用了统一的语言界面,那就是,SQL成为了关系数据库的通用语言。 1974年．IBM公司San Jose研究实验室的D．D．Chamberlin在开发SEQUEL-XRM原型时提出了SE-QUEL(Structured English Query Language),1976年又修改成为SEQUEL／2．也就是目前的SQL(StructuredQuer Language)。SEQUEL／2主要成形于IBM的Sys-tem R原型中[2],并在原有基础上大加扩展,其后逐渐完善并最终成为一种完备的结构化数据库查询语言。     

Ionic transport in amorphous anodic titania stabilised by incorporation of silicon species

Incorporation of silicon species from an alloy substrate into anodic titania is shown to stabilise the structure of the film, facilitating investigation of the ionic transport processes in amorphous titania grown at high efficiency. Thus, an amorphous anodic film developed on a sputtering-deposited Ti-6 at.%Si alloy formed to 100 V in phosphoric acid electrolyte in contrast to a partially crystalline film developed on relatively pure titanium at <20 V. Silicon species, which are immobile and act as marker species in the growing film, are present in the inner 58% of the film thickness. Evidently, the film material forms simultaneously at the film/electrolyte and alloy/film interfaces by co-operative transport of cations and anions, as is usual in amorphous anodic oxides. The phosphate anions incorporated from the electrolyte migrate inward at 0.34 times the rate of O²⁻ ions and hence are present in the outer 62% of the film thickness.