Lamin-Related Congenital Muscular Dystrophy Alters Mechanical Signaling and Skeletal Muscle Growth

Laminopathies are a clinically heterogeneous group of disorders caused by mutations in the LMNA gene, which encodes the nuclear envelope proteins lamins A and C. The most frequent diseases associated with LMNA mutations are characterized by skeletal and cardiac involvement, and include autosomal dominant Emery–Dreifuss muscular dystrophy (EDMD), limb-girdle muscular dystrophy type 1B, and LMNA-related congenital muscular dystrophy (LMNA-CMD). Although the exact pathophysiological mechanisms responsible for LMNA-CMD are not yet understood, severe contracture and muscle atrophy suggest that mutations may impair skeletal muscle growth. Using human muscle stem cells (MuSCs) carrying LMNA-CMD mutations, we observe impaired myogenic fusion with disorganized cadherin/β catenin adhesion complexes. We show that skeletal muscle from Lmna-CMD mice is unable to hypertrophy in response to functional overload, due to defective fusion of activated MuSCs, defective protein synthesis and defective remodeling of the neuromuscular junction. Moreover, stretched myotubes and overloaded muscle fibers with LMNA-CMD mutations display aberrant mechanical regulation of the yes-associated protein (YAP). We also observe defects in MuSC activation and YAP signaling in muscle biopsies from LMNA-CMD patients. These phenotypes are not recapitulated in closely related but less severe EDMD models. In conclusion, combining studies in vitro, in vivo, and patient samples, we find that LMNA-CMD mutations interfere with mechanosignaling pathways in skeletal muscle, implicating A-type lamins in the regulation of skeletal muscle growth.     

Comparing open data benchmarks: Which metrics and methodologies determine countries’ positions in the ranking lists?

An understanding of the similar and divergent metrics and methodologies underlying open government data benchmarks can reduce the risks of the potential misinterpretation and misuse of benchmarking outcomes by policymakers, politicians, and researchers. Hence, this study aims to compare the metrics and methodologies used to measure, benchmark, and rank governments' progress in open government data initiatives. Using a critical meta-analysis approach, we compare nine benchmarks with reference to meta-data, meta-methods, and meta-theories. This study finds that both existing open government data benchmarks and academic open data progress models use a great variety of metrics and methodologies, although open data impact is not usually measured. While several benchmarks’ methods have changed over time, and variables measured have been adjusted, we did not identify a similar pattern for academic open data progress models. This study contributes to open data research in three ways: 1) it reveals the strengths and weaknesses of existing open government data benchmarks and academic open data progress models; 2) it reveals that the selected open data benchmarks employ relatively similar measures as the theoretical open data progress models; and 3) it provides an updated overview of the different approaches used to measure open government data initiatives’ progress. Finally, this study offers two practical contributions: 1) it provides the basis for combining the strengths of benchmarks to create more comprehensive approaches for measuring governments’ progress in open data initiatives; and 2) it explains why particular countries are ranked in a certain way. This information is essential for governments and researchers to identify and propose effective measures to improve their open data initiatives.     

基于QoE感知的星地一体化网络公平资源分配策略

星地一体化网络是卫星网络和地面网络互联，实现卫星网络和地面网络优势互补的一体化网络。随着无线通信的快速发展，卫星网络和地面网络的同频复用技术可有效提升资源利用效率，满足日益增长的无线数据业务需求。但由于无线数据业务类型的多样化和频谱资源的稀缺性，如何实现用户数据服务的服务体验质量（Quality of experience， QoE）和公平性提升也成为星地一体化网络亟待解决的关键问题。本文综合考虑信道时变性、服务体验多样性和服务公平性，提出了一种基于QoE感知的星地一体化网络资源分配策略。为此，将目标问题描述为基于时间平均的服务体验质量的网络公平效用最大化，并利用李雅普诺夫优化理论，将复杂的动态原始优化问题进一步转化并分解为3个瞬时优化子问题，每个子问题可以在每个时隙独立求解。同时，由于无线资源分配为典型的非凹混合组合优化问题，推导了一个低复杂度的两步算法来求解第三个资源分配子问题。仿真结果揭示了在不同的公平性参数下用户的长时QoE与公平性之间的折衷情况。     

高熔体流动速率薄壁注塑聚丙烯专用料的结构与性能分析

采用多种分析测试方法，对3类高熔体流动速率薄壁注塑聚丙烯（PP）专用料的熔融结晶性能、光学性能、分子量分布、力学性能、毛细管流变性能、热收缩性能进行了分析研究。结果表明，K1860与2种市售主流PP1、PP2各项性能相当，其中K1860分子量分布相对PP1、PP2较窄，弯曲性能、拉伸性能更优，可满足大型薄壁注塑制品的生产要求。     

Lactones in the Synthesis of Prostaglandins and Prostaglandin Analogs

In the total stereo-controlled synthesis of natural prostaglandins (PGs) and their structural analogs, a vast class of compounds and drugs, known as the lactones, are encountered in a few key steps to build the final molecule, as: δ-lactones, γ-lactones, and 1,9-, 1,11-, and 1,15-macrolactones. After the synthesis of 1,9-PGF_2α and 1,15-PGF_2α lactones, many 1,15-lactones of E₂, E₃, F₂, F₃, A₂, and A₃ were found in the marine mollusc Tethys fimbria and the quest for understanding their biological role stimulated the research on their synthesis. Then 1,9-, 1,11-, and 1,15-PG lactones of the drugs were synthesized as an alternative to the corresponding esters, and the first part of the paper describes the methods used for their synthesis. The efficient Corey procedure for the synthesis of prostaglandins uses the key δ-lactone and γ-lactone intermediates with three or four stereocenters on the cyclopentane fragment to link the PG side chains. The paper describes the most used procedures for the synthesis of the milestone δ-Corey-lactones and γ-Corey-lactones, their improvements, and some new promising methods, such as interesting, new stereo-controlled and catalyzed enantioselective reactions, and methods based on the chemical/enzymatic resolution of the compounds in different steps of the sequences. The many uses of δ-lactones not only for the synthesis of γ-lactones, but also for obtaining 9β-halogen-PGs and halogen-substituted cyclopentane intermediates, as synthons for new 9β-PG analogs and future applications, are also discussed.     

Omega-6支撑身体，Omega-3支撑大脑：均衡摄入对儿童大脑发育的影响（网络首发、推荐阅读）

人体大脑和身体的发育，需要从食物中摄取均衡的营养物质。人类大脑是区分人类和其他动物的特征。食物中的必需脂肪酸是机体组织结构和功能的必要组成部分。Omega-6（O6）亚油酸（LA6）是皮肤组织的组成成分，且是炎症、血栓形成、免疫和其他信号分子的前体；Omega-3（O3）α-亚麻酸（ALA3），特别是其长链代谢产物——二十二碳六烯酸（DHA3），是大脑、视网膜和部分神经组织中的关键组分。从富含LA6脂肪酸（缺乏O3脂肪酸）的植物籽中提取出的廉价而优质油脂，是20世纪的西方国家食品工业生产的主要脂肪来源。在代谢通路中，高浓度的LA6脂肪酸可拮抗O3脂肪酸代谢，造成O3脂肪酸不足，因此，在给怀孕动物的饲料中，只提供富含LA6但缺乏O3脂肪酸的油脂作为唯一的脂肪来源，会导致幼崽大脑发育不良。过去20~30年的研究表明，低含量LA6且含DHA3的油脂可改善大脑的功能。近年来的研究较多集中在营养因素对大脑发育的影响，最新研究数据表明，脂肪酸平衡对营养不良儿童的大脑发育尤为重要。世界卫生组织（WHO）越来越重视大脑的营养健康，通过其下属的食品法典委员会，建议用于治疗严重急性营养不良儿童的即食治疗食品中，使用含有均衡脂肪酸组成/构成的脂肪。同样，脂肪酸均衡对老年人可能也很重要。目前，业界已经有了调整油脂成分的方法，以确保脂肪酸均衡，从而维持人体整个生命周期的大脑健康。     

广西2021年早晚籼优质稻主要品种品质测报分析（网络首发、推荐阅读）

通过对广西2021年度推广种植的野香优、广粮香2号、百香、丝香四个主要品种系列的早晚籼优质稻谷品种进行采样，按照《优质稻谷》《中国好粮油 稻谷》《广西好粮油 广西香米》标准中规定质量品质指标进行检测，并依据检测数据对四个主要稻谷品种在不同季节、不同种植区域以及同一种植区域不同种植季节品质变化情况进行对比分析研究，寻找优质稻因品种、产地、种植季节不同对其品质的影响规律，为优质稻谷品种的选育和推广种植工作提供科学依据和数据支持，促进广西优质粮食产业和“广西香米”产业进一步发展。     

Profiling of knowledge organisation systems for the annotation of Linked Data cultural resources

The importance of cultural heritage for supporting the knowledge economy has promoted its digitisation and online publication. Many cultural heritage repositories have published millions of digitised items using semantic web technologies and Linked Data approaches. These repositories frequently use knowledge organisation systems to classify the resources, but the domain heterogeneity makes it difficult to know if they are the most suitable ones. This paper describes the process used to discover and profile the knowledge organisation systems used in the cultural heritage domain. Additionally, for those knowledge organisation systems with a thesaurus-like structure, a detailed quality analysis is performed. The results of this analysis can be used as a key factor for the selection of knowledge organisation systems in classification tasks.     

基于线状特性的大数据地铁用户识别与定位方法

从MR原始数据特性出发研究数据结构与字段,采用基于线状特性分析MR大数据的地铁用户识别过滤方法和位置定位方法,该方法经过验证能够有效输出地铁用户数据并定位在地图上,解决人工地铁测试效率低且测试片面的问题,实现基于大数据的自动化地铁网络评估,提供海量的有效定位数据用于地铁评估与分析。     

Maximum Data Generation Rate Routing Protocol Based on Data Flow Controlling Technology for Rechargeable Wireless Sensor Networks

For rechargeable wireless sensor networks, limited energy storage capacity, dynamic energy supply, low and dynamic duty cycles cause that it is unpractical to maintain a fixed routing path for packets delivery permanently from a source to destination in a distributed scenario. Therefore, before data delivery, a sensor has to update its waking schedule continuously and share them to its neighbors, which lead to high energy expenditure for reestablishing path links frequently and low efficiency of energy utilization for collecting packets. In this work, we propose the maximum data generation rate routing protocol based on data flow controlling technology. For a sensor, it does not share its waking schedule to its neighbors and cache any waking schedules of other sensors. Hence, the energy consumption for time synchronization, location information and waking schedule shared will be reduced significantly. The saving energy can be used for improving data collection rate. Simulation shows our scheme is efficient to improve packets generation rate in rechargeable wireless sensor networks.