Suggestions of Principles to Reduce the Deviation of Steep Slope Evaluation Results through On-Site Evaluation

Geup Kyung Sa Ji (GGSJ) is the term used to refer to steep slopes managed by the Ministry of Interior and Safety (MOIS). The MOIS is a government agency of South Korea in charge of administration and safety, with evaluations and management carried out by local governments. There are 16,071 GGSJs as of March 2022. However, it is difficult to determine whether the evaluation results for these GGSJs are reliable and whether data are managed efficiently. Unlike the Ministry of Land, Infrastructure and Transport and the Korea Forest Service, there are personnel in charge of the relevant administration and welfare operations who are not experts in GGSJs. Therefore, a method for evaluating the currently managed steep slopes and the problems that arise in the process must be identified through evaluation by experts and non-experts in the field. Additionally, methods to increase the consistency and reliability of evaluation results must be determined. This study proposes methods designed to solve the problem of differences in evaluation results caused by the diversity of perspectives on the evaluation of GGSJs and which can be used in the assessment of complex slopes. This study aims to increase the efficiency and consistency of evaluation results in the integrated management of GGSJs to build trust among management staff

A review of high impact weather for aviation meteorology

This review paper summarizes current knowledge available for aviation operations related to meteorology and provides suggestions for necessary improvements in the measurement and prediction of weather-related parameters, new physical methods for numerical weather predictions (NWP), and next-generation integrated systems. Severe weather can disrupt aviation operations on the ground or in-flight. The most important parameters related to aviation meteorology are wind and turbulence, fog visibility, aerosol/ash loading, ceiling, rain and snow amount and rates, icing, ice microphysical parameters, convection and precipitation intensity, microbursts, hail, and lightning. Measurements of these parameters are functions of sensor response times and measurement thresholds in extreme weather conditions. In addition to these, airport environments can also play an important role leading to intensification of extreme weather conditions or high impact weather events, e.g., anthropogenic ice fog. To observe meteorological parameters, new remote sensing platforms, namely wind LIDAR, sodars, radars, and geostationary satellites, and in situ instruments at the surface and in the atmosphere, as well as aircraft and Unmanned Aerial Vehicles mounted sensors, are becoming more common. At smaller time and space scales (e.g., < 1 km), meteorological forecasts from NWP models need to be continuously improved for accurate physical parameterizations. Aviation weather forecasts also need to be developed to provide detailed information that represents both deterministic and statistical approaches. In this review, we present available resources and issues for aviation meteorology and evaluate them for required improvements related to measurements, nowcasting, forecasting, and climate change, and emphasize future challenges

Gravitational Waves From Known Pulsars: Results From The Initial Detector Era

We present the results of searches for gravitational waves from a large selection of pulsars using data from the most recent science runs (S6, VSR2 and VSR4) of the initial generation of interferometric gravitational wave detectors LIGO (Laser Interferometric Gravitational-wave Observatory) and Virgo. We do not see evidence for gravitational wave emission from any of the targeted sources but produce upper limits on the emission amplitude. We highlight the results from seven young pulsars with large spin-down luminosities. We reach within a factor of five of the canonical spin-down limit for all seven of these, whilst for the Crab and Vela pulsars we further surpass their spin-down limits. We present new or updated limits for 172 other pulsars (including both young and millisecond pulsars). Now that the detectors are undergoing major upgrades, and, for completeness, we bring together all of the most up-to-date results from all pulsars searched for during the operations of the first-generation LIGO, Virgo and GEO600 detectors. This gives a total of 195 pulsars including the most recent results described in this paper.United States National Science FoundationScience and Technology Facilities Council of the United KingdomMax-Planck-SocietyState of Niedersachsen/GermanyAustralian Research CouncilInternational Science Linkages program of the Commonwealth of AustraliaCouncil of Scientific and Industrial Research of IndiaIstituto Nazionale di Fisica Nucleare of ItalySpanish Ministerio de Economia y CompetitividadConselleria d'Economia Hisenda i Innovacio of the Govern de les Illes BalearsNetherlands Organisation for Scientific ResearchPolish Ministry of Science and Higher EducationFOCUS Programme of Foundation for Polish ScienceRoyal SocietyScottish Funding CouncilScottish Universities Physics AllianceNational Aeronautics and Space AdministrationOTKA of HungaryLyon Institute of Origins (LIO)National Research Foundation of KoreaIndustry CanadaProvince of Ontario through the Ministry of Economic Development and InnovationNational Science and Engineering Research Council CanadaCarnegie TrustLeverhulme TrustDavid and Lucile Packard FoundationResearch CorporationAlfred P. Sloan FoundationAstronom

Cross-sectional analysis of a large cohort with X-linked Charcot-Marie-Tooth disease (CMTX1).

OBJECTIVE: To extend the phenotypic description of Charcot-Marie-Tooth disease (CMTX1) and to draw new genotype-phenotype relationships. METHODS: Mutations in GJB1 cause the main X-linked form of CMTX (CMTX1). We report cross-sectional data from 160 patients (from 120 different families, with 89 different mutations) seen at the Inherited Neuropathies Consortium centers. RESULTS: We evaluated 87 males who had a mean age of 41 years (range 10-78 years) and 73 females who had a mean age of 46 years (range 15-84 years). Sensory-motor polyneuropathy affects both sexes, more severely in males than in females, and there was a strong correlation between age and disease burden in males but not in females. Compared with females, males had more severe reduction in motor and sensory neurophysiology parameters. In contrast to females, the radial nerve sensory response in older males tended to be more severely affected compared with younger males. Median and ulnar nerve motor amplitudes were also more severely affected in older males, whereas ulnar nerve motor potentials tended to be more affected in older females. Conversely, there were no statistical differences between the sexes in other features of the disease, such as problems with balance and hand dexterity. CONCLUSIONS: In the absence of a phenotypic correlation with specific GJB1 mutations, sex-specific distinctions and clinically relevant attributes need to be incorporated into the measurements for clinical trials in people with CMTX1. CLINICALTRIALSGOV IDENTIFIER: NCT01193075

Dissecting Molecular Differences between Wnt Coreceptors LRP5 and LRP6

Low-density lipoprotein receptor-related proteins 5 and 6 (LRP5 and LRP6) serve as Wnt co-receptors for the canonical β-catenin pathway. While LRP6 is essential for embryogenesis, both LRP5 and LRP6 play critical roles for skeletal remodeling, osteoporosis pathogenesis and cancer formation, making LRP5 and LRP6 key therapeutic targets for cancer and disease treatment. LRP5 and LRP6 each contain in the cytoplasmic domain five conserved PPPSPxS motifs that are pivotal for signaling and serve collectively as phosphorylation-dependent docking sites for the scaffolding protein Axin. However existing data suggest that LRP6 is more effective than LRP5 in transducing the Wnt signal. To understand the molecular basis that accounts for the different signaling activity of LRP5 and LRP6, we generated a series of chimeric receptors via swapping LRP5 and LRP6 cytoplasmic domains, LRP5C and LRP6C, and studied their Wnt signaling activity using biochemical and functional assays. We demonstrate that LRP6C exhibits strong signaling activity while LRP5C is much less active in cells. Recombinant LRP5C and LRP6C upon in vitro phosphorylation exhibit similar Axin-binding capability, suggesting that LRP5 and LRP6 differ in vivo at a step prior to Axin-binding, likely at receiving phosphorylation. We identified between the two most carboxyl PPPSPxS motifs an intervening “gap4” region that appears to account for much of the difference between LRP5C and LRP6C, and showed that alterations in this region are sufficient to enhance LRP5 PPPSPxS phosphorylation and signaling to levels comparable to LRP6 in cells. In addition we provide evidence that binding of phosphorylated LRP5 or LRP6 to Axin is likely direct and does not require the GSK3 kinase as a bridging intermediate as has been proposed. Our studies therefore uncover a new and important molecular tuning mechanism for differential regulation of LRP5 and LRP6 phosphorylation and signaling activity

6G Network AI Architecture for Everyone-Centric Customized Services

Mobile communication standards were developed for enhancing transmission and network performance by using more radio resources and improving spectrum and energy efficiency. How to effectively address diverse user requirements and guarantee everyone's Quality of Experience (QoE) remains an open problem. The Sixth Generation (6G) mobile systems will solve this problem by utilizing heterogenous network resources and pervasive intelligence to support everyone-centric customized services anywhere and anytime. In this article, we first coin the concept of Service Requirement Zone (SRZ) on the user side to characterize and visualize the integrated service requirements and preferences of specific tasks of individual users. On the system side, we further introduce the concept of User Satisfaction Ratio (USR) to evaluate the system's overall service ability of satisfying a variety of tasks with different SRZs. Then, we propose a network Artificial Intelligence (AI) architecture with integrated network resources and pervasive AI capabilities for supporting customized services with guaranteed QoEs. Finally, extensive simulations show that the proposed network AI architecture can consistently offer a higher USR performance than the cloud AI and edge AI architectures with respect to different task scheduling algorithms, random service requirements, and dynamic network conditions

Microphysical characteristics of frozen droplet aggregates from deep convective clouds

During the 2012 Deep Convective Clouds and Chemistry (DC3) experiment the National Science Foundation/National Center for Atmospheric Research Gulfstream V (GV) aircraft sampled the upper anvils of two storms that developed in eastern Colorado on 6 June 2012. A cloud particle imager (CPI) mounted on the GV aircraft recorded images of ice crystals at altitudes of 12.0 to 12.4&thinsp;km and temperatures (T) from −61 to −55&thinsp;∘C. A total of 22&thinsp;393 CPI crystal images were analyzed, all with maximum dimension (Dmax⁡)&lt;433&thinsp;µm and with an average Dmax⁡ of 80.7±45.4&thinsp;µm. The occurrence of well-defined pristine crystals (e.g., columns and plates) was less than 0.04&thinsp;% by number. Single frozen droplets and frozen droplet aggregates (FDAs) were the dominant habits with fractions of 73.0&thinsp;% (by number) and 46.3&thinsp;% (by projected area), respectively. The relative frequency of occurrence of single frozen droplets and FDAs depended on temperature and position within the anvil cloud. A new algorithm that uses the circle Hough transform technique was developed to automatically identify the number, size, and relative position of element frozen droplets within FDAs. Of the FDAs, 42.0&thinsp;% had two element frozen droplets with an average of 4.7±5.0 element frozen droplets. The frequency of occurrence gradually decreased with the number of element frozen droplets. Based on the number, size, and relative position of the element frozen droplets within the FDAs, possible three-dimensional (3-D) realizations of FDAs were generated and characterized by two different shape parameters, the aggregation index (AI) and the fractal dimension (Df), that describe 3-D shapes and link to scattering properties with an assumption of spherical shape of element frozen droplets. The AI of FDAs decreased with an increase in the number of element frozen droplets, with larger FDAs with more element frozen droplets having more compact shapes. The Df of FDAs was about 1.20–1.43 smaller than that of black carbon (BC) aggregates (1.53–1.85) determined in previous studies. Such a smaller Df of FDAs indicates that FDAs have more linear chain-like branched shapes than the compact shapes of BC aggregates. Determined morphological characteristics of FDAs along with the proposed reconstructed 3-D representations of FDAs in this study have important implications for improving the calculations of the microphysical (e.g., fall velocity) and radiative (e.g., asymmetry parameter) properties of ice crystals in upper anvil clouds.</p

Genetic analysis and natural history of Charcot-Marie-Tooth disease CMTX1 due to GJB1 variants

Charcot-Marie-Tooth disease (CMT) due to GJB1 variants (CMTX1) is the second most common form of CMT. It is an X-linked disorder characterised by progressive sensory and motor neuropathy with males affected more severely than females. Many reported GJB1 variants remain classified as variants of uncertain significance (VUS). In this large, international, multicentre study we prospectively collected demographic, clinical and genetic data on patients with CMT associated with GJB1 variants. Pathogenicity for each variant was defined using adapted American College of Medical Genetics criteria. Baseline and longitudinal analyses were conducted to study genotype-phenotype correlations, to calculate longitudinal change using the CMT Examination Score (CMTES), to compare males versus females, and pathogenic/likely pathogenic (P/LP) variants versus VUS. We present 387 patients from 295 families harbouring 154 variants in GJB1. Of these, 319 patients (82.4%) were deemed to have P/LP variants, 65 had VUS (16.8%) and 3 benign variants (0.8%; excluded from analysis); an increased proportion of patients with P/LP variants compared with using ClinVar's classification (74.6%). Male patients (166/319, 52.0%, P/LP only) were more severely affected at baseline. Baseline measures in patients with P/LP variants and VUS showed no significant differences, and regression analysis suggested the disease groups were near identical at baseline. Genotype-phenotype analysis suggested c.-17G>A produces the most severe phenotype of the five most common variants, and missense variants in the intracellular domain are less severe than other domains. Progression of disease was seen with increasing CMTES over time up to 8 years follow-up. Standard response mean (SRM), a measure of outcome responsiveness, peaked at 3 years with moderate responsiveness (change in CMTES (ΔCMTES) = 1.3 ± 2.6, p = 0.00016, SRM = 0.50). Males and females progressed similarly up to 8 years, but baseline regression analysis suggested that over a longer period, females progress more slowly. Progression was most pronounced for mild phenotypes (CMTES = 0-7; 3-year ΔCMTES = 2.3 ± 2.5, p = 0.001, SRM = 0.90). Enhanced variant interpretation has yielded an increased proportion of GJB1 variants classified as P/LP and will aid future variant interpretation in this gene. Baseline and longitudinal analysis of this large cohort of CMTX1 patients describes the natural history of the disease including the rate of progression; CMTES showed moderate responsiveness for the whole group at 3 years and higher responsiveness for the mild group at 3, 4 and 5 years. These results have implications for patient selection for upcoming clinical trials