Hereditary spastic paraplegia is a not common inherited neurological disorder with heterogeneous clinical expressions. ALDH18A1 (located on 10q24.1) gene-related spastic paraplegias (SPG9A and SPG9B) are rare metabolic disorders caused by dominant and recessive mutations that have been found recently. Autosomal recessive hereditary spastic paraplegia is a common and clinical type of familial spastic paraplegia linked to the SPG11 locus (locates on 15q21.1). There are different symptoms of spastic paraplegia, such as muscle atrophy, moderate mental retardation, short stature, balance problem, and lower limb weakness. Our first proband involves a 45 years old man and our second proband involves a 20 years old woman both are affected by spastic paraplegia disease. Genomic DNA was extracted from the peripheral blood of the patients, their parents, and their siblings using a filter-based methodology and quantified and used for molecular analysis and sequencing. Sequencing libraries were generated using Agilent SureSelect Human All ExonV7 kit, and the qualified libraries are fed into NovaSeq 6000 Illumina sequencers. Sanger sequencing was performed by an ABI prism 3730 sequencer. Here, for the first time, we report two cases, the first one which contains likely pathogenic NM_002860: c.475C>T: p.R159X mutation of the ALDH18A1 and the second one has likely pathogenic NM_001160227.2: c.5454dupA: p.Glu1819Argfs Ter11 mutation of the SPG11 gene and also was identified by the whole-exome sequencing and confirmed by Sanger sequencing. Our aim with this study was to confirm that these two novel variants are direct causes of spastic paraplegia.
In this study, we collect water control valves that have had accidents due to existing cracks, etc. are collected, and propose investigation items for strengthening the valve structural safety evaluation through a series of analyzes from valve specifications to physicochemical properties are proposed. The results of this study are as follows. First, there was a large variation in the thickness of the body or flange of the valves to be investigated, which is considered to be very important factor, because it may affect the safety of the valve body against internal pressure and the flange connected with the bolt nut. Second, 60% of the valves under investigation had many voids in the valve body and flange, etc. and the decrease in thickness due to corrosion was relatively large on the inner surface in contact with water rather than the outer surface. It is judged that the investigation of depth included voids is very important factor. Third, all valves to be investigated are made of gray cast iron foam, and therefore it is judged that there is no major problem in chemical composition. It is judged that the chemical composition should be investigated. Fourth, as a physical investigation item, the analysis of metal morphology structure seems to be a very important factor for nodular cast iron from rather than a gray cast iron foam water valve with a flake structure. As it was found to be 46.7~68.8% of the standard recommended by KS, it could have a direct effect on damage such as cracks, and therefore it is judged that the evaluation of tensile strength is very important in evaluating the safety of the valve.
International Journal of Computer Science & Network Security
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v.22
no.10
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pp.214-222
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2022
In these proceedings, we will address the problem of air pollution on the premises of the Cement factory SHARRCEM L.L.C. in Hani Elezit in the Republic of Kosovo respectively around the clinker cooler, rotary kiln, and raw material mill. By air pollution, we mean the introduction of chemicals, particles, or other harmful materials into the atmosphere which in one way or another causing damage to the development of plants and organisms. Air pollution occurs when certain substances are released into the air, which depending on the quantitative level, can be harmful to human health, animals, and the environment in general. The analysis of air shows the influence of the extractive and processing industry on the chemical composition of air. Parameters analyzed though under control such as the case of carbon dioxide, due to the increasing production capacity of cement, the production of hundreds of thousands of cubic meters of CO2 gas made CO2 production a concern. With the purchase of the latest technology by the SHARCEM Factory in Hani Elezit, the amount of air pollution has been reduced and the allowed parameters of environmental pollution have been kept under control. Air pollutants are introduced into the atmosphere from various sources which change the composition of the atmosphere and affect the biotic environment.The concentration of air pollutants depends not only on the quantities that are emitted from the sources of air pollution but also on the ability of the atmosphere to absorb or disperse these emissions. Sources of air pollutants include vehicles, industry, indoor sources, and natural resources. There are some natural pollutants, such as natural fog, particles from volcanic eruptions, pollen grains, bacteria, and so on.
KSII Transactions on Internet and Information Systems (TIIS)
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v.16
no.9
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pp.3172-3193
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2022
Cognitive radio-simultaneous wireless information and power transfer (CR-SWIPT) has attracted much interest since it can improve both the spectrum and energy efficiency of wireless networks. This paper focuses on the resource sharing between a point-to-point primary system (PRS) and a multiuser multi-antenna cellular cognitive radio system (CRS) containing a large number of cognitive users (CUs). The resource sharing optimization problem is formulated by jointly scheduling CUs and adjusting the transmit power at the cognitive base station (CBS). The effect of accessing CUs' spatial channel correlation on the possible transmit power of the CBS is investigated. Accordingly, we provide a low-complexity suboptimal approach termed the semi-correlated semi-orthogonal user selection (SC-SOUS) algorithm to enhance the spectrum efficiency. In the proposed algorithm, CUs that are highly correlated to the information decoding primary receiver (IPR) and mutually near orthogonal are selected for simultaneous transmission to reduce the interference to the IPR and increase the sum rate of the CRS. We further develop a spatial correlation-based resource sharing (SC-RS) strategy to improve energy sharing performance. CUs nearly orthogonal to the energy harvesting primary receiver (EPR) are chosen as candidates for user selection. Therefore, the EPR can harvest more energy from the CBS so that the energy utilization of the network can improve. Besides, zero-forcing precoding and power control are adopted to eliminate interference within the CRS and meet the transmit power constraints. Simulation results and analysis show that, compared with the existing CU selection methods, the proposed low-complex strategy can enhance both the achievable sum rate of the CRS and the energy sharing capability of the network.
International Journal of Computer Science & Network Security
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v.22
no.9
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pp.287-299
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2022
The article deals with the formation of managerial competence of the future head of preschool education institution by means of information and communication technology as a prerequisite for his ability to act competently and objectively evaluate actions and understand the interaction of forms and content of preschool education. The article aimed to study the effectiveness of information and communication technologies in the formation of managerial competence of the future head of preschool education institution. To achieve the objectives, the methods of comparative and systematic analysis were used to compare different views on the problem under study, namely, the formation of managerial competence of the future head of preschool education institution by means of information and communication technologies. The authors of the article determined that the use of information and communication technologies in the preparation of future heads of preschool educational institutions is of great importance and is an indicator in the structure of managerial competence. The priority directions of the use of various software products for the study of the modern Ukrainian language, methods of teaching the Ukrainian language contribute to the intensification of learning material. It is noted that the current state of development of information technologies and their widespread use in education satisfies the requirements of the objectivity of the assessment obtained the quality of the control process of forming the managerial competence of the future leader in the context of the general problems of pre-school education. It is noted that the means of information and communication technologies play a leading role in creating new educational policies and projects, as they motivate the way of access to knowledge.
Cho, Sung Kyum;LoCascio, Sarah Prusoff;Kim, Sungjoong
Asian Journal for Public Opinion Research
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v.9
no.2
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pp.188-211
/
2021
Decreasing response rates to traditional survey methods, like face-to-face and telephone interviews, have led survey practitioners around the world to seek new ways of conducting surveys in recent years." The COVID-19 pandemic exacerbated this problem because it made conducting face-to-face interviews even more difficult than before. For example, it made conducting face-to-face surveys infeasible in 2020 in South Korea, and so the Korean Academic Multimode Open Survey (KAMOS) was unable to conduct a planned face-to-face survey to recruit new panel members. The entire 8,514-member panel, established via two-stage probability-based sampling from 2016 to 2019, was invited to take three online/telephone surveys in 2020. Of these panel members, 1,352 responded to at least one survey in 2020. To test to what extent the panel remained representative of the adult South Korean population, we compared the two groups of panel members: those who responded to at least one survey in 2020 and those who did not. After weighting both groups on the basis of age, sex, and geographical area, we analyzed their responses to some of the questions that were asked during multiple rounds of the face-to-face panel-recruiting interviews. Using Cohen's d for survey items that could be analyzed numerically and Cramér's V for categorical items, we were able to conclude that the respondents to the 2020 surveys were equivalent to the non-respondents in terms of both demographics and in the answers they originally gave to substantive questions on a variety of topics related to social science or public opinion research, including questions about quality of life, societal issue, and politics (Cohen's d items <0.2, 95% CI; Cramér's V items <0.1, 95% CI). This analysis may provide a model for others who wish to test the continued representativeness of their panel or who would like to use a different survey mode or change some other aspect of their methodology and test whether it is equivalent to their former methodology. Our success in building a panel that retained its representativeness may be useful to those in other countries where face-to-face surveys had previously been the norm but are becoming increasingly difficult to conduct.
Journal of the Korea Society of Computer and Information
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v.26
no.5
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pp.77-86
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2021
This study aims at exploring the change of the total fertility rate(TFR) in regional level and analysing what different effects the local labor market and housing market have on the change of TFR. Previous studies have emphasized that the job and housing issues of the youth are structural factors on the decline of TFR. However, considering that youth problem is variant in local level, the relationship of job and housing issues with TFR could be different in local level. This study analyses what effects the situation of local labor market and housing market have on the TFR from 2012 to 2018 in regional level. The result is that the employment and housing factors have different effects on capital areas and non-capital areas. While the high cost of housing has negative effects on TFR in capital areas, it has rather positive effects in non-capital areas. However, labor market variables have statistically insignificant effects on TFR.
Journal of the Korea Academia-Industrial cooperation Society
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v.22
no.5
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pp.98-107
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2021
During the sea trial test for the aerovane in AOE-II, a malfunction occurred in the wind detector movement around 40 to 90° on the starboard side and around 270 to 320° on the port side. The purpose of this study was to analyze and improve the cause of the above phenomenon. The wind detector was covered with a mast structure and was not affected directly by the blowing wind, and a vortex air current was formed behind the mast structure, causing the motion malfunction. The above problem was solved by moving and installing the wind detector on the newly installed mast yard. After conducting vibration analysis and reviewing the effects of vibration, the wind detector was installed in the new mast yard and verified by a sea trial test. Since the improvement plan was slightly effective, the position of the wind detector moved away from the mast on the new mast yard and was re-verified through a sea trial test. The movement malfunction of the wind detector did not occur, confirming that it is an appropriate improvement plan.
Ju, Chanyang;Lee, Hyeonbum;Chung, Heewon;Seo, Jae Hong
Journal of the Korea Institute of Information Security & Cryptology
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v.31
no.4
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pp.675-686
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2021
According to the recent revision of Privacy Policy and the emerging importance of personal information, cooperations must verify customer identity (Know Your Costomer, KYC) while processing and managing this information so that it does not violate the Privacy Policy. One of the solution of this problem is zero-knowledge proof (ZKP). The use of the ZKP enables to verify the identity without exposing the identity information directly, thereby reducing the burden on the management of personal information while fulfilling the obligation of the cooperations to verify the identity. The ZKP could be employed to many other applications. In this paper, we analyze the ZKP technique and its applications currently being actively studied.
Small Modular Reactors (SMRs) are attracting wide attention due to their outstanding performance, extensive studies have been carried out for lead-based fast reactors (LFRs) that cooled with Lead or Lead-bismuth (LBE), and small modular natural circulation LFR is one of the promising candidates for SMRs and LFRs development. One of the challenges for the design small modular natural circulation LFR is to master the natural circulation thermal-hydraulic performance in the reactor primary circuit, while the natural circulation characteristics is a coupled thermal-hydraulic problem of the core thermal power, the primary loop layout and the operating state of secondary cooling system etc. Thus, accurate predicting the natural circulation LFRs thermal-hydraulic features are highly required for conducting reactor operating condition evaluate and Thermal hydraulic design optimization. In this study, a thermal-hydraulic analysis code is developed for small modular natural circulation LFRs, which is based on several mathematical models for natural circulation originally. A small modular natural circulation LBE cooled fast reactor named URANUS developed by Korea is chosen to assess the code's capability. Comparisons are performed to demonstrate the accuracy of the code by the calculation results of MARS, and the key thermal-hydraulic parameters agree fairly well with the MARS ones. As a typical application case, steady-state analyses were conducted to have an assessment of thermal-hydraulic behavior under nominal condition, and several parameters affecting natural circulation were evaluated. What's more, two characteristics parameters that used to analyze natural circulation LFRs natural circulation capacity were established. The analyses show that the core thermal power, thermal center difference and flow resistance is the main factors affecting the reactor natural circulation. Improving the core thermal power, increasing the thermal center difference and decreasing the flow resistance can significantly increase the reactor mass flow rate. Characteristics parameters can be used to quickly evaluate the natural circulation capacity of natural circulation LFR under normal operating conditions.
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