• Journal of Genetic Medicine
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• v.18 no.2
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• pp.127-131
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• 2021

Autosomal recessive spinocerebellar ataxia 20 (SCAR20; OMIM #616354) is a recently described disorder that is characterized by ataxia, intellectual disability, cerebellar atrophy, macrocephaly, coarse face, and absent speech. It is caused by loss-of-function mutations in SNX14. To date, all cases with homozygous pathogenic variants have been identified in consanguineous families. This report describes the first Korean cases of SCAR20 family caused by homozygous variants in SNX14. Two siblings were referred to our clinic because of severe global developmental delay. They presented similar facial features, including a high forehead, long philtrum, thick lips, telecanthus, depressed nasal bridge, and broad base of the nose. Because the older sibling was unable to walk and newly developed ataxia, repeated brain magnetic resonance imaging (MRI) was performed at the age of 4 years, revealing progressive cerebellar atrophy compared with MRI performed at the age of 2 years. The younger sibling's MRI revealed a normal cerebellum at the age of 2 years. Whole-exome sequencing was performed, and homozygous variants, such as c.2746-2A>G, were identified in SNX14 from the older sibling. Sanger sequencing confirmed homozygous SNX14 variants in the two siblings as well as a heterozygous variant in both parents. This report extends our knowledge of the phenotypic and mutational spectrum of SCAR20. We also highlight the importance of deep phenotyping for the diagnosis of SCAR20 in individuals with developmental delay, ataxia, cerebellar atrophy, and distinct facial features.

• Journal of the Korean Society for Library and Information Science
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• v.56 no.3
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• pp.119-152
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• 2022

This study examined the activities of Community of Practice (CoP) of research data curators, including their community building and maintenance activities, by using activity theory as its main theoretical framework. The study used an embedded multiple-case study design for exploring the activities of three CoP of research data curators. The case study design used document analysis and semi-structured interviews to collect data. Besides the community building and maintenance activities, other activities were performed in the CoP of research data curators, which could be largely divided into curation activities, education activities, and communication activities. Membership was largely divided into institutional membership and individual membership, and the motives for members to join and contribute to the CoP vary from extrinsic to intrinsic motivations. Finally, we identified and categorized challenges and success indicators for CoP of research data curators as perceived by participants. In particular, we identified three types of challenges or contradictions the communities faced and 14 perceived indicators of success. This study informs the practice of research data curation by providing a knowledge base for building and maintaining a CoP of research data curators.

• Earthquakes and Structures
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• v.24 no.4
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• pp.259-274
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• 2023

In this paper, mechanical properties of periodic foundation made of concrete and rubber are investigated by a parametric study using the finite element method (FEM). Periodic foundation is a special type of seismic isolation foundation used in civil engineering, which is inspired by the meso-scale structure of phononic crystals in solid-state physics. This type of foundation is capable of reducing the seismic wave propagating though the foundation, therefore providing additional protection for the structures. In the FEM analysis, layered periodic foundation is frequently modelled due to its simplicity in numerical modeling. However, the isolation effect of periodic foundation on nuclear power plant has not been fully discussed to the best knowledge of authors. In this work, we construct four numerical models of nuclear power plant with different foundations to investigate the seismic isolation effects of periodic foundations. The results show that the layered periodic foundation can increase the natural period of the nuclear power plant like traditional base isolation systems, which is beneficial to the structures. In addition, the seismic response of the nuclear power plant can also be effectively reduced in both vertical and horizontal directions when the frequencies of the incident waves fall into some specific frequency bandgaps of the periodic foundation. Furthermore, it is demonstrated that the layered periodic foundation can reduce the amplitude of the floor response spectrum, which plays an important role in the protection of the equipment.

• Nuclear Engineering and Technology
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• v.55 no.8
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• pp.3054-3070
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• 2023

Nuclear Power Plants (NPP) are widely utilized around the globe from different base forms as it is one of the most dependable renewable resources that technological advancements have offered. However, different perceptions of the usage of NPPs emerged from different generations. The purpose of this study was to investigate the acceptance of nuclear energy as an alternative source of energy among Generation Z in the Philippines by utilizing an extended Theory of Planned Behavior (TPB) approach. An online questionnaire which consisted of 31 items was distributed using a purposive sampling approach and 450 respondents of Generation Z voluntarily answered. Structural Equation Modeling (SEM) showed that the knowledge regarding NPP had significant effects on risk perception and benefit perception which subsequently led to subjective norms. In addition, perceived behavioral control and subjective norms had significant effects on behavioral intention which led to nuclear acceptance. Interestingly, the respondents perceived the benefit of NPP as slightly higher than the perceived risk. With these, it was clear that the commissioning Nuclear Power Plant must consider as an alternative source of electric energy in the Philippines. Moreover, this study is one of the first studies that investigated the acceptance of NPP among Generation Z. Lastly, the model could be a basis to strengthen the acceptance strategy of opening NPP among Generation Z, particularly in developing countries.

• Asia Marketing Journal
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• v.17 no.4
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• pp.63-88
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• 2016

K-Pop is hugely promoted offline/online in East Asia, while efforts to promote it elsewhere are kept to a minimum. Whilst addressing the role of digital marketing in the promotion of K-Pop in the Brazilian music industry this study aims to demonstrate that K-pop will provide its audience with a unique and engaging experience if it thinks globally and acts locally in its marketing and communication strategies. A survey of K-Pop's world fan base was carried out online with 1,074 fans to determine the validity of the hypothesis. The results have demonstrated the need to adapt global and intercultural strategies to local markets with increasing brand awareness through utilizing digital marketing. For example, to increase K-Pop's popularity and market share in Brazil, it is not required to sing in Brazilian Portuguese, contrary to what might be perceived but fully singing in English would help breaking into the market dominated by domestic music as songs would have a higher probability to be included in the international soundtrack of a Brazilian telenovela and promote the K-Pop artist not only across Brazil but also give the artist exposure in all the markets the Brazilian telenovela is exported to, opening an entry door to those markets. K-Pop audience segmentation and psychographic profiling is crucial to the understanding of each market's consumer's preferences, likes, dislikes and their buying habits as it was proven through Brazil's digital media, social media, digital music market and K-Pop market analysis within the global context of the study. It should be also considered that brand extensions are not standard everywhere and that there are cultures where different product categories are not directly associated with its main corporate owner which requires extensive local market knowledge to succeed. The primary and secondary data research that we conducted for this study intended to demonstrate that K-Pop can be successful in Brazil and in Latin America and increase their local and regional market share if digital marketing and communication strategies are tailored to each individual market.

• Journal of the Korea Society of Computer and Information
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• v.14 no.8
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• pp.73-80
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• 2009

It is difficult that expertise human supply and demand for industry requires by imbalance of industry necessity human and profession organs of education's Skill Mismatch. Industry can prove productivity though reeducate school graduation person in spot and master correct technology in industry special quality. This paper is research that accommodate Demand Education that industry requires and make out full text caution Curriculum Specializing Vocational High School in e-Business field. Analysis e-Business industrial classification and occupational classification. Analysis knowledge and technological level that require in industry about e-Business education and investigate and analyze the demand. Base industry, Support industry, Apply e-Business Curriculum that is examined by practical use industry to learning, Do to estimate satisfaction about Demand Education Curriculum of industry and confirm Success special quality with research and investigation and application wave. Suggested for e-Business Curriculum's basis model in this paper and school subject Curriculum. Wish to contribute in nation development through productivity elevation through e-Business education of industry request.

• Journal of Digital Convergence
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• v.20 no.5
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• pp.857-866
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• 2022

The current study is a meta analysis study to identify the factors related to infection management performance and the effect size according to the factors. Data collection was included from the data of KMBASE, RISS, KISS, DBpia, National Library of Korea, Pubmed, and EMBASE. R3.5.1 was used for the data analysis. A total of 22 factors were identified. The meta analysis showed the effect size as follows: gender(.16), age(.30), education(.32), beds(.28), needs of education in infection management(.44), participating in education of infection management(.25), recognition/knowledge in infection management(.70). Based on the findings, evidence based programs need to be provided to improve the infection management performance of health workers at Long-Term Care Hospitals.

• KSCE Journal of Civil and Environmental Engineering Research
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• v.28 no.4C
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• pp.239-245
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• 2008

In this study, a fundamental-level study was performed to establish knowledge-base for the development of optimal surface-wave method for urban areas with adjacent structures. First, theoretical modelling was performed to investigate the influence of adjacent structures on dispersion characteristics of surface waves. Later, the geotechnical sites with a concrete model of adjacent structure and a real subway box structure were tested by surface-wave method to investigate the influence of adjacent structures. The major influencing factors of adjacent structures on surface-wave propagation were direct distance between measurement array and adjacent structure, stiffness contrast between layers and type of seismic source.

• Convergence Security Journal
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• v.22 no.1
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• pp.149-158
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• 2022

R.o.K Marine Corps was established on April 15, 1949 at Deoksan Air Base in Jinhae. When they fought in the Korean War and the Vietnam War, they were sure to win, so they were loved and trusted by the people. Strong combat power is only possible when an advanced barracks culture is established. Today's new generation of soldiers have strong characteristics ; self-centered and individualistic. R.o.K Marine Corps officers must understand the characteristics of the new generation of soldiers and strive to develop creative leadership in order to make them utilize their characteristics. In addition, as cutting-edge science and technology develops along with the 4th industrial revolution, R.o.K Marine Corps officers should work hard to acquire professional tactical knowledge and self-development. Therefore, Marine Corps officers should do their best to understand the characteristics of the new generation of soldiers and demonstrate leadership to become a strong force trusted by the people.

• Journal of Information Science Theory and Practice
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• v.10 no.spc
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• pp.25-38
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• 2022

Research data means data used or created in the course of research or experiments. Research data is very important for validation of research conducted and for use in future research and projects. Recently, convergence research between various fields and international cooperation has been continuously done due to the explosive increase of research data and the increase in the complexity of science and technology. Developed countries are actively promoting open science policies that share research results and processes to create new knowledge and values through convergence research. Communities to promote the sharing and utilization of research data such as RDA (Research Data Alliance) and COAR (Confederation of Open Access Repositories) are active, and various platforms for managing and sharing research data are being developed and used. OpenAIRE (Open Access Infrastructure for Research In Europe), a research data platform in Europe, ARDC (Australian Research Data Commons) in Australia, and IRDB (Institutional Repositories DataBase) in Japan provide research data or research data related services. Korea has been establishing and implementing a research data sharing and utilization strategy to promote the sharing and utilization of research data at the national level, led by the central government. Based on this strategy, KISTI has been building a Korean research data platform (DataON) since 2018, and has been providing research data sharing and utilization services to users since January 2020. This paper reviews the characteristics of DataON and how it is used for research by showing its applications.