• Journal of Genetic Medicine
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• v.20 no.1
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• pp.6-14
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• 2023

Fabry disease (FD), a rare X-linked lysosomal storage disorder, is caused by mutations in the α-galactosidase A gene gene encoding α-galactosidase A (α-Gal A). The functional deficiency of α-Gal A results in progressive accumulation of neutral glycosphingolipids, causing multi-organ damages including cardiac, renal, cerebrovascular systems. The current treatment is comprised of enzyme replacement therapy (ERT), oral pharmacological chaperone therapy and adjunctive supportive therapy. ERT has been introduced 20 years ago, changing the outcome of FD patients with proven effectiveness. However, FD patients have many unmet needs. ERT needs a life-long intravenous therapy, inefficient bio-distribution, and generation of anti-drug antibodies. Migalastat, a pharmacological chaperone, augmenting α-Gal A enzyme activity only in patients with mutations amenable to the therapy, is now available for clinical practice. Furthermore, these therapies should be initiated before the organ damage becomes irreversible. Development of novel drugs aim at improving the clinical effectiveness and convenience of therapy. Clinical trial of next generation ERT is underway. Polyethylene glycolylated enzyme has a longer half-life and potentially reduced antigenicity, compared with standard preparations with longer dosing interval. Moss-derived enzyme has a higher affinity for mannose receptors, and seems to have more efficient access to podocytes of kidney which is relatively resistant to reach by conventional ERT. Substrate reduction therapy is currently under clinical trial. Gene therapy has now been started in several clinical trials using in vivo and ex vivo technologies. Early results are emerging. Other strategic approaches at preclinical research level are stem cell-based therapy with genome editing and systemic mRNA therapy.

• Proceedings of the Korean Society of Crop Science Conference
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• 2022.10a
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• pp.219-219
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• 2022

Granule-bound starch synthase I (GBSSI), encoded by the waxy gene, is responsible for the accumulation of amylose during the development of starch granules in rice endosperm. Despite many findings on waxy alleles, the genetic diversity and evolutionary studies are still not fully explored regarding their functional effects. Comprehensive evolutionary analyses were performed to investigate the genetic variations and relatedness of the GBSSI gene in 374 rice accessions composed of 54 wild accessions and 320 bred cultivars (temperate japonica, tropical japonica, indica, aus, aromatic, and admixture). GBSS1 coding regions were analyzed from a VCF file retrieved from whole-genome resequencing data, and eight haplotypes were identified in the GBSSI coding region of 320 bred cultivars. The genetic diversity indices revealed the most negative Tajima's D value in the tropical-japonica, followed by the aus and temperate-japonica, while Tajima's D values in indica were positive, indicating balancing selection. Diversity reduction was noticed in temperate japonica (0.0003) compared to the highest one (wild, 0.0044), illustrating their higher genetic differentiation by F_ST-value (0.604). The most positive Tajima's D value was observed in indica (0.5224), indicating the GBSSI gene domestication signature under balancing selection. In contrast, the lowest and negative Tajima's D value was found in tropical japonica (-0.5291), which might have experienced a positive selection and purified due to the excess of rare alleles. Overall, our study offers insights into haplotype diversity and evolutionary fingerprints of GBSSI. It ako provides genomic information to increase the starch content of cooked rice.

• Korean Medical Education Review
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• v.25 no.3
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• pp.192-197
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• 2023

Physicians should be able to address health-related issues of patients and populations from a multidimensional perspective. Therefore, medical schools have a social responsibility to develop and implement curricula that enable trainees to acquire the competencies needed to improve all aspects of patient care and healthcare delivery. This study explored the concept of health systems science concept as the third pillar of medical education (the other two are basic science and clinical medicine) in the Korean context, as well as related educational needs. The theoretical foundation of health systems science is the biopsychosocial conceptual model, which emphasizes the biological, psychological, and social factors surrounding patients. We concluded that the three domains (core functional, foundational, linking) and 12 subcategories of health systems science proposed by the Association of American Medical Colleges could be applied to Korean medical education. Health systems science education must be emphasized to solve the various healthcare problems facing Korea today and to train physicians to provide medical services in line with society's needs. Introducing a health systems science curriculum will be challenging in the Korean medical environment, which has traditionally emphasized basic science and clinical medical education. Health systems science education should begin in the basic medical education phase, where physicians' professional identity is formed, and continue through graduate medical education. It is essential to understand related educational needs, develop curricular content, conduct faculty development programs, and provide financial resources for the development of an integrated curriculum.

• The Transactions of the Korea Information Processing Society
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• v.13 no.2
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• pp.26-33
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• 2024

There has been a recent surge of interest in the Digital Twin technology. The Digital Twin is technique for optimizing objects by simulating physical phenomena or objects through computer-based simulations. Currently, single Digital Twin is being developed to optimize processes limited to specific fields, but there is a limitation in that the independent Digital Twins cannot analyze the vast and complex processes of the real world. To overcome this, the concept of federated Digital Twin has been introduced. To date, the federated Digital Twin research has primarily focused on how to optimize macroscopic objects such as cities. However, by leveraging the interconnected nature of twins, existing implementations of the single Digital Twins can be modularized. In this study, we define the concepts and interrelationships of the single Digital Twin and the federated Digital Twin from a functional perspective related to process optimization and design a modularization technique for the single Digital Twin using the federated Digital Twin. Furthermore, this study aims to discuss the proposed methodology's efficacy by designing a model applying modularization to a real-world fabric manufacturing case.

• Journal of Ginseng Research
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• v.48 no.1
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• pp.68-76
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• 2024

Background: Although the survival outcomes of childhood cancer patients have improved, childhood cancer survivors suffer from various degrees of immune dysfunction or delayed immune reconstitution. This study aimed to investigate the effect of Korean Red Ginseng (KRG) on T cell recovery in childhood cancer patients who underwent autologous hematopoietic stem cell transplantation (ASCT) from the perspective of inflammatory and senescent phenotypes. Methods: This was a single-arm exploratory trial. The KRG group (n = 15) received KRG powder from month 1 to month 12 post-ASCT. We compared the results of the KRG group with those of the control group (n = 23). The proportions of T cell populations, senescent phenotypes, and cytokine production profiles were analyzed at 1, 3, 6, and 12 months post-ASCT using peripheral blood samples. Results: All patients in the KRG group completed the treatment without any safety issues and showed a comparable T cell repopulation pattern to that in the control group. In particular, KRG administration influenced the repopulation of CD4⁺ T cells via T cell expansion and differentiation into effector memory cell re-expressing CD45RA (EMRA) cells. Although the KRG group showed an increase in the number of CD4⁺ EMRA cells, the expression of senescent and exhausted markers in these cells decreased, and the capacity for senescence-related cytokine production in the senescent CD28^- subset was ameliorated. Conclusions: These findings suggest that KRG promotes the repopulation of CD4⁺ EMRA T cells and regulates phenotypical and functional senescent changes after ASCT in pediatric patients with cancer.

• Journal of Dental Rehabilitation and Applied Science
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• v.40 no.1
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• pp.24-30
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• 2024

Complicated crown fractures of maxillary anterior teeth caused by trauma in adolescence can cause functional and aesthetic problems. For crown fractures with pulp exposure, various restorative methods can be considered depending on the amount of remaining tooth structure. Direct resin restorations are the most traditional and effective method, but they are likely to discolor and break over time. Fixed prosthesis have a high possibility of re-restoration due to marginal disharmony due to tooth movement during the growth period, and restorations using post which are mainly performed for extensive crown fractures increase the risk of root perforation and root fracture. However, endocrown is an integrated structure that gains retention force from the pulp space, enabling effective reconstruction from a biomechanical perspective and providing advantages in restoring function and aesthetics. Therefore, endocrown can be considered as a restoration method for complicated crown fractures caused by trauma in adolescence.

• Journal of the Korea Fashion and Costume Design Association
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• v.26 no.2
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• pp.57-72
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• 2024

Currently, Korea is on the verge of entering into an ultra-aged society and the associated market size is growing. In this regard, customer-centered design that understands the consumer needs of the new silver generation, which is emerging as a new consumer group, and reflects them in products is the key to the success of the high-value-added fashion industry. Therefore, this study reviewed the changes in physical, biological, social, and psychological characteristics of the new silver generation, through a review of related books and previous studies, and secured the direction for clothing product development for the new silver generation. The literature review was supplemented by group interviews to accurately identify the needs of silver consumers. In addition, the study conducted case analysis through web searches of fashion magazines, newspaper articles, brand web sites, and trend information sites to investigate the market trends of one-mile wear products that have emerged due to the COVID-19 pandemic, the characteristics of one-mile wear brands, and products for new silver women, including size specification analysis and sewing techniques that minimize skin irritation while reflecting the consumer's characteristics. Based on the results of this study, a competitive design and product development were proposed from an economic and industrial perspective that can enhance the product value and maximize the marketing effect by developing consumer-oriented one-mile comfort wear products with aesthetic, practical, functional, and economic values that satisfy the characteristics and needs of new silver women.

• Journal of the Korean Institute of Landscape Architecture
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• v.40 no.2
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• pp.1-14
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• 2012

The purpose of this study is to derive a desirable riverfront construction plan to me for the activities of citizens through the evaluation and analysis of the urban riverfront space from the perspective of universal design. Therefore, previous studies were examined in order to induce evaluation tools that bhve been grafted from the universal design concept; in addition, a field survey was conducted in Shincheon, Daegu, which was selected as the study target, in order to induce problems and improvement directions from the perspective of universal design. The major results can be summarized as follows. (1) In the 'fairness' aspect, all the items such as installation of integrated functional signage, showing pictures, symbols, foreign language signs, and restroom entrances signage were determined to be 'All Unsuitable' for all sections; and therefore, it is necessary to improve the fairness of usage for everybody. (2) In the 'Functionality(Usability)' aspect, all items such as installation of bicycle paths beside access roads, installation of integrated functional signs, and night light signs were determined to be 'All Unsuitable' for all sections; therefore, it is necessary to improve the functionalities of these facilities. (3) In the 'Convenience' aspect, all items such as the installation of bicycle parking areas, continuous rest facilities, and back and ann support(handles) at resting facilities were determined to be 'All Unsuitable' for many sections; therefore, it is necessary to improve these facilities for the convenience of usage. (4) In the 'Information(Recognizability)' aspect, all items such as showing pictures, symbols, foreign languages and installation of night light signs, and restroom entrances signage were determined to be 'All Unsuitable' for all sections; therefore, it is necessary to improve the recognizability to minimize misunderstandings and confusion. (5) In the 'Safety' aspect, all items such as the installation of safe pedestrian paths in parking areas, using anti-slip and shock absorption materials on restroom floors, and the continuous installation of pedestrian paths that are separate from bicycle paths were determined to be 'All Unsuitable' for all sections; therefore, it is necessary to improve the safety to prevent accidents. (6) In the 'Amenity' aspect, access roads, parking areas, hygiene facilities, convenience facilities, and waterside facilities for many sections were determined to be 'All Unsuitable'; therefore, it is necessary to conduct more concentrated hygiene management. (7) In the 'Accessibility(Mobility)' aspect, all items such as the installation of safe pedestrian paths in parking areas, and continuous pedestrian paths that are separate from bicycle paths were determined to be 'All Unsuitable' for all sections; therefore, it is necessary to improve the accessibility to provide safety and convenience. (8) In the 'Durability' aspect, access roads, parking areas, rest facilities, convenience facilities, fitness facilities, and waterside facilities were determined to be 'All Unsuitable' for many sections, therefore, it is necessary to improve sunken or damaged areas by inspecting facilities by section.

• Asia-Pacific Journal of Business Venturing and Entrepreneurship
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• v.10 no.4
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• pp.95-111
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• 2015

This purpose of this study is to academically define what "luxury products" mean, and to clarify the cognitive structural relationship between product attributes and "luxuriousness" in relation to the determinant of luxuriousness that eventually affects the purchase intention of consumers. This study is also written under the theory that luxuriousness leads to benefits such as functional benefit, experiential benefit, symbolic benefit, and exclusive benefit; which all provide core values to consumers and serve as effect factors that influence the psychological satisfaction of consumers. The commonly discussed measure of the determinant of luxuriousness has often failed to adequately describe the reasons to why the consumers prefer luxury products. This study also aims to suggest a new path in how and what the consumers perceive as luxury products, and to conceptualize what luxuriousness means in the marketing perspectives on the background of a previous theory. The part of product attributes are divided in the following sections: the quality and design of functional cues and brands of symbolic cues, as well as prices. The part of the determinant of luxuriousness is divided in sections of superiority, scarcity, differentiation, and traditionality. Therefore, this study used the theoretical concept used in such common measures as well as 282 examples to empirically analyze the relationship between product attributes and luxuriousness of luxury products, and how such affect the purchase intentions of consumers. The survey used to aid this study targeted luxury product consumers regarding "B" brand of automobiles. Using the analysis through a structural equation model, the study draws a conclusion that in a relationship between product attributes and luxuriousness, quality(which is one of the functional cues) affects the facts of superiority, differentiation in a significant way, and design affects the factors of scarcity in a significant way. And brand(one of the symbolic cues) significantly affects the factors of traditionality within the luxuriousness factors, and price appeared to affect superiority and scarcity. Additionally, the brand of product attributes significantly affects purchase intention, and superiority and scarcity of luxuriousness affects purchase intention in a significant way. The structural concept of luxury and luxuriousness suggested in the study will provide the theoretical basis for building a new case study, and the determinants of luxuriousness in the marketing perspective will be a practical help for checking the consumers' psychological reasons for purchase.

• Asia pacific journal of information systems
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• v.20 no.3
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• pp.139-166
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• 2010

The supply chain management (SCM) systems have emerged as strong managerial tools for manufacturing firms in enhancing competitive strength. Despite of large investments in the SCM systems, many companies are not fully realizing the promised benefits from the systems. A review of literature on adoption, implementation and success factor of IOS (inter-organization systems), EDI (electronic data interchange) systems, shows that this issue has been examined from multiple theoretic perspectives. And many researchers have attempted to identify the factors which influence the success of system implementation. However, the existing studies have two drawbacks in revealing the determinants of systems implementation success. First, previous researches raise questions as to the appropriateness of research subjects selected. Most SCM systems are operating in the form of private industrial networks, where the participants of the systems consist of two distinct groups: focus companies and vendors. The focus companies are the primary actors in developing and operating the systems, while vendors are passive participants which are connected to the system in order to supply raw materials and parts to the focus companies. Under the circumstance, there are three ways in selecting the research subjects; focus companies only, vendors only, or two parties grouped together. It is hard to find researches that use the focus companies exclusively as the subjects probably due to the insufficient sample size for statistic analysis. Most researches have been conducted using the data collected from both groups. We argue that the SCM success factors cannot be correctly indentified in this case. The focus companies and the vendors are in different positions in many areas regarding the system implementation: firm size, managerial resources, bargaining power, organizational maturity, and etc. There are no obvious reasons to believe that the success factors of the two groups are identical. Grouping the two groups also raises questions on measuring the system success. The benefits from utilizing the systems may not be commonly distributed to the two groups. One group's benefits might be realized at the expenses of the other group considering the situation where vendors participating in SCM systems are under continuous pressures from the focus companies with respect to prices, quality, and delivery time. Therefore, by combining the system outcomes of both groups we cannot measure the system benefits obtained by each group correctly. Second, the measures of system success adopted in the previous researches have shortcoming in measuring the SCM success. User satisfaction, system utilization, and user attitudes toward the systems are most commonly used success measures in the existing studies. These measures have been developed as proxy variables in the studies of decision support systems (DSS) where the contribution of the systems to the organization performance is very difficult to measure. Unlike the DSS, the SCM systems have more specific goals, such as cost saving, inventory reduction, quality improvement, rapid time, and higher customer service. We maintain that more specific measures can be developed instead of proxy variables in order to measure the system benefits correctly. The purpose of this study is to find the determinants of SCM systems success in the perspective of vendor companies. In developing the research model, we have focused on selecting the success factors appropriate for the vendors through reviewing past researches and on developing more accurate success measures. The variables can be classified into following: technological, organizational, and environmental factors on the basis of TOE (Technology-Organization-Environment) framework. The model consists of three independent variables (competition intensity, top management support, and information system maturity), one mediating variable (collaboration), one moderating variable (government support), and a dependent variable (system success). The systems success measures have been developed to reflect the operational benefits of the SCM systems; improvement in planning and analysis capabilities, faster throughput, cost reduction, task integration, and improved product and customer service. The model has been validated using the survey data collected from 122 vendors participating in the SCM systems in Korea. To test for mediation, one should estimate the hierarchical regression analysis on the collaboration. And moderating effect analysis should estimate the moderated multiple regression, examines the effect of the government support. The result shows that information system maturity and top management support are the most important determinants of SCM system success. Supply chain technologies that standardize data formats and enhance information sharing may be adopted by supply chain leader organization because of the influence of focal company in the private industrial networks in order to streamline transactions and improve inter-organization communication. Specially, the need to develop and sustain an information system maturity will provide the focus and purpose to successfully overcome information system obstacles and resistance to innovation diffusion within the supply chain network organization. The support of top management will help focus efforts toward the realization of inter-organizational benefits and lend credibility to functional managers responsible for its implementation. The active involvement, vision, and direction of high level executives provide the impetus needed to sustain the implementation of SCM. The quality of collaboration relationships also is positively related to outcome variable. Collaboration variable is found to have a mediation effect between on influencing factors and implementation success. Higher levels of inter-organizational collaboration behaviors such as shared planning and flexibility in coordinating activities were found to be strongly linked to the vendors trust in the supply chain network. Government support moderates the effect of the IS maturity, competitive intensity, top management support on collaboration and implementation success of SCM. In general, the vendor companies face substantially greater risks in SCM implementation than the larger companies do because of severe constraints on financial and human resources and limited education on SCM systems. Besides resources, Vendors generally lack computer experience and do not have sufficient internal SCM expertise. For these reasons, government supports may establish requirements for firms doing business with the government or provide incentives to adopt, implementation SCM or practices. Government support provides significant improvements in implementation success of SCM when IS maturity, competitive intensity, top management support and collaboration are low. The environmental characteristic of competition intensity has no direct effect on vendor perspective of SCM system success. But, vendors facing above average competition intensity will have a greater need for changing technology. This suggests that companies trying to implement SCM systems should set up compatible supply chain networks and a high-quality collaboration relationship for implementation and performance.