• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.18 no.3
• /
• pp.99-106
• /
• 2018

Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.

• Journal of Life Science
• /
• v.28 no.11
• /
• pp.1354-1360
• /
• 2018

The aim of this study was to develop a simple, environmentally friendly synthesis of silver nanoparticles (SNPs) without the use of chemical reducing agents by exploiting the extracellular synthesis of SNPs in a culture supernatant of Bacillus thuringiensis CH3. Addition of 5 mM $AgNO_3$ to the culture supernatant at a ratio of 1:1 caused a change in the maximum absorbance at 418 nm corresponding to the surface plasmon resonance of the SNPs. Synthesis of SNPs occurred within 8 hr and reached a maximum at 40-48 hr. The structural characteristics of the synthesized SNPs were investigated by various instrumental analysis. FESEM observations showed the formation of well-dispersed spherical SNPs, and the presence of silver was confirmed by EDS analysis. The X-ray diffraction spectrum indicated that the SNPs had a face-centered cubic crystal lattice. The average SNP size, calculated using DLS, was about 51.3 nm and ranged from 19 to 110 nm. The synthesized SNPs exhibited a broad spectrum of antimicrobial activity against a variety of pathogenic Gram-positive and Gram-negative bacteria and yeasts. The highest antimicrobial activity was observed against C. albicans, a human pathogenic yeast. The FESEM observations determined that the antimicrobial activity of the SNPs was due to destruction of the cell surface, cytoplasmic leakage, and finally cell lysis. This study suggests that B. thuringiensis CH3 is a potential candidate for efficient synthesis of SNPs, and that these SNPs have potential uses in a variety of pharmaceutical applications.

• International Area Studies Review
• /
• v.22 no.3
• /
• pp.121-148
• /
• 2018

This paper examines the role of accounting conservatism on investment expenditure for non-financial Korean listed firms around the 2007-2008 global financial crisis using a differences-in-differences design. Specifically, this paper examines the association between an ex ante classification of firms by their level of accounting conservatism prior to the credit crisis and the ex post magnitude of the decline in investment. Consistent with prior literature, this study found that firms experienced a decline in their investment when hit by the financial crisis (Campello et al. 2010). And also this study found that firms with more conservative financial reporting experienced a smaller decline in investment activity following the financial crisis than did firms with less conservative financial reporting. Together, the results suggest that negative shocks to the supply of external finance hampers firm-level investment and that conservative financial reporting can lessen the sensitivity of firms' investment to such negative shocks. Next, this study shows that the magnitude of our findings is greater for firms more likely to suffer from underinvestment (as opposed to overinvestment). Firms that are financially constrained or have greater demand for external finance are more likely to experience underinvestment. Consistent with the predictions, this study finds stronger benefits of conservatism for firms that face relatively greater costs in raising external capital (i.e., financially constrained firms) or that have a relatively greater need to do so (i.e., firms that lack internal financial resources). This study also finds that the role for conservatism is greater in firms with a higher level of information asymmetry, consistent with the notion that conservatism mitigates financing frictions arising from information problems.

• Journal of Climate Change Research
• /
• v.5 no.1
• /
• pp.1-12
• /
• 2014

With continued economic growth, Korea has seen an increase in the nighttime activities of its citizens as hours of activity have extended into night. There is an increasing trend in energy consumption related to citizens' nighttime activities. In order to analyze ideas for an efficient replacement of the power consumption of streetlights and for profit generation by applying grid-type solar systems, this study used an RETScreen model. Through energy analysis and cost analysis, the application benefit and viability of grid-type solar street light systems were analyzed. With analysis result of a total weekly power generation of 114 kWh via a grid-connected solar streetlight system, it was shown that the net present value of a grid-connected solar street light system is 155,362 KRW, which would mean a payback period of about 5.2 years, and as such, it was shown that profit could be generated after about 6 years. In addition, if the grid-connected solar power generation system proposed by this study is to be applied, it was shown that 401,935 KRW in profit could be generated after the 20-year useful life set for the solar system. In addition, the sensitivity analysis was performed taking into account the price fluctuations of SMP, maintenance. As a result, a payback period has increased by 1~2 years, and there were no significant differences. Because the most important factor that affect the economic analysis is the cost of supply certification of renewable energy, a stable sales and acquisition of this certification are very important. the Seoul-type Feed in Tariff(FIT) connected to other institutions will enable steady sales by confirming to purchase the certification for 12 years. Therefore, if those issues mentioned above are properly reflected, Central-grid PV system project will be able to perform well in the face of unfavorable condition of solar PV installation.

• Korean Journal of Heritage: History & Science
• /
• v.52 no.2
• /
• pp.20-37
• /
• 2019

Three Inter-Korea summits and a North Korea-USA summit that were previously unexpected provide a basis for an optimistic outlook for the future development of Korean archaeology. While Korean archaeology has witnessed a great advance since the mid-20th century, it also exhibits significant weaknesses in explaining cultural changes in prehistory and the early historical period in the Korean Peninsula due to the paucity of information on archaeological evidence of North Korea. Recent development of favorable conditions for research and excavations of North Korean cultural heritage could be a valuable opportunity for Korean archaeology to overcome the current adversity. Especially, given the expected large-scale SOC industrial project in North Korea, we need to prepare for the systematic research and excavation of archaeological materials. The present essay attempts to provide a suggestion for the joint archaeological expeditions to excavate and manage cultural resources in North Korea based on a critical review of previous salvage excavations in South Korea, such as those conducted before the construction of the Korean rapid transit railway system (KTX). We suggest that professional archaeologists should be included in the project and oversee the planning and design of road and railway constructions and other SOC projects in order to minimize the cost of trial and error processes that were well exemplified by the KTX salvage excavations. The Korean Archaeological Society and North Korean Archaeological Society may organize a common association that will supervise joint archaeological expeditions. Importantly, The Korean Archaeological Society and other related institutions should prepare to build an organization that conducts impending archaeological excavation in North Korea. While we likely face challenges and difficulties during the various stages of archaeological research and excavations in North Korea, only through thorough and systematic preparation can we avoid the destruction of valuable cultural heritage and find an opportunity for the further development of Korean archaeology.

• The Korean Journal of Archival Studies
• /
• no.59
• /
• pp.207-275
• /
• 2019

This archiving project of the survivors of suicide was done with the survivor supporting team of the Seoul Suicide Prevention Center. The survivor supporting team was operating a Self-help Support Group for the emotional support of the survivors of suicide. A Self-help Support Group is a place for the survivors of suicide to regularly meet and share their suffering by talking of topics hard to discuss elsewhere. As the Self-help Support Group progressed members who acted as the leader of the group appeared. They formed an essay group that writes together. Two fathers who lost their sons, two mothers who lost their daughters, a mother who lost her son, a wife who lost his husband. The essay group met each week in a place facing Sajik Park. Through the windows that took up the whole side of the room, evening was coming in. The things that happened during the day went away towards Inwang mountain following the setting sun. Ten people (six members of the essay group, three from the survivor support team, a historian for unique conversation) sat around a table, facing each other. "Now, what shall we do?" History for unique conversation is a time that archives life by sharing conversations. At times a complete stranger, and other times people who share their ordinary lives sit around together (3-9 people, sometimes about 15). On the table there is coffee, bread, fruits and salads, and sometimes a dish someone heartily prepared. When a bottle of wine is placed on the table, each takes a glass. Morning, afternoon, the time the evening is welcomed in, late night. It does not matter which. For six months, 3 hours when meeting every week, 6 hours when at every other week. A room where the ambience is like that of a kitchen where sunlight enters, or a cozy living room is the best location. However, there are many times when it is held in a multipurpose room in the suburbs where many meetings are held, or in a classroom of a school. The meeting place is decided according to different situations of the time. There are no participation requirements as it is said to be for themselves to write down according to archiving form while looking back their lives thoroughly, and they are the only ones to stop themselves. The archives landscape from far away would seem like trying to do some talking. However, when going into a microscopic situation one must leave themselves to the emotional dynamics. It is because it archives the frustration and failures one experienced through life. A participator of history for unique conversation must face the sufferings of their life. The archiving project took place in 2013 to 2014. Many years have passed. Has the objective distance for archiving the situation of that time been secured? That may be uncertain, but I will speak of a few stray thoughts on archiving while depicting the process and method of operation.

• Korean Journal of Heritage: History & Science
• /
• v.45 no.2
• /
• pp.54-71
• /
• 2012

This paper investigates Buddhist sculptures of the Gulsan-sa School of Seon Buddhism, which was established by the priest Beomil(梵日) at Myeongju(溟州), present-day Gangneung(江陵) area, and prospered until the late Goryeo period. There are very few surviving Buddhist images at Gulsan2 temple-site showing the Buddhist art when Beomil or his disciples, Gaecheong(開淸) and Haengjeok(行寂), were active, except a set of huge stone pillars for temple-banners and a stone monk stupa. It is therefore necessary to focus on pieces of artworks in Gangneung and nearby areas to explore the characteristics of Buddhist art of Gulsan-sa from the late Silla to early Goryeo period. For example, the stone relief seated Buddha image, one of Four Buddhas of Four Directions, and a stone relief of standing Guardian King both from the stone pagoda at Mujin temple-site(無盡寺址) can be compared with stupa reliefs at the capital Gyeongju area in their high quality of carving. The stone octagonal base showing seven lions on each of seven sides at Boheon-sa(普賢寺) demonstrates that it was made as a part of an octagonal lotus pedestal for a Vairocana Buddha now lost. Since Boheon-sa was erected by Gaechung, a disciple of Beomil as a branch of Gulsan-sa, it is fair to assume that the Vairocana image at Boheon-sa might have been closely related to the Buddhist images at Gulsan-sa in its style and iconography. The stone seated Bodhisattva from Hansong temple-site(寒松寺址) displays a benign face, exquisite necklace, and exotic iconography in its hand gesture and high cylindrical crown. The stone seated Buddha at Cheonghak-sa(靑鶴寺), brought from a temple-site where fragments of roof-tiles with the inscription of Heukam-sa(黑岩寺) were discovered, displays the late Silla and early Goryeo period. Heukam-sa seems to have been related to Gulsan-sa or have been one of the branches of Gulsan-sa. Extant fragments of artworks at Myeongju implicate the high quality of Buddhist art of Gulsan-sa in its iconography and style as well as unique features of Gulsan-sa Seon School.

• Korean Journal of Environment and Ecology
• /
• v.33 no.3
• /
• pp.303-320
• /
• 2019

Plants interact with various biotic and abiotic environmental factors. It requires much information to understand the traits of a plant species. A shortage of information would restrict the assessment, especially in the evaluation of what kind of factors influence a plant species to face extinction. Polygonatum stenophyllum Maxim. is one of the northern plants of which Korea is the southern distribution edge. The Korean Ministry of Environment had designated it to be the endangered species until December 2015. Although it is comparatively widespread, and a large population has recently been reported, it is assessed to be vulnerable due to the low population genetic diversity. This study evaluated the current distribution of Polygonatum stenophyllum Maxim. We investigated the vegetational environment, population structures, phenology, soil environment, and self-incompatibility based on the results. Lastly, we evaluated the current threats observed in the habitats. The habitats tended to be located in the areas where the masses at the edge of the stream accumulated except for those that were located on slopes of some mountainous areas. Most of them showed a stable population structure and had re-established or recruited seedlings. Polygonatum stenophyllum Maxim. had the difference in time when the shoots appeared above the ground depending on the depth of the rhizome located in the underground. In particular, the seedlings and juveniles had their rhizome located shallow in the soil. Visits by pollinator insects and success in pollination were crucial factors for bearing of fruits by Polygonatum stenophyllum Maxim. The threats observed in the habitat of Polygonatum stenophyllum Maxim. included the expansion of cultivated land, construction of new buildings, and construction of river banks and roads. Despite such observed risk factors, it is not likely that there would be rapid population reduction or extinction because of its widespread distribution with the total population of more than 2.7 million individuals and the new populations established by the re-colonization.

• Phonetics and Speech Sciences
• /
• v.11 no.3
• /
• pp.9-21
• /
• 2019

This study aimed to compare the prosodic characteristics of North Korean defectors and South Koreans in three types of sentences (declarative, interrogative, and negative) in two reading tasks (short and dialogue) through acoustic analysis and auditory-perceptual evaluation. In addition, this study examined the relationship between the auditory-perceptual evaluation scores and self-assessment questionnaires on intonation for North Korean defectors. The participants were 15 North Korean defectors and 15 Korean speakers with standard Seoul accents. For statistical analysis, three-way mixed ANOVA and multivariate analysis were performed within the three types of sentences in the reading tasks through acoustic analysis and the Mann-Whitney U Test for auditory-perceptual evaluation. Pearson's product-moment correlation coefficients were also used to identify the correlations between the results of the self-assessment questionnaire on intonation and the auditory-perceptual evaluation. The North Korean defectors were found to have a significantly lower pitch range and auditory-perceptual evaluation score than South Koreans in reading tasks. Moreover, there was a significant correlation between their auditory-perceptual evaluations and self-assessment questionnaires on intonation. The study findings suggest that North Korean defectors, who face many challenges with intonation, showed a tendency to think that their intonation differed from the standard Korean intonation and showed better auditory evaluation results for interrogative sentences.

• Journal of Intelligence and Information Systems
• /
• v.25 no.3
• /
• pp.89-115
• /
• 2019

This study was conducted to present the stylistic differences between Arthur Conan Doyle and Agatha Christie, famous as writers of classical mystery novels, through data analysis, and further to present the analytical methodology of the study of style based on text mining. The reason why we chose mystery novels for our research is because the unique devices that exist in classical mystery novels have strong stylistic characteristics, and furthermore, by choosing Arthur Conan Doyle and Agatha Christie, who are also famous to the general reader, as subjects of analysis, so that people who are unfamiliar with the research can be familiar with them. The primary objective of this study is to identify how the differences exist within the text and to interpret the effects of these differences on the reader. Accordingly, in addition to events and characters, which are key elements of mystery novels, the writer's grammatical style of writing was defined in style and attempted to analyze it. Two series and four books were selected by each writer, and the text was divided into sentences to secure data. After measuring and granting the emotional score according to each sentence, the emotions of the page progress were visualized as a graph, and the trend of the event progress in the novel was identified under eight themes by applying Topic modeling according to the page. By organizing co-occurrence matrices and performing network analysis, we were able to visually see changes in relationships between people as events progressed. In addition, the entire sentence was divided into a grammatical system based on a total of six types of writing style to identify differences between writers and between works. This enabled us to identify not only the general grammatical writing style of the author, but also the inherent stylistic characteristics in their unconsciousness, and to interpret the effects of these characteristics on the reader. This series of research processes can help to understand the context of the entire text based on a defined understanding of the style, and furthermore, by integrating previously individually conducted stylistic studies. This prior understanding can also contribute to discovering and clarifying the existence of text in unstructured data, including online text. This could help enable more accurate recognition of emotions and delivery of commands on an interactive artificial intelligence platform that currently converts voice into natural language. In the face of increasing attempts to analyze online texts, including New Media, in many ways and discover social phenomena and managerial values, it is expected to contribute to more meaningful online text analysis and semantic interpretation through the links to these studies. However, the fact that the analysis data used in this study are two or four books by author can be considered as a limitation in that the data analysis was not attempted in sufficient quantities. The application of the writing characteristics applied to the Korean text even though it was an English text also could be limitation. The more diverse stylistic characteristics were limited to six, and the less likely interpretation was also considered as a limitation. In addition, it is also regrettable that the research was conducted by analyzing classical mystery novels rather than text that is commonly used today, and that various classical mystery novel writers were not compared. Subsequent research will attempt to increase the diversity of interpretations by taking into account a wider variety of grammatical systems and stylistic structures and will also be applied to the current frequently used online text analysis to assess the potential for interpretation. It is expected that this will enable the interpretation and definition of the specific structure of the style and that various usability can be considered.