• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Journal of Environmental Impact Assessment
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• v.24 no.6
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• pp.561-577
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• 2015

The heavy metal accumulations of avian eggshells were studied in order to test a feral pigeon (Columba livia)'s eggshell as an indicator for the environmental monitoring of pollutants. The reviews on the eggs of the different 19 avian species showed that it is the eggshell rather than the egg content that can better reflect the heavy metals in the environment; in most cases the CVs (coefficients of variations) of the heavy metal concentrations in the eggshells were higher than those in the egg contents. This can indicate that the heavy metal accumulations are homeostatically controlled in the egg contents, but the accumulations in the eggshells are varied according to the environmental conditions. To test the reviews, the feral pigeon eggs from the two different sites, one representing urban and the other rural environment, were analyzed for lead (Pb) and cadmium (Cd). The result showed that the eggshells of the urban pigeons (Hangang) had the higher metal concentrations than those of the rural pigeons (Hampyeong). The same difference can also be found in the internal organs (liver, bone) and blood. However, the analyses of the egg contents between the two sites did not reveal the differences. In other words, the result suggests that the feral pigeons, like the other avian species, are able to control the heavy metals into the egg contents homeostatically. Therefore, it is more useful to use the feral pigeon eggshell rather than the egg content in case of monitoring heavy metals in different habitats.

• Korean Journal of Environmental Agriculture
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• v.31 no.4
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• pp.300-307
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• 2012

BACKGROUND: The Transfer Factor (TF) of heavy metals from soil to plant is important, because TF is an indicator of heavy metal in soils and a factor that quantifies bioavailability of heavy metals to agricultural products. This study was conducted to investigate the transfer ability of Arsenic (As), Cadmium (Cd), and Lead (Pb) from soil to agricultural products. METHODS AND RESULTS: We investigated heavy metals (As, Cd and Pb) concentrations in 9 agricultural products (rice, barely, corn, pulse, lettuce, pumpkin, apple, pear, tangerin) and soil. TF of agricultural products was evaluated based on total and HCl-extractable soil concentration of As, Cd, and Pb. Regression analysis was used to predict the relationship of total and HCl-extractable concentration with agricultural product contents of As, Cd, and Pb. The result showed that TF was investigated average 0.006~0.309 (As), 0.002~6.185 (Cd), 0.003~0.602 (Pb). The mean TF value was the highest as rice 0.309 in As, lettuce 6.185, pear 0.717, rice 0.308 in Cd, lettuce 0.602, pumpkin 0.536 in Pb which were dependent on the vegetable species and cereal is showed higher than fruit-vegetables in As. CONCLUSION(S): Soil HCl-extractable concentration of As, Cd, and Pb had the larger effects on thier contents in agricultural products than total soil concentrations. We suggests that TF are served as influential factor on the prediction of uptake. Further study for uptake and accumulation mechanism of toxic metals by agricultural products will be required to assess the human health risk and need TF of more agricultural products.

• Journal of Life Science
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• v.27 no.5
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• pp.509-516
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• 2017

Perilla frutescens (L.) Britton var. sprouts (PFS) is a plant of the labiatae family. The purpose of this work was to assess the preventive effects of PFS ethanolic extracts (PFSEs) on cytokine-induced ${\beta}$-cell damage. Cytokines, which are released by the infiltration of inflammatory cells around the pancreatic islets, are involved in the pathogenesis of type 1 diabetes mellitus. The combination of interleukin-$1{\beta}$ (IL-1), interferon-${\gamma}$ (IFN-${\gamma}$), and tumor necrosis factor-${\alpha}$ (TNF-${\alpha}$) induced formation of reactive oxygen species (ROS). Accumulation of intracellular ROS led to ${\beta}$-cell dysfunction and apoptosis. PFSEs possess antioxidant activity and thus lead to downregulation of ROS generation. Cytokines decrease cell viability, stimulate the expression of inducible nitric oxide synthase (iNOS) and cyclooxygenase-2 (COX-2), and induce the production of nitric oxide (NO). PFSEs prevented cytokine-induced cell viability in a dose-dependent manner. Incubation with PFSE resulted in significant reduction in cytokine-induced NO production that correlated with reduced levels of the iNOS and COX-2 protein expression. Furthermore, PFSE significantly decreased the activation of nuclear factor ${\kappa}B$ (NF-${\kappa}B$) by inhibition of $I{\kappa}B{\alpha}$ phosphorylation in RINm5F cells. In summary, our results suggest that the protective effects of PFSE might serve to counteract cytokine-induced ${\beta}$-cell destruction. Findings indicate that consumption of Perilla frutescens (L.) Britton var. sprouts alleviates hyperglycemia-mediated oxidative stress and pro-inflammatory cytokine-induced ${\beta}$-cell damage and thus has beneficial anti-diabetic effects.

• Journal of the Korean Society of Marine Environment & Safety
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• v.16 no.2
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• pp.141-151
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• 2010

Harmful algal blooms (HABs), commonly known as red tides, are aquatic phenomena caused by the rapid growth and accumulation of certain microalgae, which can lead to marked discoloration of surface waters, and severe impacts on public health, commerce, and the environment. In South Korea, the red tides have been a serious and recurrent problem, especially along the south coast. Plenty of yellow loess was used to control an outbreak of the red tides for 15 years from 1996 until now. Yellow loess was almost sprayed in the vicinity of a large fish farming industry. In this research, the distribution characteristics and density distribution of zooplankton were investigated in autumn (Oct. 2008) and spring (Apr. 2009) using volume backscattering strength (SV) calculated by the zooplankton collected with north pacific standard (NORPAC) net and the echo intensity measured with ADCP at stations on the study area in the spraying ocean of yellow loess (SOYL), and the non-spraying ocean of yellow ocean (NOYL) by the red tide generating every year. The species number and the individuals per unit volume of the zooplankton collected in NOYL was high and it which was collected in SOYL was low. As a result of comparing the volume backscattering strength ($SV_c$) calculated by species and length of the zooplankton collected with NORPAC net with the volume backscattering strength ($SV_m$) calculated by the echo intensity measured with ADCP at stations on the study area, although $SV_c$ and $SV_m$ of NOYL were generally in agreement, $SV_m$ of SOYL was higher than $SV_c$ 4.3dB, i.e. ADCP is greatly influenced by suspended solid in SOYL. The horizontal distribution map of $SV_m$ at the study area in autumn (Oct. 2008) and spring (Apr. 2009) was drawn. $SV_m$ of SOYL is higher than NOYL and autumn is higher than spring. $SV_m$ can suppress the overestimate or underestimate of $SV_c$.

• Radiation Oncology Journal
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• v.24 no.1
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• pp.58-66
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• 2006

Purpose: Renal irradiation can lead to the development of radiation nephropathy, and this is characterized by the accumulation of extracellular matrix and final fibrosis. To determine the possible role of the glomerular epithelial cell, the radiation-induced changes in the expression of its genes associated with the extracellular matrix were analyzed. Materials and Methods: Rat glomerular epithelial cells (GEpC) were irradiated with a single dose of 0, 2, 5, 10 and 20 Gy with using 6 MV LINAC (Siemens, USA), and the samples were collected 6, 24, 48 and 72 hours post-irradiation, respectively. Northern blotting, western blotting and zymography were used to measure the expression level of fibronectin (Fn), plasminogen activator inhibitor-1 (Pai-1), matrix metalloproteinases-2, 9 (MMP-2, 9), tissue inhibitor of metalloproteinase-2 (TIMP-2), tissue-type plasminogen activator (t-PA) and urokinase-type plasminogen activator (u-PA). Results: Irradiation with a single dose of 10 Gy resulted in a significant increase in Fn mRNA since 24 hours post-irradiation, and a single dose of 5 and 10 Gy significantly increased the Fn immunoreactive protein measured 48 hours post-irradiation. An increase in Pai-1 mRNA and protein was also observed and especially, a single dose of 10 Gy significantly increased the mRNA measured 24 and 48 hours post-irradiation. The active MMP-2 measured 24 hours post-irradiation slightly increased in a dose dependent manner, but this increase did not reach statistical significance. The levels of MMP-9, TIMP-2, t-PA and u-PA appeared unaltered after irradiation. Conclusion: Irradiation of the glomerular epithelial cells altered the expression of genes associated with the extracellular matrix, implying that the glomerular epithelial cell may be involved in the development of radiation nephropathy.

• The Korean Journal of Archival Studies
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• no.8
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• pp.137-180
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• 2003

The term 'Business Archives' is not familiar with us in our society. Some cases can be found that materials are collected for publishing the history of a firm on commemoration of some decades of its foundation. However, the appropriate management of these collected materials doesn't seem to be followed in most of companies. The Records and archives management is inevitable in order to maximize the utility of Information and knowledge in the business world. The interest in records management has been grown, especially in the fields of business management and information technology. However, the importance of business archives hasn't been conceived yet. And also no attention has been paid to the business archives as social resources and the responsibility of the society as a whole for their preservation. The company archives doesn't have a long history in Germany although the archives of the nation, the aristocracy, communes and churches have a long tradition. However the company archives of Krupps which was established in 1905, is regarded as the first business archives in the world, It means that Germany has taken a key role to lead the culture of business archives. This paper focuses on the process of the establishment of business archives in Germany and its characteristics. The business archives in Germany can be categorized in three types: company archives, regional business archives and branch archives. It must be noted here that each type of these was generated in the context of the accumulation of the social resources and its effective use. A company archives is established by an individual company for the preservation of and use of the archives that originated in the company. The holdings in the company archives can be used as materials for decision making of policies, reporting, advertising, training of employees etc. They function not only as sources inside the company, but also as raw sources for the scholars, contributing to the study of the social-economic history. Some archives of German companies are known as a center of research. A regional business archives manages materials which originated m commerce chambers, associations and companies in a certain region. There are 6 regional business archives in Germany. They collect business archives which aren't kept in a proper way or are under pressure of damage in the region for which they are responsible. They are also open to the public offering the sources for the study of economic history, social history like company archives, so that they also play a central role as a research center. Branch business archives appeared relatively late in Germany. The first one is established in Bochum in 1969. Its general duties and goals are almost similar with ones of other two types of archives. It has differences in two aspects. One is that the responsibility of the branch business archives covers all the country, while regional business archives collects archives in a particular region. The other is that a branch business archives collects materials from a single industry. For example, the holdings of Bochum archives are related with the mining industry. The mining industry-specialized Bochum archives is run as an organization in combination with a museum, which is called as German mine museum, so that it plays a role as a cultural center with the functions of exhibition and research. The three types of German business archives have their own functions but they are also closely related each other under the German Association of Business Archivists. They are sharing aims to preserve primary materials with historical values in the field of economy and also contribute to keeping the archives as a social resources by having feed back with the public, which leads the archives to be a center of information and research. The German case shows that business archives in a society should be preserved not only for the interest of the companies, but also for the utilities of social resources. It also shows us how business archives could be preserved as a social resource. It is expected that some studies which approach more deeply on this topic will be followed based on the considerations from the German case.

• Journal of Korean Society of Disaster and Security
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• v.16 no.4
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• pp.45-59
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• 2023

The global focus on mitigating climate change has traditionally centered on carbon dioxide, but recent attention has shifted towards methane as a crucial factor in climate change adaptation. Natural settings, particularly aquatic environments such as wetlands, reservoirs, and lakes, play a significant role as sources of greenhouse gases. The accumulation of organic contaminants on the lake and reservoir beds can lead to the microbial decomposition of sedimentary material, generating greenhouse gases, notably methane, under anaerobic conditions. The escalation of methane emissions in freshwater is attributed to the growing impact of non-point sources, alterations in water bodies for diverse purposes, and the introduction of structures such as river crossings that disrupt natural flow patterns. Furthermore, the effects of climate change, including rising water temperatures and ensuing hydrological and water quality challenges, contribute to an acceleration in methane emissions into the atmosphere. Methane emissions occur through various pathways, with ebullition fluxes-where methane bubbles are formed and released from bed sediments-recognized as a major mechanism. This study employs Biochemical Methane Potential (BMP) tests to analyze and quantify the factors influencing methane gas emissions. Methane production rates are measured under diverse conditions, including temperature, substrate type (glucose), shear velocity, and sediment properties. Additionally, numerical simulations are conducted to analyze the relationship between fluid shear stress on the sand bed and methane ebullition rates. The findings reveal that biochemical factors significantly influence methane production, whereas shear velocity primarily affects methane ebullition. Sediment properties are identified as influential factors impacting both methane production and ebullition. Overall, this study establishes empirical relationships between bubble dynamics, the Weber number, and methane emissions, presenting a formula to estimate methane ebullition flux. Future research, incorporating specific conditions such as water depth, effective shear stress beneath the sediment's tensile strength, and organic matter, is expected to contribute to the development of biogeochemical and hydro-environmental impact assessment methods suitable for in-situ applications.

• Korean Journal of Heritage: History & Science
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• v.57 no.3
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• pp.24-35
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• 2024

Recently, research using deep learning technologies such as artificial intelligence, convolutional neural networks, etc. has been actively conducted in various fields including healthcare, manufacturing, autonomous driving, and security, and is having a significant influence on society. In line with this trend, the present study attempted to apply deep learning to the classification of archaeological artifacts, specifically ancient Korean roof-end tiles. Using 100 images of roof-end tiles from each of the Goguryeo, Baekje, and Silla dynasties, for a total of 300 base images, a dataset was formed and expanded to 1,200 images using data augmentation techniques. After building a model using transfer learning from the pre-trained EfficientNetB0 model and conducting five-fold cross-validation, an average training accuracy of 98.06% and validation accuracy of 97.08% were achieved. Furthermore, when model performance was evaluated with a test dataset of 240 images, it could classify the roof-end tile images from the three dynasties with a minimum accuracy of 91%. In particular, with a learning rate of 0.0001, the model exhibited the highest performance, with accuracy of 92.92%, precision of 92.96%, recall of 92.92%, and F1 score of 92.93%. This optimal result was obtained by preventing overfitting and underfitting issues using various learning rate settings and finding the optimal hyperparameters. The study's findings confirm the potential for applying deep learning technologies to the classification of Korean archaeological materials, which is significant. Additionally, it was confirmed that the existing ImageNet dataset and parameters could be positively applied to the analysis of archaeological data. This approach could lead to the creation of various models for future archaeological database accumulation, the use of artifacts in museums, and classification and organization of artifacts.