• Journal of Genetic Medicine
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• v.4 no.1
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• pp.38-44
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• 2007

Purpose : Menkes disease is an X-linked recessively inherited disorder caused by the mutation of the ATP7A gene encoding copper-transporting P-type AT Pase. The phenotypic features are progressive neurological degeneration, mental retardation, loose skin, and vascular complications. Early diagnosis and treatment are very important for the prognosis of Menkes disease. Here, we describe nov el mutations of the ATP7A gene and prenatal diagnosis by mutation analysis. Methods : Five unrelated Korean Menkes patients were included in this study. They presented with depigmented wool-like hair, progressive neurologic deterioration, and hypotonia in infancy. Serum copper and ceruloplasmin levels w ere decreased. Brain magnetic resonance imaging revealed tortuous intracranial vessels. Mutation analysis has been carried out using cDNA from cultured skin fibroblasts or genomic DNA from peripheral leukocytes. Prenatal diagnosis was performed in two cases using chorionic villi samples or amniocytes. Results : Four novel mutations have been identified from four different families; c.3511+1G>A (p.E1099_N1171delinsMfsX 18), c.4005+5 G>A (p.V1268_R1335del), c.1870_2172del (p.S624_Q724del), and c.3352 G>A (p.G1118S). T he remaining one was previously reported (c.1933 C>T (p.V 1268_R1335del)). On prenatal DNA analysis, one w as diagnosed as normal, while the other turned out to be a female heterozygote with p.S624_Q724del mutation of the ATP7A gene. Conclusion : We identified 4 novel mutations of the ATP7A gene. Prenatal diagnosis in families at risk is critical in order to choose preventiv e options including an early treatment with copper-histidine therapy or therapeutic termination. Most mutations of the ATP7A gene were frame-shift mutations and prenatal diagnosis has been successfully carried out.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• Applied Chemistry for Engineering
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• v.19 no.3
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• pp.299-303
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• 2008

A thermally cross-linkable polymer, poly[(2,5-dimethoxy-1,4-phenylenevinylene)-alt-(1,4-phenylenevinylene)] (Cross-PPV), was synthesized by the Heck coupling reaction. In order for the polymer to be cross-linkable, 20 mol% excess divinylbenzene was added. The chemical structure of Cross-PPV and thermally crosslinked Cross-PPV were confirmed by FT-IR spectroscopy. From the FT-IR, UV-Vis, and PL spectral data, thermally crosslinked Cross-PPV was insoluble in common organic solvents. The HOMO and LUMO energy level of thermally cross-linked Cross-PPV were estimated -5.11 and -2.56 eV, respectively, which were determined by the cyclic voltammetry and UV-Vis spectroscopy. From the energy level data, one can easily notice that thermally crosslinked Cross-PPV can be used for hole injection layer effectively. Bilayer structured device (ITO/crosslinked Cross-PPV/PM-PPV/Al) was fabricated using poly(1,4-phenylenevinylene-(4-dicyanomethylene-4H-pyran)-2,6-vinylene-1,4-phenylenevinylene-2,5-bis(dodecyloxy)-1,4-phenylenevinylene (PM-PPV) as the emitting layer, which have HOMO and LUMO energy levels of -5.44 eV and -3.48 eV, respectively. The bilayered device had much enhanced the maximum efficiency (0.024 cd/A) and luminescence ($45cd/m^2$) than those of a single layer device (ITO/PM-PPV/Al, 0.003 cd/A, $3cd/m^2$). The enhanced performance originated from that fact that cross-linked Cross-PPV facilitatse the hole injection to the emissive layer and the injected hole and electron from ITO and Al are recombined in emitting layer (PM-PPV) effectively.

• Journal of the Korean Magnetics Society
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• v.17 no.3
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• pp.120-123
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• 2007

The typical double-barrier magnetic tunnel junction (DMTJ) structure examined in this paper consists of a Ta 45/Ru 9.5/IrMn 10/CoFe7/$AlO_x$/free layer/AlO/CoFe 7/IrMn 10/Ru 60 (nm). The free layer consists of an $Ni_{16}Fe_{62}Si_8B_{14}$ 7 nm, $Co_{90}Fe_{10}$ (fcc) 7 nm, or CoFe $t_1$/NiFeSiB $t_2$/CoFe $t_1$ layer in which the thicknesses $t_1$ and $t_2$ are varied. The DMTJ with an NiFeSiB-free layer had a tunneling magnetoresistance (TMR) of 28%, an area-resistance product (RA) of $86\;k{\Omega}{\mu}m^2$, a coercivity ($H_c$) of 11 Oe, and an interlayer coupling field ($H_i$) of 20 Oe. To improve the TMR ratio and RA, a DMTJ comprising an amorphous NiFeSiB layer that could partially substitute for the CoFe free layer was investigated. This hybrid DMTJ had a TMR of 30%, an RA of $68\;k{\Omega}{\mu}m^2$, and a of 11 Oe, but an increased of 37 Oe. We confirmed by atomic force microscopy and transmission electron microscopy that increased as the thickness of NiFeSiB decreased. When the amorphous NiFeSiB layer was thick, it was effective in retarding the columnar growth which usually induces a wavy interface. However, if the NiFeSiB layer was thin, the roughness was increased and became large because of the magnetostatic $N{\acute{e}}el$ coupling.

• Journal of the Korean Association of Geographic Information Studies
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• v.22 no.2
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• pp.82-96
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• 2019

This study evaluated the status of watershed health in Geum River Basin by SWAT (Soil and Water Assessment Tool) hydrology and water quality. The watershed healthiness from watershed hydrology and stream water quality was calculated using multivariate normal distribution from 0(poor) to 1(good). Before evaluation of watershed healthiness, the SWAT calibration for 11 years(2005~2015) of streamflow(Q) at 5 locations with 0.50~0.77 average Nash-Sutcliffe model efficiency and suspended solid (SS), total nitrogen(T-N), and total phosphorus(T-P) at 3 locations with 0.67~0.94, 0.59~0.79, and 0.61~0.79 determination coefficient($R^2$) respectively. For 24 years (1985~2008) the spatiotemporal change of watershed healthiness was analyzed with calibarted SWAT and 5 land use data of 1985, 1990, 1995, 2000, and 2008. The 2008 SWAT results showed that the surface runoff increased by 40.6%, soil moisture and baseflow decreased by 6.8% and 3.0% respectively compared to 1985 reference year. The stream water quality of SS, T-N, and T-P increased by 29.2%, 9.3%, and 16.7% respectively by land development and agricultural activity. Based on the 1985 year land use condition. the 2008 watershed healthiness of hydrology and stream water quality decreased from 1 to 0.94 and 0.69 respectively. The results of this study be able to detect changes in watershed environment due to human activity compared to past natural conditions.

• Journal of Korea Water Resources Association
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• v.52 no.5
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• pp.349-360
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• 2019

The purpose of this study is to evaluate the stream flow and water quality (SS, T-N, and T-P) behavior of Nakdong river basin ($23,609.3km^2$) by simulating the dam and weir operation scenarios using SWAT (Soil and Water Assessment Tool). The operation senarios are the simultaneous release for all dam and weirs (scenario 1), simultaneous release for all weirs (scenario 2), and sequential release for the weirs with one month interval from upstream weirs (scenario 3). Before evaluation, the SWAT was calibrated and validated using 11 years (2005-2015) daily multi-purpose dam inflow at 5 locations (ADD, IHD, HCD, MKD, and MYD), multi-function weir inflow at 7 locations (SHW, GMW, CGW, GJW, DSW, HCW, and HAW), and monthly water quality monitoring data at 6 locations (AD-4, SJ-2, EG, HC, MK-4, and MG). For the two dam inflow and dam storage, the Nash-Sutcliffe efficiency (NSE) was 0.56~0.79, and the coefficient of determination ($R^2$) was 0.68~0.90. For water quality, the $R^2$ of SS, T-N, and T-P was 0.64~0.79, 0.51~0.74, and 0.53~0.72 respectively. For the three scenarios of dam and weir release combination suggested by the ministry of environment, the scenario 1 and 3 operations were improved the stream water quality (for T-N and T-P) within the 3 months since the time of release, but it showed the negative effect for 3 months after compared to scenario 2.

• KIPS Transactions on Software and Data Engineering
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• v.9 no.12
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• pp.431-438
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• 2020

In this paper, the method to extract lip contour by multiple threshold is proposed. Spyridonos et. el. proposed a method to extract lip contour. First step is get Q image from transform of RGB into YIQ. Second step is to find lip corner points by change point detection and split Q image into upper and lower part by corner points. The candidate lip contour can be obtained by apply threshold to Q image. From the candidate contour, feature variance is calculated and the contour with maximum variance is adopted as final contour. The feature variance 'D' is based on the absolute difference near the contour points. The conventional method has 3 problems. The first one is related to lip corner point. Calculation of variance depends on much skin pixels and therefore the accuracy decreases and have effect on the split for Q image. Second, there is no analysis for color systems except YIQ. YIQ is a good however, other color systems such as HVS, CIELUV, YCrCb would be considered. Final problem is related to selection of optimal contour. In selection process, they used maximum of average feature variance for the pixels near the contour points. The maximum of variance causes reduction of extracted contour compared to ground contours. To solve the first problem, the proposed method excludes some of skin pixels and got 30% performance increase. For the second problem, HSV, CIELUV, YCrCb coordinate systems are tested and found there is no relation between the conventional method and dependency to color systems. For the final problem, maximum of total sum for the feature variance is adopted rather than the maximum of average feature variance and got 46% performance increase. By combine all the solutions, the proposed method gives 2 times in accuracy and stability than conventional method.

• Ecology and Resilient Infrastructure
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• v.8 no.2
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• pp.79-87
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• 2021

Because active interactions occur among vegetation, hydrology, and geomorphology in riparian systems, any changes in one of these factors can significantly affect the other two. In this study, we evaluated these interactions at four sites (two in Gajeong and two in Hahan) along the Seomjin-gang River that was substantially devastated by an extreme flood in 2020. We examined the relationship between the riparian vegetation and the hydraulic characteristics of the flood using remote sensing, hydraulic modeling, and field surveys combined. The evaluation results showed that the floods caused a record-breaking rise of up to 43.1 m above sea level at the Yeseong-bridge stage gauge station (zero elevation 27.4 m) located between the Gajeong and Hahan sites, with the shear stress being four times higher in Hahan than in Gajeong. Additionally, the water level during the flood was estimated to be a maximum of 1 m higher depending on the location in the presence of riparian plants. Furthermore, both sites underwent extensive biological damage due to the flood, with 78-80% loss in vegetation, with preferential damage observed in large willow species, compared to Quercus acutissima. The above findings imply that all plant species exhibit different vulnerabilities towards extreme floods and do not induce similar behavior towards events causing a disturbance. In conclusion, we developed strategies for effectively managing riparian trees by minimizing flood hazards that could inevitably cause damage.

• Journal of International Area Studies (JIAS)
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• v.15 no.1
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• pp.109-134
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• 2011

Drug-related crime has increased in spite of visible results of Uribe government's hard-line policies on drug eradication and illegally armed organizations which were pursued under U.S. support, without the accompaniment of quantity change in drug cultivation and trade. Military disputes of left-right illegally armed communities surrounding illegal crop cultivation rights were rather intensified, and the number of refugees was increased through enforced displaced people. The 2005 refugee registration committee RUPD reports that 3,316,862 people, 7.3% of total population, were refugees. In particular, the number of refugees presented a large increase rate of 624% when compared to the past year due to enforced displaced people. Main discharge areas of enforced displaced people are connected with drug crime and activities of illegally armed organizations, and are places of increased armed disputes in the process of occupied territory expansion of illegally armed communities and militia. Undiscriminated attacks were executed on farmers in the process of occupation of illegal crop cultivation sites by illegally armed organization and militia to emit enforced displaced people, who moved to border areas by crossing national borders. Enforced displaced people were restricted to certain areas before the appearance of Uribe administration. However, enforced displaced people not only presented quantitative expansion, but also showed tendency of nationwide expansion after national security policy was pursued. With the closing of the Amazon area, previously the main route of drug trade, activity base of illegally armed organizations was moved to the Pacific region, and Panama border area experienced refugee increase due to the new policy of enforced displaced people. This study aims to understand the actual condition and cause for the increase in refugees in Colombia based on border areas of Venezuela, which is the nation of highest dispersion of Columbian refugees, Panama, which has appeared as a new destination for refugees after the 90s, and Ecuador, which has experienced sudden refugee increase in 2000.

• The Journal of Korean Academy of Prosthodontics
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• v.61 no.4
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• pp.257-267
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• 2023

Purpose. The purpose of the study was to fabricate a prototype robotic simulator for dental education, to test whether it could simulate mandibular movements, and to assess the possibility of the stimulator responding to stimuli during dental practice. Materials and methods. A virtual simulator model was developed based on segmentation of the hard tissues using cone-beam computed tomography (CBCT) data. The simulator frame was 3D printed using polylactic acid (PLA) material, and dentiforms and silicone face skin were also inserted. Servo actuators were used to control the movements of the simulator, and the simulator's response to dental stimuli was created by pressure and water level sensors. A water level test was performed to determine the specific threshold of the water level sensor. The mandibular movements and mandibular range of motion of the simulator were tested through computer simulation and the actual model. Results. The prototype robotic simulator consisted of an operational unit, an upper body with an electric device, a head with a temporomandibular joint (TMJ) and dentiforms. The TMJ of the simulator was capable of driving two degrees of freedom, implementing rotational and translational movements. In the water level test, the specific threshold of the water level sensor was 10.35 ml. The mandibular range of motion of the simulator was 50 mm in both computer simulation and the actual model. Conclusion. Although further advancements are still required to improve its efficiency and stability, the upper-body prototype simulator has the potential to be useful in dental practice education.