• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.49-53
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• 2012

X-linked adrenoleukodystrophy (ALD) is a rare inherited metabolic disease which results in impaired peroxisomal ${\beta}$-oxidation and the accumulation of very long chain fatty acids (VLCFA) in the adrenal cortex, the myelin of the central nervous system, and the testes. X-linked ALD is caused by mutations in the ABCD1 gene encoding an ATP-binding cassette transporter superfamily located in the peroxisomal membrane. This disease is characterized by a variety of phenotypes. The classic childhood cerebral ALD is a rapidly progressive demyelinating condition affecting the cerebral white matter before the age of 10 years in boys. We report the case of a 8-year-old with childhood cerebral X-linked ALD who developed inattention, hyperactivity, motor incoordination and hemiparesis. We diagnosed ALD with elevated plasma very long chain fatty acid level and diffuse high signal intensity lesions in both parieto-occipital white matter and cerebellar white matter in brain MRI. We identified a novel c.983delT (p.Met329CysfsX7) mutation of the ABCD1 gene. There is no correlation between X-ALD phenotype and mutations in the ABCD1 gene. Further studies for searching additional non-genetic factor which determine the phenotypic variation will be needed.

• Proceedings of the Korean Institute of Surface Engineering Conference
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• 2018.06a
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• pp.39-39
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• 2018

최근 플라즈마 의학이 발달하면서 제트, 펜, 니들, 토치 등의 다양한 형태의 플라즈마 발생기가 개발되었으며 내부의 가스라인으로 가스의 종류, 유속, 조성 등을 조절하여 생물학적 효과를 극대화 할 수 있고 안정적으로 플라즈마 방전상태를 유지할 수 있으나 처리 면적이 좁아 실제 생물학적 시스템 (세포, 조직, 그리고 박테리아) 적용에 있어 한계점이 존재한다. 이러한 한계점을 극복하기 위해서 유전체격벽방전 (Dielectric barrier discharge, DBD) 방식을 이용한 플렉서블 활성종 발생기를 제작하고 생물학적 시스템에 적용하기 위한 방전 특성 평가를 진행하였으며, 간단한 in vitro 모델인 한천 젤을 이용하여 플라즈마 처리에 따른 전달물질의 침투거리를 확인하였다. 플라즈마 방전 시 생성되는 수산화기 [OH], 과산화수소 [$H_2O_2$], 초산소음이온 [$O_2{^-}$], 오존 [$O_3$], 그리고 산화질소 [$NO_x$]와 같은 산소 및 질소 활성종 (Reactive oxygen and nitrogen species, RONS)은 세포벽 또는 세포막의 주요 구성성분인 다당류와 인지질의 과산화 반응을 통해 구조를 변화시키고 생물학적 시스템의 표면의 pH를 낮춘다. 이러한 RONS의 작용은 살균, 소독 뿐만 아니라 약물의 침투를 돕는다. 일반적으로 한천 겔은 농도에 따라 생체 내 뇌 조직과 물리적 특성이 유사하고, 미생물학 기질, 방사선학 연구를 위한 조직모델로 사용되기 때문에 본 연구에서는 3%와 5% 농도의 한천 젤을 사용하여 침투거리를 확인하였다. 한천 젤은 $2.5{\times}2.5{\times}2.5cm^3$의 크기로 준비되었고 대조군으로 염료가 포함된 에멀젼을 0.01 g 도포하고, 실온에서 30분간 보존 후 단면을 잘라 현미경으로 침투거리를 확인하였으며, 실험군으로 플라즈마 전처리 후 에멀젼을 도포한 시표와 에멀젼 도포 후 플라즈마 처리한 시료에 대해 에멀젼 침투거리의 변화를 확인하였다. 본 연구의 플렉서블 활성종 발생기는 인체에 부착하여 사용되기 때문에 화상, 홍반을 유발을 방지하기위해 $40^{\circ}C$의 온도에서 실험을 진행하였고 이때에 플라즈마 방전조건은 $0.065W/cm^2$ 수준의 전력을 소모하는 1.7 kV의 전압, 16 kHz의 주파수로 10분간 처리하였다. 그 결과 3%의 한천 젤의 경우 침투거리 0.779 mm에서 0.826 mm, 0.942 mm까지 침투거리가 증가하였고 5%의 한천 젤의 경우 0.859 mm, 0.949 mm로 증가하였다. 이러한 침투거리 증가는 젤 표면의 다당류를 구성하고 있는 단량체가 플라즈마 처리시 화확적 구조가 끊어져 결론적으로 약물 침투가 증가된 것으로 판단된다.

• Applied Microscopy
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• v.42 no.2
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• pp.87-93
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• 2012

In this study, fine structures of silica, titanium dioxide, talc and kaolin used in decorative cosmetics and the mixture extracted from BB cream cosmetics were observed by scanning electron microscopy. Kaolin had plate like shape structures of polygon with smooth surface and edge of kaolin had a relatively smooth appearance in comparison with talc. Also, thickness of each layer was estimated to about $0.1{\mu}M$ in the lump formed in stratum of several layers. Talc was observed by lumps shape phase of layering very thin flake. Boundary of thin flake was sharp or angular phase and thickness of flake was approximately 600 nm in diameter. When comparing the thickness of kaolin and talc, we was confirmed that kaolin was thicker than talc. Diameter of titanium dioxide was estimated to 0.2~0.3 ${\mu}M$ and surface of particle was a soft cubic form. Silica was confirmed that variety of size from 200 nm to $15{\mu}M$ of globular shape was measured. From the observation of inorganic pigments, silica was homogeneous dispersed in the BB cream cosmetics and among each other was filled with relatively small size like talc, kaolin, titanium dioxide and iron oxide. In conclusion, we suggest that silica at decorative cosmetics were formed in cosmetic coat at the skin as the minimum thickness.

• Journal of Life Science
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• v.28 no.11
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• pp.1268-1276
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• 2018

The cytidine analog decitabine (DEC) acts as a nucleic acid synthesis inhibitor, whereas ammonium pyrrolidine dithiocarbamate (PDTC) is an inhibitor of nuclear factor-${\kappa}B$. The aim of this study was to investigate the possible synergistic inhibitory effect of these two inhibitors on proliferation of human gastric cancer AGS cells. The inhibitory effect of PDTC on AGS cell proliferation was significantly increased by DEC in a concentration-dependent manner, and this inhibition was associated with cell cycle arrest at the G2/M phase and the induction of apoptosis. This induction of apoptosis by the co-treatment with PDTC and DEC was related to the induction of DNA damage, as assessed by H2AX phosphorylation. Further studies demonstrated that co-treatment with PDTC and DEC induced the disruption of mitochondrial membrane potential, increased the generation of intracellular reactive oxygen species (ROS) and the expression of pro-apoptotic Bax, and down-regulated the expression of anti-apoptotic Bcl-2, ultimately resulting in the release of cytochrome c from the mitochondria into the cytoplasm. Co-treatment with PDTC and DEC also activated caspase-8 and caspase-9, which are representative caspases of the extrinsic and intrinsic apoptosis pathways. Co-treatment also activated caspase-3, which was accompanied by proteolytic degradation of poly (ADP-ribose) polymerase. Taken together, these data clearly indicated that co-treatment with PDTC and DEC suppressed the proliferation of AGS cells by increasing DNA damage and activating the ROS-mediated extrinsic and intrinsic apoptosis pathways.

• Journal of the Korean Society of Food Science and Nutrition
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• v.36 no.2
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• pp.163-173
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• 2007

Lipid peroxidation is one of the main manifestations of oxidative damage and has been found to play an important role in the toxicity and carcinogenesis of many carcinogens. This study was carried out to determine the effects of vitamin C on lipid contents and fatty acid compositions of serum and liver in male rats treated with radiation or aflatoxin $B_1\;(AFB_1)$. Six week-old male Sprague-Dawley rats were randomly divided into 7 groups; control group, radiation exposed group, $AFB_1$ treated group, X-ray and $AFB_1$ co-treated group. Three groups, except control group, were each further divided into vitamin C administered group and not administered groups. For this study, vitamin C was injected with 10 mg/kg of body weight by intraperitoneal injection and 1 hr later, 0.4 mg/kg of $AFB_1$ was injected by the same method. These administrations were repeated every 3 days over a period of 15 days. Only one time, X-ray was irradiated on whole liver with 1,500 cGy. Then vitamin C and AFB1 were administered by the same level and same method described above. On the 16th day of treatments, the animals were sacrificed. From the analysis of the serum lipid patterns, significant decrease (p<0.01) in triglyceride (TG) and total cholesterol levels were observed in X-ray and $AFB_1$ co treated group administered with vitamin C (group 7). In liver lipids, the levels of free cholesterol and total cholesterol were also decreased in X-ray and $AFB_1$ co treated group administered with vitamin C (group 7). The levels of serum free cholesterol and hepatic TG were not significantly different among all groups according to vitamin C administrations. The high density lipoprotein (HDL)-cholesterol level of serum was significantly (p<0.01) increased while the low density lipoprotein (LDL)-cholesterol level was decreased in X-ray and $AFB_1$ co treated group administered with vitamin C (group 7). In the phospholipid fatty-acid compositions of serum and liver tissue, group 3, 5 and 7 showed an increase in polyunsaturated fatty-acid (PUFA) but a decrease in saturated fatty acid (SFA) when compared to the control group. The composition ratio of fatty acid varied according to vitamin C administration. These results suggested that vitamin C has partly suppressive effects on lipid contents and fatty acid composition of serum and liver in rats treated by radiation and $AFB_1$.

• Korean Journal of Food Science and Technology
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• v.35 no.3
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• pp.510-518
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• 2003

Protective effects of natural components including genistein (4',5,7-trihydroxyisoflavone) from Glycine max MERRILL on the rose-bengal sensitized photohemolysis of human erythrocytes were investigated. Genistein $(10{\sim}100\;{\mu}m)$ suppressed photohemolysis in a concentration-dependent manner, and was more effective than the lipid peroxidation chain blocker, ${\alpha}$-tocopherol (Vit. E). Glycoside of genistein, genistin, the water-soluble antioxidant, L-ascorbate, and the iron chelator, myo-inositol hexaphosphoric acid dodecasodium salt (sodium phytate) did not exhibit protective effect against photohemolysis. L-Ascorbate and sodium phytate stimulated photohemolysis at high concentration $(500\;{\mu}m)$. ${\alpha}$-Carotene 3,3'-diol (lutein), a singlet oxygen $(^1O_2)$ quencher, exhibited pronounced protective effect, an indication that $^1O_2$ is important in photohemolysis sensitized by rose-bengal. Reactive oxygen scavenging activities $(OSC_{50})$ of natural antioxidants including genistein on reactive oxygen species (ROS) generated in $Fe^{3+}-EDTA/H_2O_2$ system using the luminol-dependent chemiluminescence assay were in the order of sodium phytate > L-ascorbate > ${\alpha}$-tocopherol > genistein > genistin. $OSC_{50}$ value of genistein, genistin, ${\alpha}$-tocopherol, L-ascorbate, and sodium phytate were 41.0, 109.0, 9.0, 5.2, and $0.56{\mu}m$ respectively. The order of free radical (1,1-diphenyl-2-picrylhydrazyl, DPPH) scavenging activity $(FSC_{50})$ was L-ascorbate > ${\alpha}$-tocopherol > genistein > genistin. These results indicate that genistein can function as an antioxidant in biological systems, particularly skin exposed to solar UV radiation by scavenging $^1O_2$ and other ROS, and to protect cellular membranes against ROS.

• Food Science and Preservation
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• v.18 no.3
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• pp.374-382
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• 2011

Aflatoxin ($AFB_1$) is a potent hepatotoxic and hepatocarcinogenic mycotoxin in humans. It is also well-known to be accumulated in animal tissues via various metabolic pathways. This study was conducted to determine the effects of vitamin C on the residual $AFB_1$ in rat sera that were treated with radiation and $AFB_1$. Six week-old male Sprague-Dawley rats were randomly divided into five groups: a control group, $AFB_1$-treated group, the group treated with $AFB_1$ and vitamin C, the group treated with X-ray and AFB1, and the group treated with X-ray and $AFB_1$ with vitamin C. On the first day of the experiment, only one dose of X-rays was exposed to the entire liver at 1,500 cGy. Next, vitamin C was injected at 10 mg/kg body weight via intraperitoneal injection, followed 1 hr later by the administration of 0.4 mg/kg of $AFB_1$ via intraperitoneal injection. These treatments were then administered every three days over a period of 15 days. On the 16th day of treatments, the animals were sacrificed. The contents of $AFB_1$ in rat sera were determined via indirect competitive ELISA and HPLC method. In the quantitative analysis of $AFB_1$ in rat sera via ELISA, $5.17{\pm}0.34$ ng/mL of $AFB_1$ was detected in the $AFB_1$-treated groups, but the amount more significantly decreased to $3.23{\pm}0.76$ ng/mL in the groups treated with $AFB_1$ and vitamin C (p<0.01) than in the $AFB_1$-treated groups. The $AFB_1$ contents of the rat sera of the groups treated with X-ray and $AFB_1$ did not significantly decreased with the administration of vitamin C. The $AFB_1$ content of the rat sera that was analyzed via HPLC showed a tendency similar to that of the content that was analyzed via ELISA. With regard to these data, vitamin C was very effective in reducing $AFB_1$ residue in rat sera.

• Journal of the Korean Electrochemical Society
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• v.4 no.2
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• pp.58-64
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• 2001

YSZ ($8mol\%$ yttria-stabilized zirconia)-modified LSM $(La_{0.85}Sr_{0.15}MnO_3)$ composite cathodes were fabricated by formation of YSZ film on triple phase boundary (TPB) of LSM/YSZ/gas. The YSZ coating film greatly enlarged electrochemical reaction sites from the increase of additional TPB. The composite cathode was formed on thin YSZ electrolyte (about 30 Um thickness) supported on an anode and then I-V characterization and AC impedance analyses were performed at temperature between $700^{\circ}C\;and\;800^{\circ}C$. As results of the impedance analysis on the cell at $800^{\circ}C$ with humidified hydrogen as the fuel and air as the oxidant, R1 around the frequency of 1000 Hz represents the anode Polarization. R2 around the frequency of 100Hz indicates the cathode polarization, and R3 below the frequency of 10 Hz is the resistance of gas phase diffusion through the anode. The cell with the composite cathode produced power density of $0.55\;W/cm^2\;and\;1W/cm^2$ at air and oxygen atmosphere, respectively. The I-V curve could be divided into two parts showing distinctive behavior. At low current density region (part I) the performance decreased steeply and at high current density region (part II) the performance decreased gradually. At the part I the performance decrease was especially resulted from the large cathode polarization, while at the part H the performance decrease related to the electrolyte polarization.

• Journal of Life Science
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• v.13 no.3
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• pp.333-342
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• 2003

The pathogenic effort of high glucose, possibly in concert with fatty acids, is mediated to vascular complications of diabetes via increased production of reactive oxygen species(ROS), reactive nitrogen species(RNS), and subsequent oxidative stress. This study was carried out to investigate the suppressive effect of buchu(Allium tuberosum) on oxidative stress in streptozotocin(STZ)-induced diabetes in Sprague Dawley male rats. The effect of buchu supplementation (10%) on lipid peroxidation, and antioxidative defense system in blood and liver was compared among normal rats fed basal diet(normal) and diabetic rats fed basal diet(DM-control) or 10% buchu-supplemented diet(DM-buchu). Diabetes was experimentally induced by the femoral muscle injection of 50 mg STZ per kg of body weight. Animals were sacrificed after 4 wks of experimental diets feeding. The induction of diabetes by STZ elevated the level of lipid peroxidation represented by thiobarbituric acid-reactive substances(TBARS) and conjugated dienes in plasma, LDL, liver, and erythrocytes. 10% buchu-supplemented diet significantly reduced the levels of conjugated dienes in erythrocytes(p<0.05) and lowered TBARS in liver and LDL to the levels of control. Induction of diabetes by STZ elevated Mn-superoxide dismutase(Mn-SOD) activity and lowered activities of glutathionine reductase(GSH-red) and glutathionine peroxidase(GSH-px). Catalase activity was not affected by the induction of diabetes by STZ. However, buchu supplementation to diabetic rats significantly elevated catalase activity(p<0.05) and slightly elevated GSH-px and GSH-red activities in liver. GSH levels of blood and liver were lowered or not changed by induction of diabetes by STZ, respectively, while buchu supplementation to diabetic rats significantly elevated hepatic GSH level (p<0.05). In conclusion, it can be concluded that buchu might be a food source to attenuate oxidative stress in diabetic patients by inhibiting lipid peroxidation, by increasing hepatic GSH level, and by inducing anti-oxidative enzyme systems.

• (The) Research of the performance art and culture
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• no.18
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• pp.413-444
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• 2009

This paper is an analysis of stage and performance elements for the ceremonial procedures and dance featured in bongsudang-jinchan, a feast celebrating the 60th birthday of Hyegyeong-gung Hongssi (Crown Princess Hong of Hyegyeonggung), the mother of King Jeongjo, which took place in Hwaseong haegung palace in 1795. The primary sources used are data on bongsudangjinchan recorded in Wonhaeng-eulmyojeongriuigwe, Jeongjo-sillok, Hongjae-jeonseo, pictorial sources such as Folding Screens of Hwaseong-neunghaeng and Hwaseong Ilgi, which is a journal in Korean by Yi Hui-pyeong. A court ceremony to offer music, dance, flowers, and food, as well as wine and poetry which express the sentiments of chung (fidelity) and hyo (filial piety) was considered a national ceremony and has constituted a unique musical culture during the 500 years of Joseon dynasty. However, after the fall of Joseon dynasty, ceremonial music and dance, which have been organically linked within the overall symbolic system of ye (courtesy), became scattered to become independent 'pieces.' As a result, all of their philosophy, principles, and the time-space interpretation of court music and dance became greatly reduced, leaving only the artistic expression and formal structure of the music and dance to become emphasized. Since the 1990s, there has been many research and events aiming to re-create the court ceremonial tradition, resulting in the increase of the related performance activities. This is especially true with bongsudang-jinchan, which is now being performed on modern stage in various forms. However there are still many problems to be solved, such as the issue of re-creating and restoring the original, and the question of artistic value found in the traditional pieces. Until now, much focus has been paid to the outer re-construction of uiju document as recorded in Wonhaeng-eulmyo-jeongriuigwe. On the other hand, there lacked an in-depth study which analyzes the stage situation and performance elements. Therefore in this paper, after focusing on the stage structure and performance elements, it is concluded that bongsudang-jinchan, the only court feast to be held in Haeng gung, not only consists of the fundamental aspects of court performance principle as 'governing through ye and ak (music),' but also served as an important occasion to bring together the sovereign and the subject. Bongsudang-jinchan had features of both naeyeon (feast for ladies) and oeyeon (feast for gentlemen). It minimized the use of screens and allowed every guest to enjoy food, music, and dance together, but provided a separate tent for foreign guests, maintaining the ideal balance between equality and distinction among different gender and social status. A screen symbolizing the venue for the feast is placed for all of the government officials. The king then pronounces the beginning of the banquet in which the ideal of gunsin-dongyeon (king and officials dining together) is realized. This indicates that bongsudang-jinchan, compared to other court ceremonies that emphasize the principle of yeak (courtesy and music), focuses more on the spirit of harmony and rapprochement. The king played a more active role in bongsudang-jinchan than in any other royal feasts. Examples as recorded in uiju documents are; Jeongjo's conversation with his retainers after the 7th wine, king's bestowing of food and flowers to the officials, writing his own majesty's poems with regard to the festival, and asking the retainers to write replying poems. All these played an important part in making the occasion more rich, extensive, and meaningful. Moreover, as analysis of the structure of orchestral music and court dance featured in bongsudang-jinchan shows, it was like any other court banquet in that it employed minimal use of extravagance in movements and conversation. However, the colors and tonal texture used in the music and dance were more brilliant in this case. Compared to other banquets that took place before king Jeongjo, the dance style was more diverse, which included some of the latest additions. There were past performances arranged anew. Noteworthy are; the incorporation of "Seonyurak (Boat Dance)" and "Geommu (Sword Dance)," traditionally used for local officials and civilians feast, to suit the court taste; and the use of saenghwang (mouth organ), which was a rising instrument in pungnyubang (literati's private salon), for "Hakmu (Cranes Dance)." This especially indicates the nature of the 'open structure' pursued by the court banquets at the time, which strove to break away from the traditional rules and customs and accept something new.