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A Study on the Screening of the Novel Genes Associated with Lysosomal Trafficking and Mutation Detection in Fibroblasts of the Patients with Mucolipidosis type II and III (리소좀 교통 이상을 초래하는 뮤코지방증 2형과 3형 환자의 섬유아세포를 이용한 신규 유전자 탐색 및 돌연변이에 대한 연구)

  • Song, Seng Mi;Chang, Soo Hee;Paik, Kyung Hoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.5 no.1
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    • pp.65-75
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    • 2005
  • Purpose: To understand genetic differences and similarities between mucolipidosis and control. Methods: Using the fibroblast of the mucolipidosis II and control, forward and reverse subtracted libraries were constructed. Among these clones, we investigated mutations in the GNPTA (MGC4170) gene, which codes for the ${\alpha}/{\beta}$ subunits of phosphotransferase, and in the GNPTAG gene, which codes for the ${\gamma}$ subunits in 5 Korean patients with mucolipidosis type II or IIIA. Result: Several differentially expressed cDNAs were cloned and their sequences were determined. Mutation analysis of the interested gene, GNPTA was performed and we identified 7 mutations in the GNPTA gene, but none in the GNPTAG gene. The mutations in type II patients included p.Q104X(c.310C>T), p.R1189X(c.3565C>T), p.S1058X(c.3173C>G), p.W894X(c.2681G>A) and p.H1158fsX15(c.3474_3475delTA), all of which are non-sense or frame shift mutations. However, a splicing site mutation, IVS13+1G>A (c.2715+1G>A) was detected along with a non-sense or a frame shift mutation (p.R1189X or p.E858fsX3(c.2574_2575delGA)) in two mucolipidosis type IIIA patients. Conclusion: This report shows that mutations in the GNPTA gene coding for the ${\alpha}{\beta}$subunits of phosphotransferase, and not mutations in the GNPTAG gene, account for most of mutations found in Korean patients with mucolipidosis type II or IIIA.

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The Effect of Deodeok Contents on the Quality of Deodeok Wine (더덕 함량이 더덕 침출주의 품질에 미치는 영향)

  • Kwon, Dong-Jin;Choi, Shin-Yang
    • Food Science and Preservation
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    • v.14 no.4
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    • pp.414-418
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    • 2007
  • The physicochemical characteristics and sensory properties of Deodeok wine, formed by leaching of Deodeok at room temperature for 180 days, were investigated over the following range of Deodeok levels: 10, 15 and 20% (all w/v). The higher the level of Deodeok, the greater were the final values of total sugars, reducing sugars, total polyphenols, and crude saponins. The Hunter's b-value (yellowness) of Deodeok wine varied markedly with Deodeok levels, and yellowness was highest in Deodeok wine containing 20% (w/v) Deodeok. Non-volatile compounds, that form the basis of the liquor tax law, were 0.64, 1.38 and 2.11% (all w/v), respectively, at day 160. Of these values, that of 2.11% (w/v), the level of non-volatile compounds in Deodeok wine containing 20% (w/v) Deodeok, was in accord with the liquor tax law (that requires this figure to be 2.0%). Sensory evaluation showed that Deodeok wine containing 20% (w/v) Deodeok was superior to the other wines tested.

Clinical Study of Pulmonary Thromboembolism (폐혈전색전증의 임상적 연구)

  • Bak, Sang-Myeon;Lee, Sang-Hwa;Lee, Sin-Hyung;Sin, Cheol;Cho, Jae-Youn;Shim, Jae-Jeong;In, Kwang-Ho;Kang, Kyung-Ho;Yoo, Se-Hwa
    • Tuberculosis and Respiratory Diseases
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    • v.50 no.1
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    • pp.106-116
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    • 2001
  • Background : Pulmonary thromboembolism is relatively frequent and potentially fatal. However, it is commonly misdiagnosed. The incidence of pulmonary thromboembolism is not decreasing despite advances in diagnosis and effective prophylatic measures. Its potential for significant sequela necessitates a prompt diagnosis and treatment. Unfortunately, there are many difficulties and problems regarding accurate diagnosis. There is a low prevalence of deep vein thrombosis and pulmonary thromboembolism in Korea and only few reports on this subject are available. Method : The clinical features of 36 patients, who were diagnosed with pulmonary thromboembolism at the Korea University medical center, were reviewed. Results : 1) There was no significant difference in prevalence between men an women, and the mean age was 50.9 years in men 59.2 years in women. 2) The frequent causes of pulmonary thromboembolism were malignancies (22.2%), surgery (22.2%), and heart disease(8.2%). Specific causes were not identified in 33.3%. 3) The most common symptom was dyspnea(72.2%), and the most common sign was tachypnea(61.1%). 4) The EKG findings were normal in 28.6%, an S1Q3T3 pulmonale pattern in 25.7%, ST or QRS changes in others. 5) The chest X-ray findings indicated pulmonary infiltration in 37.5%, cardiomegaly in 15.6%, pleural effusion in 12.5%, and normal in 27.8%. The perfusion lung scan showed a high probability in 66.7%, and intermediate or low probability in 33.3%. 6) The pulmonary arterial pressure(PAP) in the high probability groups was 57.9mmHg with a higher mortality rate(35%). Conclusion : Pulmonary thromboembolism is not uncommon in Korea and its clinical features do not differ greatly from thase reported in the literature. When pulmonary thromboemblism of unknown causes are diagnosed, a search for an occult malignancy is recommended. Rapid diagnosis and treatment are achieved when thromboemblism is suspected.

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Effects of Ginsenosides and Their Metabolites on Voltage-dependent Ca2+ Channel Subtypes

  • Lee, Jun-Ho;Jeong, Sang Min;Kim, Jong-Hoon;Lee, Byung-Hwan;Yoon, In-Soo;Lee, Joon-Hee;Choi, Sun-Hye;Lee, Sang-Mok;Park, Yong-Sun;Lee, Jung-Ha;Kim, Sung Soo;Kim, Hyoung-Chun;Lee, Boo-Yong;Nah, Seung-Yeol
    • Molecules and Cells
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    • v.21 no.1
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    • pp.52-62
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    • 2006
  • In previous reports we demonstrated that ginsenosides, active ingredients of Panax ginseng, affect some subsets of voltage-dependent $Ca^{2+}$ channels in neuronal cells expressed in Xenopus laevis oocytes. However, the major component(s) of ginseng that affect cloned $Ca^{2+}$ channel subtypes such as ${\alpha}_{1C}$(L)-, ${\alpha}_{1B}$(N)-, ${\alpha}_{1A}$(P/Q)-, ${\alpha}_{1E}$(R)- and ${\alpha}_{1G}$(T) have not been identified. Here, we used the two-microelectrode voltage clamp technique to characterize the effects of ginsenosides and ginsenoside metabolites on $Ba^{2+}$ currents ($I_{Ba}$) in Xenopus oocytes expressing five different $Ca^{2+}$ channel subtypes. Exposure to ginseng total saponins (GTS) induced voltage-dependent, dose-dependent and reversible inhibition of the five channel subtypes, with particularly strong inhibition of the ${\alpha}_{1G}$-type. Of the various ginsenosides, $Rb_1$, Rc, Re, Rf, $Rg_1$, $Rg_3$, and $Rh_2$, ginsenoside $Rg_3$ also inhibited all five channel subtypes and ginsenoside $Rh_2$ had most effect on the ${\alpha}_{1C}$- and ${\alpha}_{1E}$-type $Ca^{2+}$ channels. Compound K (CK), a protopanaxadiol ginsenoside metabolite, strongly inhibited only the ${\alpha}_{1G}$-type of $Ca^{2+}$ channel, whereas M4, a protopanaxatriol ginsenoside metabolite, had almost no effect on any of the channels. $Rg_3$, $Rh_2$, and CK shifted the steady-state activation curves but not the inactivation curves in the depolarizing direction in the ${\alpha}_{1B}$- and ${\alpha}_{1A}$-types. These results reveal that $Rg_3$, $Rh_2$ and CK are the major inhibitors of $Ca^{2+}$ channels in Panax ginseng, and that they show some $Ca^{2+}$ channel selectivity.

A Study on Neurosis According to Experience of Sexual Abuse among Female High School Students in Taegu (대구지역 여고생들의 성(性)적 학대 경험에 관련된 신경증에 관한 연구)

  • Park, Hyung-Bae;Kim, Jin-Sung;Jang, Sang-Ruyl;Rho, Tae-Yong;Suh, Hye-Soo;Kim, Chang-Su;Lee, Kwang-Hun;Kim, Young-Uck
    • Journal of Yeungnam Medical Science
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    • v.12 no.1
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    • pp.113-123
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    • 1995
  • This study was intended to identify the neurotic traits related to sexual abuse in female high school students. The Neurosis Scale of Middlesex Hospital Questionnaire was conducted to 320 female high school students in Taegu, Korea from July 10 to July 17, 1994. The results were as follows: 1) The sexual abuse victim group showed significantly higher neurotic traits of Free floating anxiety (p<0.05), Phobic(p<0.01), Obsession(p<0.01), Somatic(p<0.05), Depression(p<0.01) than the control group. 2) The Neurotic traits of Somatic(p<0.01), Depression(p<0.01), Hysteric(p<0.01) were higher in the multiple-abused than the single-abused. 3) When the duration of being abused was concerned, subjects with more than 1 year duration had significantly higher neurotic traits of Obsession(p<0.05), Somatic(p<0.05), Depression(p<0.05) than subjects with less than 1 year duration. 4) The neurotic traits of Phobic, Somatic, Depression were significantly higher in the subjects with higher intensity of sexual abuse than with lower intensity. S) Among 68 victims, 31 girls(45%) were assaulted by strangers, 10(14%) were by relatives, 7(10.3%) were by siblings, 6(8.8%) were by neihbors. Among them, 34 girls(50%) bad counselling or psychotherapy and the people to whom they had sought for help were friends(23 girls; 68%), parents (7 girls; 11.7%) and mental health professonals (2 girls; 5.9%).

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Isotopic Determination of Food Sources of Benthic Invertebrates in Two Different Macroalgal Habitats in the Korean Coasts (동위원소 분석에 의한 동해와 남해 연안의 상이한 해조류 군락에 서식하는 저서무척추동물 먹이원 평가)

  • Kang, Chang-Keun;Choy, Eun-Jung;Song, Haeng-Seop;Park, Hyun-Je;Soe, In-Soo;Jo, Q-Tae;Lee, Kun-Seop
    • The Sea:JOURNAL OF THE KOREAN SOCIETY OF OCEANOGRAPHY
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    • v.12 no.4
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    • pp.380-389
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    • 2007
  • Stable carbon and nitrogen isotopes were analyzed in suspended particulate organic matter, macroalgae and macrobenthic invertebrates in order to determine the importance of primary organic matter sources in supporting food webs of rocky subtidal and intertidal macroalgal beds in the Korean coasts. Investigations were conducted at the inter tidal sites within Gwangyang bay, a semi-enclosed and eutrophicated bay, and the subtidal sites of the east coast, a relatively oligotrophic and open environment, in May and June 2005. Water-column suspension feeders showed more negative $\delta^{13}C$ values than those of the other feeding guilds, indicating trophic linkage with phytoplankton and thereby association with pelagic food chains. In contrast, animals of the other feeding guilds, including interface suspension feeders, herbivores, deposit feeders, omnivores and predators, displayed relatively less negative $\delta^{13}C$ values than those of the water-column suspension feeders and similar with that of macroalgae, indicating exclusive use of macroalgae-derived organic matter and association with benthic food chains. Most the macrobenthic species were considered to form strong trophic links with benthic food chains. In addition, the distribution of higher $\delta^{15}N$ values in macrobenthic consumers and macroalgae at the intertidal sites of Gwangyang Bay than those at the subtidal sites of the east coast suggests that anthropogenic nutrients may enhance the macroalgal production at the intertidal sites and in turn be incorporated into the particular littoral food web in Gwangyag Bay. These results confirm the dominant role of macroalgae in supporting rocky subtidal and intertidal food webs in the Korean coasts.

Effects of Boliing, Steaming, and Chemical Treatment on Solid Wood Bending of Quercus acutissima Carr. and Pinus densiflora S. et. Z. (자비(煮沸), 증자(蒸煮) 및 약제처리(藥劑處理)가 상수리나무와 소나무의 휨가공성(加工性)에 미치는 영향(影響))

  • So, Won-Tek
    • Journal of the Korean Wood Science and Technology
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    • v.13 no.1
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    • pp.19-62
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    • 1985
  • This study was performed to investigate: (i) the bending processing properties of silk worm oak (Quercus acutissima Carr.) and Korean red pine (Pinus densiflora S. et Z.) by boiling and steaming treatments; (ii) the effects of interrelated factors - sapwood and heartwood, annual ring placement, softening temperature and time, moisture content. and wood defects on bending processing properties; (iii) the changing rates of bending radii after release from a tension strap, and (iv) the improving methods of bending process by treatment with chemicals. The size of specimens tested was $15{\times}15{\times}350mm$ for boiling and steaming treatments and $5{\times}10{\times}200mm$ for treatments with chemicals. The specimens were green for boiling treatments and dried to 15 percent for steaming treatments. The specimens for treatments with chemicals were soaked in saturated urea solution, 35 percent formaldehyde solution, 25 percent polyethylene glycol -400 solution, and 25 percent ammonium hydroxide solution for 5 days and immediately followed the bending process, respectively. The results obtained were as follows: 1. The internal temperature of silk worm oak and Korean red pine by boiling and steaming time was raised slowly to $30^{\circ}C$ but rapidly from $30^{\circ}C$ to $80-90^{\circ}C$ and then slowly from $80-90^{\circ}C$ to $100^{\circ}C$. 2. The softening time required to the final temperature was directly proportional to the thickness of specimen. The time required from $25^{\circ}C$ to $100^{\circ}C$ for 15mm-squared specimen was 9.6-11.2 minutes in silk worm oak and 7.6-8.1 minutes in Korean red pine. 3. The moisture content (M.C.) of specimen by steaming time was increased rapidly first 4 minutes in the both species, and moderately from 4 to 20 minutes and then slowly and constantly in silk worm oak, and moderately from 4 to 15 minutes and then slowly and constantly in Korean red pine. The M.C. of 15mm-squared specimen in 50 minutes of steaming was increased to 18.0 percent in the oak and 22.4 percent in the pine from the initial conditioned M.C. of 15 percent The rate of moisture adsorption measured was therefore faster in the pine than in the oak. 4. The mechanical properties of the both species were decreased significantly with the increase of boiling rime. The decrement by the boiling treatment for 60 minutes was measured to 36.6-45.0 percent in compressive strength, 12.5-17.5 percent in tensile strength, 31.6-40.9 percent in modulus of rupture, and 23.3-34.6 percent in modulus of elasticity. 5. The minimum bending radius (M.B.R.) of sapwood and heartwood was 60-80 mm and 90 mm in silk worm oak, and 260 - 300 mm and 280 - 300 mm in Korean red pine, respectively. Therefore, the both species showed better bending processing properties in sapwood than in heartwood. 6. The M.B.R. of edge-grained and flat-grained specimen in suk worm oak was 60-80 mm, but the M.B.R. in Korean red pine was 240-280 mm and 260-360 mm, respectively. Comparing the M.B.R. of edge-grained with flat-grained specimen, in the pine the edge-grained showed better bending processing property than the flat-grained. 7. The bending processing properties of the both species were improved by the rising of softening temperature from $40^{\circ}C$ to $100^{\circ}C$. The minimum softening temperature for bending was $90^{\circ}C$ in silk worm oak and $80^{\circ}C$ in Korean red pine, and the dependency of softening temperature for bending was therefore higher in the oak than in the pine. 8. The bending processing properties of the both species were improved by the increase of softening time as well as temperature, but even after the internal temperature of specimen reaching to the final temperature, somewhat prolonged softening was required to obtain the best plastic conditions. The minimum softening time for bending of 15 mm-squared silk worm oak and Korean red pine specimen was 15 and 10 minutes in the boiling treatment, and 30 and 20 minutes in the steaming treatment, respectively. 9. The optimum M.C. for bending of silk worm oak was 20 percent, and the M.C. above fiber saturation point rather degraded the bending processing property, whereas the optimum M.C. of Korean red pine needed to be above 30 percent. 10. The bending works in the optimum conditions obtained as seen in Table 24 showed that the M.B.R. of silk worm oak and Korean red pine was 80 mm and 240 mm in the boiling treatment, and 50 mm and 280 mm in the steaming treatment, respectively. Therefore, the bending processing property of the oak was better in the steaming than in the boiling treatment, but that of the pine better in the boiling than in the steaming treatment. 11. In the bending without a tension strap, the radio r/t of the minimum bending radius t to the thickness t of silk worm oak and Korean red pine specimen amounted to 16.0 and 21.3 in the boiling treatment, and 17.3 and 24.0 in the steaming treatment, respectively. But in the bending with a tension strap, the r/t of the oak and the pine specimen decreased to 5.3 and 16.0 in t he boiling treatment, and 3.3 and 18.7 in the steaming treatment, respectively. Therefore, the bending processing properties of the both species were significantly improved by the strap. 12. The effect of pin knot on the degradation of bending processing property was very severe in silk worm oak by side, e.g. 90 percent of the oak specimens with pin knot on the concave side were ruptured when bent to a 100 mm radius but only 10 percent of the other specimens with pin knot on the convex side were ruptured. 13. The changing rate in the bending radius of specimen bent to a 300 mm radius after 30 days of exposure to room temperature conditions was measured to 4.0-10.3 percent in the boiling treatment and 13,0-15.0 percent in the steaming treatment. Therefore, the degree of spring back after release was higher in the steaming than in the boiling treatment. And the changing rate of moisture-proofing treated specimen by expoxy resin coating was only -1.0.0 percent. 14. Formaldehyde, 35 percent solution, and 25 percent polyethylene glycol-400 solution found no effect on the plasticization of the both species, but saturated urea solution and 25 percent ammonium hydroxide solution found significant effect in comparison to non-treated specimen. But the effect of the treatment with chemicals alone was inferior to that of the steaming treatment, and the steaming treatment after the treatment with chemicals improved 10-24 percent over the bending processing property of steam-bent specimen. 15. Three plasticity coefficients - load-strain coefficient, strain coefficient, and energy coefficient - were evaluated to be appropriate for the index of bending processing property because the coefficients had highly significant correlation with the bending radius. The fitness of the coefficients as the index was good at load-strain coefficient, energy coefficient, and strain coefficient, in order.

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Development of Conformal Radiotherapy with Respiratory Gate Device (호흡주기에 따른 방사선입체조형치료법의 개발)

  • Chu Sung Sil;Cho Kwang Hwan;Lee Chang Geol;Suh Chang Ok
    • Radiation Oncology Journal
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    • v.20 no.1
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    • pp.41-52
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    • 2002
  • Purpose : 3D conformal radiotherapy, the optimum dose delivered to the tumor and provided the risk of normal tissue unless marginal miss, was restricted by organ motion. For tumors in the thorax and abdomen, the planning target volume (PTV) is decided including the margin for movement of tumor volumes during treatment due to patients breathing. We designed the respiratory gating radiotherapy device (RGRD) for using during CT simulation, dose planning and beam delivery at identical breathing period conditions. Using RGRD, reducing the treatment margin for organ (thorax or abdomen) motion due to breathing and improve dose distribution for 3D conformal radiotherapy. Materials and Methods : The internal organ motion data for lung cancer patients were obtained by examining the diaphragm in the supine position to find the position dependency. We made a respiratory gating radiotherapy device (RGRD) that is composed of a strip band, drug sensor, micro switch, and a connected on-off switch in a LINAC control box. During same breathing period by RGRD, spiral CT scan, virtual simulation, and 3D dose planing for lung cancer patients were peformed, without an extended PTV margin for free breathing, and then the dose was delivered at the same positions. We calculated effective volumes and normal tissue complication probabilities (NTCP) using dose volume histograms for normal lung, and analyzed changes in doses associated with selected NTCP levels and tumor control probabilities (TCP) at these new dose levels. The effects of 3D conformal radiotherapy by RGRD were evaluated with DVH (Dose Volume Histogram), TCP, NTCP and dose statistics. Results : The average movement of a diaphragm was 1.5 cm in the supine position when patients breathed freely. Depending on the location of the tumor, the magnitude of the PTV margin needs to be extended from 1 cm to 3 cm, which can greatly increase normal tissue irradiation, and hence, results in increase of the normal tissue complications probabiliy. Simple and precise RGRD is very easy to setup on patients and is sensitive to length variation (+2 mm), it also delivers on-off information to patients and the LINAC machine. We evaluated the treatment plans of patients who had received conformal partial organ lung irradiation for the treatment of thorax malignancies. Using RGRD, the PTV margin by free breathing can be reduced about 2 cm for moving organs by breathing. TCP values are almost the same values $(4\~5\%\;increased)$ for lung cancer regardless of increasing the PTV margin to 2.0 cm but NTCP values are rapidly increased $(50\~70\%\;increased)$ for upon extending PTV margins by 2.0 cm. Conclusion : Internal organ motion due to breathing can be reduced effectively using our simple RGRD. This method can be used in clinical treatments to reduce organ motion induced margin, thereby reducing normal tissue irradiation. Using treatment planning software, the dose to normal tissues was analyzed by comparing dose statistics with and without RGRD. Potential benefits of radiotherapy derived from reduction or elimination of planning target volume (PTV) margins associated with patient breathing through the evaluation of the lung cancer patients treated with 3D conformal radiotherapy.