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Organic Acidopathies as Etiologic Diseases of Developmental Delay in Korean Childhood and Adolescent Age Group (한국인 소아청소년기 발달지연의 원인질환으로서의 유기산대사이상질환)

  • Lee, Jong Yoon;Lee, Ye Seung;Choi, Joong Wan;Bae, Eun Joo;Park, Won Il;Oh, Phil Soo;Lee, Hong Jin
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.2
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    • pp.85-93
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    • 2012
  • Purpose: Developmental delay is caused by very diverse etiologic diseases. Most chronic disorders has some influence on development. Chronic or acute disorders of CNS are main etiologic diseases of developmental delay. Up to now, over 60 diseases are included in organic acidopathies and most of them causes acute or chronic recurrent CNS damage and developmental delay. We have done this study to find out the importance of organic acidopathies causing developmental delay in Korean childhood and adolescent patients. Method: Retrograde analysis for 738 patients with developmental delay whose clinical informations are available and have done urine organic acid analysis for 5 years period, between Jan. 1st 2007 to Dec. 31th 2011. Statistical analysis was done with Student's t test using SPSS. Result: Out of 738 patients, 340 patients (46.1%) showed abnormalities on urine organic acid analysis. The most frequent disease was mitochondrial respiratory chain disorders (MRCD) (253, 34.3%), followed by ketolytic defects(39, 5.3%), 3-hydroxyisobutyric aciduria (26, 3.5%), glutaric aciduria type II (8, 1.1%), pyruvate dehydrogenase deficiency (3, 0.4%), 3-methylglutaric aciduria (2, 0.3%), glutaric aciduria type I (2, 0.3%), ethylmalonic aciduria (1, 0.15%), methylmalonic aciduria (1, 0.15%), HMG-CoA lyase deficiency (1, 0.15%), 3-methylcrotonylglycinuria (1, 0.15%), fatty acid oxidation disorders(1, 0.15%) and FAOD (1, 0.15%). Conclusion: Mitochondrial disorders are most frequent etiologic disease on all age group, followed by ketolytic defects and various organic acidopathies. The number and diversities of organic acidopathies emphasize meticulous evaluation of basic routine laboratory examinations and organic acid analysis with initial sample on every developmental patient.

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Monitoring of Restaurant Beef Labeling System (음식점 식육 원산지 표시 모니터링)

  • Hong, Jin;Leem, Dong-Gil;Kim, Mi-Gyeong;Park, Kyoung-Sik;Yoon, Tae-Hyung;No, Ki-Mi;Jeong, Ja-Young
    • Journal of Food Hygiene and Safety
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    • v.25 no.2
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    • pp.162-169
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    • 2010
  • The compulsory beef labelling system has launched from January 1st 2007 by the amended Food Hygiene Law, we were checked the actual conditions of beef origin with a nationwide scale by the Hanwoo differentiation specific test method which was developed by Korea FDA using 90 SNP biomarkers. The test method is useful tool to differentiate the beef origin carrying out the mission of KFDA's annual food safety management guidance. Also we have technically transferred the Hanwoo differentiation specific test method to other institutes as well regional KFDA and established the training program as a regular course in Korea Human Resource Development Institute for Health and Welfare. The beef used in this study were collected according to the 2009 Food safety guidance in roast beef restaurants where business site area greater than 100 $m^2$. Total 216 samples were consisted of 48 samples of the Seoul area and 168 of the region. The monitoring result from restaurants in all the region of Korea showed that 3 of 216 Hanwoo-labelled beefs were found out as a non-Hanwoo (1.3%). This results are gradually deceasing trend compared with 34.0% in 2005, 30.1% in 2006, 3.2% in 2007 and 5.14% in 2008. From these data, the Hanwoo differentiation specific test method on the settlement of the compulsory beef labelling system has an important role. As a outcome of this project, we might be considered the early settlement of the compulsory beef labelling system, technically transferred to other institutes and the establishment of regular training program of the test method.

A Preliminary Survey of a Typhoid Epidemic in the City of Samchunpo During 1967 (1967년(年) 삼천포시(三千浦市)에 발생(發生)된 장(腸)지브스유행(流行)에 관(關)한 조사(調査))

  • Ryu, Young-Hai;Kee, Ryong-Sook
    • The Journal of the Korean Society for Microbiology
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    • v.3 no.1
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    • pp.35-41
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    • 1968
  • An outbreak of typhoid fever in Samchunpo city was surveyed and the results were summarized as follows: 1. 638 of clinical cases were detected in 17 Dongs(districts) of the city of Samchunpo,(54,064), during the epidemic period from 1st September to the end of November, 1967. 2. The morbidity rate was 1,189 per 100,000 population;(1,300 female, and 1,060 male). 3. The highest peak was reached in the third week of September and a second peak appeared at the end of September 1967. 4. The mode of infection was suspected strongly as a water-borne and the source of infection as an old public well called Gal-Dae-Saim, since the causative agent was found in close public latrine and the contaminated sewage ditch witch was connected with the well. 5. All patients and carriers were treated at their home under the supervision of local medical authorities. 6. The Gal-Dae-Saim was closed immediately on 7th October, 1967 by the order of the mayor. 7. At the end of November, 1967 when the outbreak in Samchunpo was almost ended, another small epidemic occurred in Koseong county which bordered the eastern outskirt of the city. 8. During the survey, a strain of Shigella flexneri was isolated from the sewage located three meters from Gal-Dae-Saim and also from one case. 9. It was reported by the local health center in May, 1968, that no carrier had been detected in the survey made among the persons who had had typhoid fever in 1967. Also thereafter no cases of typhoid fever were reported through October, 1968.

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Effect of raw soy flour addtion to Jeung-Pyun pizza on fermentation time and viscosity of batters and texture and general desirability of Jeung-Pyun pizza (날콩가루를 첨가한 증편 피자판 개발에 관한 연구)

  • Yoon, Sun;Lee, Chun-Ja; Park, Hye-Won;Myoung, Chun-Ok;Choi, Eun-Jung;Lee Ji-Jung
    • Korean journal of food and cookery science
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    • v.16 no.3
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    • pp.267-271
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    • 2000
  • The present study was designed to develop the standardized formula for the preparation Jeung-Pyun with added raw soy flour and to investigate the applicability of Jeung-Pyun as the substitute of regular pizza crust made with wheat flour. The effect of adding raw soy flour at 3%, 5% level on fermentation time and viscosity of batters and texture and general desirability of Jeung-Pyun pizza were also studied. As the level of raw soy flour was increased the time required for 1 st and 2nd fermentation decreased and viscosity of batters increased significantly. Jeung-Pyun crust after steaming found to have appropriate texture and form for a substitute of wheat flour pizza crust. Texture parameters determined by QST showed that Jeung-Pyun crust with added raw soy flour had lower values in hardness, cohesiveness and gumminess without any significant difference. However, chewiness of 5% raw soy flour group was significantly lower than control group. Texture of Jeung-Pyun crust and general desirability of Jeung-Pyun pizza were determined by sensory evaluation. Textural parameters of Jeung-Pyun crust were evaluated on the basis of hardness, cohesiveness, adhesiveness, tenderness and wetness. As a result of sensory evaluation Jeung-Pyun crust made with 5% raw soy flour had significant lower values in hardness, cohesiveness, tenderness and wetness than the control. However, the textural parameters between control and 3 % raw soy flour group were not significantly different. Jeung-Pyun pizza with topping were evaluated by sensory panel. Jeung-Pyun pizza made with 3% raw soy flour received the highest score in apperance and general desirability without any significant difference. 5% raw soy flour group had significantly lower score in general desirability among 3 treatment groups. In conclusion, Jeung-Pyun crust made by traditional Jeung-Pyun preparation method will be a good alternative to replace wheat flour pizza crust. Adding raw soy flour at 3% level did not affect the quality of Jeung-Pyun crust and had an effect of promoting fermentation. Jeung-Pyun Pizza crust was expected to have tender texture and slower retrogradation rate than regular pizza crust with wheat flour.

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Temporal and Spatial Variation Analysis of Suspended Solids, Ionic Contents, and Habitat Quality in the Woopo Wetland Watershed (우포늪 수계에서 부유물, 이온농도 및 서식지 특성에 대한 시 ${\cdot}$ 공간적 변이 분석)

  • Bae, Dae-Yeul;Choi, Ji-Woong;An, Kwang-Guk
    • Korean Journal of Ecology and Environment
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    • v.39 no.4 s.118
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    • pp.498-507
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    • 2006
  • The main objective of present study was to evaluate how seasonal rainfall influenced natural habitat conditions of 10 metric habitat variables along with ionic conditions and suspended solids in the Woopo Wetland during August 2002-July 2003. Largest spatial variabilities in total suspended solids (TSS) occurred during the summer monsoon and the inorganic suspended solids (ISS), expressed as a inorganic proportion of total solids, showed linearly increasing trend from the upstream to downstream. This phenomenon was mainly attributed to counter flow of turbid water from the main Nakdong-River. During the flooding, ISS : TSS ratio showed large increases (92%) in the downstream than the upstream (43%). For this reason, transparency declined (mean=0.13 m, range=0.08-0.21 m) largely in the downstream reach and thus, chlorophyll-a concentration showed low values (range: $4.2-8.6\;{\mu}g\;L^{-1}$), indicating a direct influence on primary productivity or algal growth by inorganic turbidity. In the 2nd survey, ISS averaged 4.0 mg $L^{-1}$ (3.3-4.8 mg $L^{-1}$), thus the ISS decreased by 14 fold, compared to the ISS in the 1st survey during the flooding, while organic suspended solids (OSS) values were greater than those of ISS, indicating a dominance of organic solids. This condition was similar to solid contents in the 3rd survey, but showed a large difference compared to the 4th survey during the growing season. Habitat health assessments, based on 10 metric habitat variables, showed that QHEI values were greatest in the growing season (May) than any other seasons and largest spatial variations occurred in the 2nd survey. Overall, dataset suggest that seasonal episodic flooding during the monsoon may largely contribute nutrient cycling and sediment contents in the Woopo Wetland and Topyung Stream.

Long-term Changes of the Fish Fauna and Community Structure in the Jungrang Creek, Seoul, Korea (중량천의 어류상과 어류군집 구조의 장기 변동)

  • Kim, Gyeo-Bung;Kil, Hye-Kyung;Yu, Byung-Tae;Kim, Min-Young;Bae, Kyung-Seok
    • Korean Journal of Ecology and Environment
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    • v.35 no.2 s.98
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    • pp.63-70
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    • 2002
  • Long-term changes of the fish fauna and its community structure from the Jungrang Creek of the Han River system in Seoul were investigeted from 1990 to 2000. Total species occurred during the survey period were 14 species in 6 families, and total individual number was 108,366. Fishes at the Jungrang Creek had not been distributed in the 1980s and 1990 because of heavy water pollution as well as environmental damage in the riparian areas. However, Cyprinus carpio and Carassius auratus in cyprinidae were rehabitated since the early 1990s when the water quality was improved because of foundation of sewage division pipe. Species nombers gradually increased to 6 species in 1996, 9 species in 1998, and 11 species in 2000. Individual numbers rapidly increased to 164 individuals in 1996, 146 individuals in 1998, and 108,094 individuals in 2000. A large number of Cyprinus carpio and Carassius auratus, which are abundantly distributed in the main course of the Han River, were found when they migrate to the upper reaches of the Jungrang Creek for spawning. Mass fish floatings were occurred on the 21st of April and the 11th of June in 2000 due to DO depletion at the lower site (Site 4) of discharging point of Jungrang sewage treatment plant and stagnant spot(Site 3) of dammed pool. Major dominant species were Cyprinus carpio and Carassius auratus (Cyprinidae). Others dominant species were Pseudorasbora lab대 (Cyprinidae) and Silurus asotus (Siluridae). Dominance indices were relatively high (0.79 to 1.00). Species diversity andrichness indices were relatively low (0 to 1.66and 0 to 1.41, respectively). Species diversity and richness indices were slightly higher at the middle reaches (Site 2 and Site 3) than the upper reaches and the mouth of the Jungrang Creek. However, the fish fauna of the Jungrang Creek was very poor and its community structure was very simple.

Association of Genetic Variations with Pemetrexed-Induced Cytotoxicity in Non-Small Cell Lung Cancer Cells (비소세포폐암 세포주에서 pemetrexed의 세포독성과 유전학적 다형성과의 상관성 조사)

  • Yoon, Seong-Ae;Choi, Jung-Ran;Kim, Jeong-Oh;Shin, Jung-Young;Zhang, XiangHua;Kang, Jin-Hyoung
    • Journal of Life Science
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    • v.20 no.1
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    • pp.103-112
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    • 2010
  • Pemetrexed has demonstrated clinical activity in non-small cell lung cancer (NSCLC) as well as other solid tumors. It transports into the cells via reduced folate carrier (RFC) and is polyglutamated by folypolyglutamate synthetase (FPGS). Pemetrexed directly inhibits several folate-dependent enzymes such as thymidylate synthase (TS), dihydrofolate reductase (DHFR), and glycinamide ribonucleotide formyltransferase (GARFT). We investigated the effects of genetic variations and the expression of RFC, FPGS, TS and DHFR enzymes on drug sensitivity to pemetrexed in NSCLC cells. Polymorphisms in RFC, FPGS, and DHFR were genotyped in four NSCLC cells - A549, PC14, HCC-1588, and H226. Real-time RT-PCR and Western blot was performed to evaluate mRNA transcripts and protein of these genes. The cytotoxicity of pemetrexed was measured by SRB assay. In PC14 and H226 cells, increased mRNA expressions of RFC and FPGS were associated with higher cytotoxicity to pemetrexed. 2R/2R genotype of TS and its increased mRNA expression were associated with drug resistance to pemetrexed in A549 cells, whereas 3R/3R genotype in TS with decreased mRNA expression was associated with higher sensitivity in H226 cells. After pemetrexed treatment, an inverse change of DHFR mRNA and protein expression was found. The strongest linkage disequilibrium (LD) was discovered between-1726C>T and -1188A>C SNP of DHFR gene. Our findings suggest the cytotoxic effect of pemetrexed may be associated with genetic polymorphisms and the expression level of genes involved in pemetrexed metabolisms in NSCLC cells.

Effect of Water Temperature on the Growth of Triops longicaudatus (LeConte) (Notostraca: Triopsidae) (수온이 긴꼬리투구새우(배갑목: 투구새우과)의 생장에 미치는 영향)

  • Kwon, Soon-Jik;Jun, Yung-Chul;Park, Jae-Heung;Won, Doo-Hee;Seo, Eul-Won;Lee, Jong-Eun
    • Journal of Life Science
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    • v.20 no.11
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    • pp.1662-1666
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    • 2010
  • Growth and other biological processes in aquatic organisms are particularly dependent on water temperatures. This study examined the effects of water temperature on the growth of Triops longicaudatus. The influence of water temperature fluctuations was that growth rate was increased at higher temperatures. The mean carapace length was 5.7 (${\pm}2.1$) mm in a water temperature of $20^{\circ}C$ and 7.5 (${\pm}0.5$) mm in a water temperature of $28^{\circ}C$ on the 14th day after submergence. It was 6.9 (${\pm}2.8$) mm in a water temperature of $20^{\circ}C$ and 7.8 (${\pm}2.0$) mm in a water temperature of $28^{\circ}C$ on the 21st day after submergence. The mean carapace length grew rapidly within 14 days after submergence, but increase in carapace length beyond this time was slow. The influence of water depth fluctuations was low as the mean carapace length was 9.3 (${\pm}2.1$) mm under a water depth of 80 mm and 9.5 (${\pm}1.3$) mm under a water depth of 190 mm on the 19th day after submergence. Biomass showed that the carapace length of 5, 10, 16 and 20 mm was a dry-weight of 1.1 (${\pm}0.3$), 18.0 (${\pm}3.7$), 26.0 (${\pm}0.0$) and 52.3 (${\pm}4.0$) mg respectively. The number of eggs increased rapidly with increments in carapace length. The mean number of eggs was 20 (${\pm}0.0$) at a carapace length of 7.0 mm, but at a carapace length of 17.0 mm, the mean number of eggs was 560 (${\pm}0.0$). The results suggested that differences in water temperature accounted for the differences in length of the carapace and the number of eggs.

Analysis of the Contents of Clothing and Textiles Education of Practical Arts and Home Economics Education between before and after the Revision of the 7th Curriculum (2007 교육과정 개정 전과 후의 실과 및 가정과 의생활 교육내용 분석)

  • Park, Soon-Ja
    • Journal of Korean Home Economics Education Association
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    • v.19 no.3
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    • pp.37-60
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    • 2007
  • This study has been historically reviewed the clothing and textiles education through references. The clothing and textiles education had been informally offered as a part of household skill, skill of daily life and home skill before the law of school education was established. Therefore, we have reviewed the changes in the clothing and textiles education by separating the prior period between the end of the old Korea and the liberation in 1945, from the later period from the liberation up to now. And also contents of clothing and textiles education between before and after the revision of the 7th curriculum were compared and analyzed. The clothing and textiles education had put emphasis on sewing, handicraft and household during the prior period from the end of the old Korea to the liberation, but after liberation, it became involved in Home Economics Education. The number of classes and the contents of clothing and textiles education had been increased and deepened from the 1st term to the 5th term of curriculum. In particular, the 4th and 5th term of curriculum became a turning point where home life section was intensified. Even though the number of classes was reduced during the 6th term of curriculum, with increase in target school grades for this education, and Home Economics in the middle school remained an independent subject matter. However, during the 7th term of curriculum, Technology-Home Economics Education in the middle school courses led to losing independent subject name and shrinking its contents. Through the revision in the 7th curriculum, the clothing and textiles education achieved great improvement and progress by gaining balance in distribution of contents among school grades and balance between theory and practice. However it still remains as an issue to be more studied and resolved how well the contents are matched with the interests and attention of students. We need to consider and reflect request of students under learner-centered curriculum. Considering enormous changes in the environment surrounding the clothing and textiles education, it is crystal clear that the gap between existing contents of education and teaching methods and our real life will be widened. Because students' perceptions of value have been diversified, it is our task to develop better contents and learning system in order to help students have interests, attention and desire in clothing and textiles life in line with social needs for desirable clothing and textiles education.

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Immobilization of Burkholderia cepacia Lipase on Weak Base Styrene Resin Using Polyethyleneimine with Cross-linking (PEI(Polyethyleneimine)를 이용하여 음이온계 레진에 고정화된 Lipase AH 제조 및 효소적 Interesterification을 통한 반응 특성 연구)

  • Lee, Chi Woo;Lee, Ki Teak
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.43 no.7
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    • pp.1025-1035
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    • 2014
  • This study assessed the effect of immobilized lipase on weak base styrene resin using polyethyleneimine (PEI) with cross-linking. Two procedures were used in this study. The first one, "mono-layer" lipase immobilization, involves washing PEI after adsorption. The second procedure, "multi-layer" lipase immobilization, has no washing before the cross-linking step. Treverlite XS-100200 (weak base styrene resin) was immersed with PEI solution (2.2 mg/mL). Lipase AH (from Burkholderia cepacia) was adsorbed onto the support coated with PEI before cross-linking with glutaraldehyde. Structured lipid was synthesized by immobilized lipase-catalyzed interesterification using canola oil, palmitic ethyl ester (PEE), and stearic ethyl ester (StEE). Total fatty acid contents of triacylglycerol (TAG) in structured lipids were analyzed to investigate activity, properties, and reusability of immobilized lipases. Activities of immobilized lipases on the multi-layer and mono-layer increased at a high concentration (8 mg/mL) of lipase solution used for immobilization. The results show that immobilized lipase with the mono-layer method at pH 8.0 on resin had the highest total saturated fatty acid content (26.17 area%). Activity of immobilized lipase with the multi-layer method at pH 7.5 on support was lower than that of the mono-layer, but total saturated fatty acid content was 16.79 area% higher than that of lipase AH (15.01 area%).