• Title/Summary/Keyword: G418

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A Case of Ornithine Transcarbamylase Deficiency in 11-month-old Female who Presented Periodic Vomiting and Intermittent Consciousness Change (반복적인 구토 및 간헐적 의식 변화를 주소로 진단된 Ornithine Transcarbamylase Deficiency 여아 1례)

  • Kim, Jin Ah;Kim, Jin Sup;Huh, Rimm;Cho, Sung Yoon;Jin, Dong Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.3
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    • pp.165-170
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    • 2015
  • Ornithine transcarbamylase (OTC) deficiency is a rare X-linked genetic disorder of urea synthesis in newborns. It is the most common urea cycle disorder and leads to elevated levels of ammonia in the blood. Excessive ammonia can cause various symptoms, including vomiting, lethargy, and coma. Boys have a more serious form of OTC deficiency than girls. If not treated immediately, severe OTC deficiency can lead to neurologic abnormalities, hyperammonemic coma, and death. Because late-onset OTC deficiency, which is more common in girls, presents mild symptoms, it is easy to miss diagnosis and prompt treatment. We describe an 11-month-old girl who presented periodic vomiting, intermittent lethargy, and seizure. She was diagnosed with OTC deficiency by elevated serum ammonia and urine orotic acid levels. Genetic analysis of the OTC gene revealed a missense mutation in exon 5 (c.418G>C). We reported an experience of exact diagnosis and successful treatment of late-onset OTC deficiency in our patient.

Comparison of the habitat distribution characteristics of Aquatic Oligochaeta according to the construction of weirs in four major rivers in South Korea (4대강 보 건설에 따른 실지렁이류(Aquatic Oligochaeta) 서식분포 특성 비교)

  • Wang, Ju Hyoun;Park, Hyung Joon;Park, Jung Ho;Song, Haeng Seop;Kim, Ho Joon;Park, Yeon Jeong;Choi, Jun Kil;Lee, Hwang Goo
    • Korean Journal of Environmental Biology
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    • v.37 no.4
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    • pp.607-617
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    • 2019
  • This study examined the habitat distribution characteristics of Aquatic Oligochaeta according to the construction of weirs in the four major rivers in South Korea. The area was investigated 10 times from March to October in 2018 and a total of 18 species and 35,390 ind. m-2 were sampled. The dominant species by number was Limnodrilus hoffmeisteri(77.1%) and the subdominant species was Branchiura sowerbyi(9.5%). A total of 32.418 g m-2 dry weight was analyzed. The correlation analysis showed that the number of Aquatic Oligochaeta individuals correlated with the sediment depth, clay ratio, silt ratio, sand ratio, cobble ratio, pebble ratio, pH and TOC mg L-1. Specifically, the clay ratio and sediment depth were associated with increases in the number of individuals. The total organic carbon(TOC) increased from March to October and, conversely, the number of individuals tended to decrease.

Study on Characteristics of Fine Bottom Ash Based Geopolymer Mortar (미분쇄 바텀애시 기반 지오폴리머 모르타르 특성에 관한 연구)

  • Lim, Gwi-Hwan;Lee, Jeong-Bae;Jeong, Hyun-Kyu;Kim, Seong-Soo
    • Journal of the Korean Recycled Construction Resources Institute
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    • v.4 no.4
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    • pp.418-424
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    • 2016
  • This study is an experimental study on the recycling of bottom ash in coal ash discharged from a thermal power plant. Bottom ash has limited research on recycling because it has more porous and higher water absorption ratio than fly ash. In this paper, the bottom ash was pulverized to a specific surface area of $4,000cm^2/g$ in order to use as a binder, and the flow, compressive strength test and microstructure analysis of the bottom ash based geopolymer mortar were performed. The flow measurement results of the geopolymer mortar showed that the flow rate was improved by increasing mixing water as the molar concentration of activator was increased. Compressive strength increased with increasing curing temperature and molar concentration. Through the microstructure analysis, we could confirm the geopolymer gel produced by the reaction of the condensation polymerization. It is considered that it is possible to make the bottom ash based geopolymer concrete through proper molar concentration of activator and high temperature curing.

growth of Cadmium Sulfide (CdS) Thin Film by Solution Growth Technique and Study of Quantum Size Effects (용액성장법에 의한 Cadmium Sulfide(CdS) 박막 성장 및 양자 사이즈 효과에 관한 연구)

  • 임상철
    • Journal of the Microelectronics and Packaging Society
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    • v.4 no.1
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    • pp.1-12
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    • 1997
  • 본 연구에서는 용액성장법에 의해 양자 입자로 구성된 CdS 박막을 슬라이드 유리기 판위에 성장시키고 이들의 구조적 광학적 특성에 대하여 연구하였고 이들 결과를 토대로 용 액성장법으로 성장된 CdS 박막의 양자 사이즈 효과에 대하여 연구하였다. 성장시간은 1, 3, 10, 20분이었고 성장온도는 75$^{\circ}C$였다. X-선 회절 분석결과 본 연구에서 합성된 CdS 박막은 hexagonal상의 결정구조를 갖는 것으로 나타났고 성장시간에 따라 막의 투께는 61~195nm, 입자사이즈는 8.5~22.5nm로 나타났다. 광에너지 변화에 따른 투과도 측정결과 본 연구의 CdS 시료는 성장시간에 따라 에너지 밴드갭이 2.43~2.51 eV로 나타나서 벌크 CdS의 에너 지 밴드갭인 2.42 ev보다 높은 에너지 밴드갭을 갖게 되어 양자 사이즈 효과에 의한 blue shift 현상이 용액성장법에 의해 합성된 CdS 시료에도 존재한다는 것이 밝혀졌다 그리고 이 같은 용액성장법으로 성장된 CdS에 대해 최초로 수행된 Raman 산란 실험결과 이성장방법 으로 성장된 CdS에는 1TO, E2, 1LO 포논 모드가 존재함을 알수 있었고 또한 입자 사이즈 감소에 의한 1LO포논 모드의저주파수 shift 현상 즉 포논 모드의 softening 현상이 있음이 밝혀졌고 softening은 최대6.2%까지 발생하였다. 이와같은 높은 softening은 본연구의 CdS 박막 내 양자 입자의 입도가 작은것에 기인하는 것으로 밝혀졌다. 또한 본 CdS 시료의 양 자 사이즈 효과의 결과로 1TO 포논도 나타났는데 이 1TO 포논과 E2 포논의 Raman shift 는 성장시간 즉 막의 두께와는 무관한 것으로 나타났다.행렬모형(二重比例行列模型)을 이용하여, 산업구조의 변화로 인한 직업별 인력수요 변화가 충분히 고려되도록 하였다. 전망의 결과에 따르면 향후 우리 경제는 지식기반경제(knowledge-based economy)로 이행하고 있다고 볼 수 있다. 우선 산업구조면에서 지식집약적산업으로의 구조조정이 일어나게 되고 이에 따라 산업별 취업구조에서도 고기술산업의 취업준비중이 급속히 증가하게 된다. 직업별 취업분포에 있어서도 전문기술직 행정관리직 등의 고숙련 사무직의 비중은 크게 증가하는 반면 생산관련직과 농림어업직의 비중은 감소하게 된다. 이처럼 경제가 지식집약화되어 감에 따라 고학력자에 대한 수요는 지속적으로 증가하지만 현재 적절한 인력양성과 공급이 이루어지지 않고 있어 향후 기술이나 기능에 따른 수급부일정(需給不一政)(skill mismatch)현상이 매우 심해질 것으로 보인다. 따라서 앞으로의 인력정책에서 가장 주안점을 두어야 할 부분은 첨단기술산업과 관련된 인력의 양성에 있다고 하겠다.2시간까지 LPDG용액은 MEC용액보다 비교적 나은 회복을 보였고 재관류 3일과 7일의 폐기능 평가에서 두 용액 모두에서 폐기능의 점차적 소실을 보였으며 이는 병리조직검사에서 보듯이 폐혐에 의한 외적인 요소라고 생각되며 따라서 LPDG용액은 허혈재관류손상 방지 및 급성폐렴 등 염증을 잘 관리한다면 20시간 이상 LPDG용액의 안전한 폐보존의 가능성 을 얻을 수 있었다.ic 형태로 외래유전자가 발현되었지만 대조구에서 87.0% (26/30개) 배반포기가 $\beta$-Gal 활력을 보인 반면, G418 처리구에서는 모든 배반포기가 $\beta$-Gal 활력을 보였다 (P<0.05). 그러나 대조구 및 G418 처리구의 ICM

Effects of $\beta$-glucan from Schizophillum commune on Non-specific Immune Parameters in Common Carp (Cyprinus carpio) and Flounder (Paralichthys olivaceus) by Oral Administration (경구투여 $\beta$-glucan이 잉어와 넙치의 비특이적 면역활성에 미치는 영향)

  • PARK Sung-Woo;KWAK Jung-Ki;KOO Jae-Geun;CHO Man-Gi
    • Korean Journal of Fisheries and Aquatic Sciences
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    • v.34 no.4
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    • pp.412-418
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    • 2001
  • The effects of dietary $\beta$-glucan administration on non-specific immune parameters in common carp, Cyprinus carpio, (1.0 g and 68.7 g of body weight) and flounder, Paralichthys olivcaces (12.1 g and 54.0 g of body weight) were evaluated. All fishes were fed an experimental diet supplemented with $\beta$-glucan at $0.1\%$ per kg diet for 5 weeks. A week intermission with basal diet occurred between first 2 weeks and second 2 weeks of $\beta$-glucan administration, The changes in the numbers of peripheral neutrophils and macrophages were counted under light microscopy and serum lysozyme activity was also analysed at a week of interval during the experiment. Phagocytic activities of leucocytes from the swimm bladder of carp and the peritonium of flounder were measured 5 weeks after feeding. The oral adminisration of $\beta$-glucan induced significant reduction in mortality after an artificial challenge with $1\times10^6$ cells of Aeromonas hydrophila in larger carp and $1\times10^5$ cells of Edwardsiella tarda in larger flounder but did not in other groups. The numbers of peripheral macrophages and neutrophils, phagocytic acitivies of leucocytes, and the activity of serum lysozyme were greatly increased in the fish fed a $\beta$-glucan supplemented diet. These suggest that $\beta$-glucan administration by oral route can enhance leucocyte phagocytic activity, serum lysozymal activity, and survival rate against artificial infections depending on the infected fish size and challenged bacterial concentration.

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Optimization of uranium biosorption in solutions by Sargassum boveanum using RSM method

  • Hashemi, Nooshin;Dabbagh, Reza;Noroozi, Mostafa;Baradaran, Sama
    • Advances in environmental research
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    • v.9 no.1
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    • pp.65-84
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    • 2020
  • The potential use of Sargassum boveanum algae for the removal of uranium from aqueous solution has been studied by varying three independent parameters (pH, initial uranium ion concentration, S. boveanum dosage) using a central composite design (CCD) under response surface methodology (RSM). Batch mode experiments were performed in 20 experimental runs to determine the maximum metal adsorption capacity. In CCD design, the quantitative relationship between different levels of these parameters and heavy metal uptake (q) were used to work out the optimized levels of these parameters. The analysis of variance (ANOVA) of the proposed quadratic model revealed that this model was highly significant (R2 = 0.9940). The best set required 2.81 as initial pH(on the base of design of experiments method), 1.01 g/L S. boveanum and 418.92 mg/L uranium ion concentration within 180 min of contact time to show an optimum uranium uptake of 255 mg/g biomass. The biosorption process was also evaluated by Langmuir, Freundlich, Temkin and Dubinin-Radushkevich isotherm models represented that the experimental data fitted to the Langmuir isotherm model of a suitable degree and showed the maximum uptake capacity of 500 mg/g. FTIR and scanning electron microscopy were used to characterize the biosorbent and implied that the functional groups (carboxyl, sulfate, carbonyl and amine) were responsible for the biosorption of uranium from aqueous solution. In conclusion, the present study showed that S. boveanum could be a promising biosorbent for the removal of uranium pollutants from aqueous solutions.

Effect on Eel Anguilla japonica and Crop Growth by the Development of a Biofloc Technology (BFT) Aquaponic System (바이오플락 기반 아쿠아포닉스 시스템 개발에 의한 뱀장어(Anguilla japonica)와 재배작물의 성장에 미치는 영향)

  • Hwang, Ju-Ae;Lee, Jeong-Ho;Park, Jun Seong;Choe, Jong Ryeol;Lee, Donggil;Kim, Hyeongsu
    • Korean Journal of Fisheries and Aquatic Sciences
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    • v.54 no.4
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    • pp.418-425
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    • 2021
  • The effects of an aquaponic system based on biofloc technology (BFT-AP) were analyzed for eel Anguilla japonica as aquaculture species and caipira Lactuca sativa as the cultivated crops. The rate of weight gain rate by the eels was 178% (BFT-AP 200 head) > 136% (BFT-AP 100 head) > 100% (BFT). The eel body weight in the BFT-AP (200 head) significantly increased when compared to the BFT only eel group (P<0.05). The weight of the upper layer of caipira was 91±8.5 g (200 head) > 90±8.9 g (100 head) > 48±8.3 g (Hydroponic crop, HP). The crop growth in all BFT-AP groups was higher than the control, the hydroponic group. The total ammonia nitrogen (TAN) and NO2--N concentrations decreased in the BFT-AP group when compared to the BFT group. It was possible to remove nitric acid from the aquaponics system and reuse it as eel rearing water. Although some nutrient concentrations were low in BFT when compared to HP, the nutrient concentration was sufficient for plant growth. The results show that BFT has the potentially to provide a sustainable aquaponic system.

Alkaline Peroxide Pretreatment of Waste Lignocellulosic Sawdust for Total Reducing Sugars

  • Satish Kumar Singh;Sweety Verma;Ishan Gulati;Suman Gahlyan;Ankur Gaur;Sanjeev Maken
    • Korean Chemical Engineering Research
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    • v.61 no.3
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    • pp.412-418
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    • 2023
  • The surge in the oil prices, increasing global population, climate change, and waste management problems are the major issues which have led to the development of biofuels from lignocellulosic wastes. Cellulosic or second generation (2G) bioethanol is produced from lignocellulosic biomass via pretreatment, hydrolysis, and fermentation. Pretreatment of lignocellulose is of considerable interest due to its influence on the technical, economic and environmental sustainability of cellulosic ethanol production. In this study, furniture waste sawdust was subjected to alkaline peroxide (H2O2) for the production of reducing sugars. Sawdust was pretreated at different concentrations from 1-3% H2O2 (v/v) loadings at a pH of 11.5 for a residence time of 15-240 min at 50, 75 and 90 ℃. Optimum pretreatment conditions, such as time of reaction, operating temperature, and concentration of H2O2, were varied and evaluated on the basis of the amount of total reducing sugars produced. It was found that the changes in the amount of lignin directly affected the yield of reducing sugars. A maximum of 50% reduction in the lignin composition was obtained, which yielded a maximum of 75.3% total reducing sugars yield and 3.76 g/L of glucose. At optimum pretreatment conditions of 2% H2O2 loading at 75 ℃ for 150 min, 3.46 g/L glucose concentration with a 69.26% total reducing sugars yield was obtained after 48 hr. of the hydrolysis process. Pretreatment resulted in lowering of crystallinity and distortion of the sawdust after the pretreatment, which was further confirmed by XRD and SEM results.

Synthesis, Spectroscopic, and Biological Studies of Chromium(III), Manganese(II), Iron(III), Cobalt(II), Nickel(II), Copper(II), Ruthenium(III), and Zirconyl(II) Complexes of N1,N2-Bis(3-((3-hydroxynaphthalen-2-yl)methylene-amino)propyl)phthalamide (N1,N2-bis(3-((3-hydroxynaphthalen-2-yl)methylene-amino)propyl)phthalamide의 크롬(III), 망간(II), 철(III), 코발트(II), 니켈(II), 구리(II), 루테늄(III) 및 산화 지르코늄(II) 착물에 대한 합성과 분광학 및 생물학적 연구)

  • Al-Hakimi, Ahmed N.;Shakdofa, Mohamad M.E.;El-Seidy, Ahemd M.A.;El-Tabl, Abdou S.
    • Journal of the Korean Chemical Society
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    • v.55 no.3
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    • pp.418-429
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    • 2011
  • Novel chromium(III), manganese(II), iron(III), cobalt(II), nickel(II), copper(II), ruthenium(III), and zirconyl(II) complexes of $N^1,N^2$-bis(3-((3-hydroxynaphthalen-2-yl)methylene-amino)propyl)phthalamide ($H_4L$, 1) have been synthesized and characterized by elemental, physical, and spectral analyses. The spectral data showed that the ligand behaves as either neutral tridentate ligand as in complexes 2-5 with the general formula $[H_4LMX_2(H_2O)]{\cdot}nH_2O$ (M=Cu(II), Ni(II), Co(II), X = Cl or $NO_3$), neutral hexadentate ligand as in complexes 10-12 with the general formula $[H_4LM_2Cl_6]{\cdot}nH_2O$ (M=Fe(III), Cr(III) or Ru(III)), or dibasic hexadentate ligand as in complexes 6-9 with the general formula $[H_2LM_2Cl_2(H_2O)_4]{\cdot}nH_2O$ (M = Cu(II), Ni(II), Co(II) or Mn(II), and 13 with general formula $[H_4L(ZrO)_2Cl_2]{\cdot}8H_2O$. Molar conductance in DMF solution indicated the non-ionic nature of the complexes. The ESR spectra of solid copper(II) complexes 2, 5, and 6 showed $g_{\parallel}$ >g> $g_e$, indicating distorted octahedral structure and the presence of the unpaired electron in the $N^1,N^2$ orbital with significant covalent bond character. For the dimeric copper(II) complex $[H_2LCu_2Cl_2(H_2O)_4]{\cdot}3H_2O$ (6), the distance between the two copper centers was calculated using field zero splitting parameter for the parallel component that was estimated from the ESR spectrum. The antibacterial and antifungal activities of the compounds showed that, some of metal complexes exhibited a greater inhibitory effect than standard drug as tetracycline (bacteria) and Amphotricene B (fungi).

A New Sweetpotato Cultivar for Use of Bioethanol 'Daeyumi' (바이오에탄올용 고구마 신품종 '대유미')

  • Lee, Joon-Seol;Ahn, Young-Sup;Chung, Mi-Nam;Kim, Hag-Sin;Jeong, Kwang-Ho;Bang, Jin-Ki;Song, Yeon-Sang;Shim, Hyeong-Kwon;Han, Seon-Kyeong;Suh, Sae-Jung
    • Korean Journal of Breeding Science
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    • v.42 no.6
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    • pp.674-678
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    • 2010
  • A new sweetpotato variety, 'Daeyumi', was developed by Bioenergy Crop Research Center, National Institute of Crop Science (NICS), RDA in 2008. This variety was obtained from the cross between 'Jinhongmi' and 'Xusju 18' in 2000. The seedling and line selections were performed from 2001 to 2003, preliminary and advanced yield trials were carried out from 2004 to 2005, and the regional yield trials were conducted at six locations from 2006 to 2008. 'Daeyumi' has cordate leaf, green vine and petiole, elliptic storage root, red skin and yellow flesh color of storage root. This variety is also resistant to Fusarium wilt and nematode. The starch value was 25.9%, ethanol yield was 418 L/Ton, which was 7% higher than that of 'Yulmi' variety, and the total sugar content was 2.47 g/100g, dry weight. 'Daeyumi's initial temperature of starch gelatinization was lower, 76.2$^{\circ}C$, and the retrogradation process was earlier than 'Yulmi'. The average yield of storage root was 27.8 ton/ha in the regional yield trials, which was 36% higher than that of 'Yulmi' variety. Number of storage roots over 50 gram per plant was 3.0, and the average weight of one storage root was 152 gram. This variety can be used for the production of bioethanol and starch processing.