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Case Report on NTBC Treatment of Type 1 Tyrosinemia Diagnosed through Newborn Screening (신생아 선별검사를 통해 진단된 1형 타이로신혈증의 NTBC 치료 사례 보고)

  • Ji Eun Jeong;Hwa Young Kim;Jung Min Ko
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.23 no.2
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    • pp.39-44
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    • 2023
  • Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by biallelic pathogenic variants in the fumarylacetoacetate hydrolase (FAH) gene, which impairs the function of the FAH enzyme, resulting in the accumulation of tyrosine's toxic metabolites in hepatocytes and renal tubular cells. As a consequence, individuals with HT-1 exhibit symptomatic manifestations. Rapid diagnosis and treatment of HT-1 can prevent short-term death and long-term complications. A 15-day-old boy presented to the outpatient department with elevated levels of tyrosine on his newborn screening tests conducted at the age of 3 and 10 days, respectively. Further blood tests revealed increased levels of alpha-fetoprotein and amino acids including tyrosine and threonine. Urine organic acid tests indicated a significant elevation in tyrosine metabolites, as well as the presence of succinylacetone (SA), which led to the diagnosis of HT-1. Two pathogenic and likely pathogenic variants of FAH compatible with HT-1 were also detected. He began a tyrosine-restricted diet at one month old and received nitisinone (NTBC) at two months old. With continued treatment, the patient's initially elevated AFP level, detection of SA in the urine, and mild hepatomegaly showed improvement. During four years and seven months of treatment, there were no exceptional complications apart from an increase in tyrosine levels and a delay in speech. We report a case of tyrosinemia type 1 detected through newborn screening, treated with dietary restriction and NTBC, with a good prognosis.

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Characterization and Purification of Subtilosin A Produced by Bacillus vallismortis MCBL 1012 Isolated from Seasoned Dried Radish

  • Se-Yeon Lee;Dae-Ook Kang
    • Journal of Life Science
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    • v.34 no.8
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    • pp.576-587
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    • 2024
  • In this study, diverse bacterial strains were isolated from fermented foods to screen those with antibacterial activity. Among them, one strain, identified as Bacillus vallismortis MCBL 1012 through 16S rRNA gene sequence analysis, was selected for its bacteriocin production. The culture supernatant of B. vallismortis MCBL 1012 showed antibacterial activity, mainly against Gram-positive bacteria. Scanning electron microscopy (SEM) revealed that bacteriocin treatment led to cellular content leakages in Listeria monocytogenes KCCM 40307, Enterococcus faecium KCCM 12118, and Streptococcus mutans KCTC 3065. PCR analysis confirmed B. vallismortis MCBL 1012 harbored subtilosin A gene (sbo A). Antibacterial activity was decreased by proteolytic enzymes like proteinase K, subtilisin A, and α-chymotrypsin. The bacteriocin demonstrated stability at 40℃ and 60℃ for 120 min, and up to 80℃ for 60 min, with rapid activity loss at 100℃. It retained full antibacterial activity within a pH range of 4.0 to 8.0 and was not affected by up to 100% organic solvents like ethanol, methanol, acetonitrile, and tetrahydrofuran. Nevertheless, activity decreased with more than 40% isopropanol and 80% acetone. Most tested inorganic salts and detergents had no effect on antibacterial activity except, CuSO4 and NiSO4 at specified concentrations. The bacteriocin exerted its antibacterial effect through bactericidal action against L. monocytogenes KCCM 40307. The bacteriocin was purified by ammonium sulfate precipitation, DEAE anion exchange chromatography, and RP-HPLC. The purification resulted in a final yield of 0.03% and a 283.7-fold increase in specific activity. MALDI-TOF MS analysis determined the exact molecular weight of purified bacteriocin to be 3,326.1 Da.

Quality Characteristics of the Mashes of Takju Prepared Using Different Yeasts (효모종류를 달리한 탁주 술덧의 품질특성)

  • Lee, Heung-Sook;Park, Chang-Sook;Choi, Jin-Young
    • Korean Journal of Food Science and Technology
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    • v.42 no.1
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    • pp.56-62
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    • 2010
  • Quality characteristics such as alcohol, acid, and sugar contents of takju brewing mashes prepared using several yeasts were investigated during 12-days of fermentation. Among the yeasts examined, S. cerevisiae led to the highest level of ethanol (10.2-13.4%) and total minor alcohols (0.729-0.831 mg/mL). Regardless of the yeasts used, the acidity showed drastic changes (pH from 3.4-4.2 down to 2.4-2.7) during the first 2 days, and displayed negligible changes from day 4. The total acid contents rapidly increased to the first 4 days of fermentation and leveled off from 4 to the end of fermentation period. The common organic acid components were lactic, succinic and acetic acids. The total and reducing sugar contents varied depending on the yeasts used, with mashes prepared using S. coreanus and S. rouxii producing the maximum total sugar contents (5.43-5.5%) at the end of fermentation. The reducing sugar showed its maximum (7.53-14.89%) at day 2, after which it decreased to its minimum levels (3.04-4.52%). The common free sugar components were glucose and fructose, while S. ellipsoideus led to a higher free sugar level (0.35-5.29%).

A Comparative Study on Thermal and Belt Press Dewatering for Waterworks Sludge Rduction (열 탈수와 벨트프레스 탈수장치의 현장적용에 따른 탈수성 비교연구)

  • Lee, Jung-Eun
    • Journal of Korean Society of Environmental Engineers
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    • v.28 no.10
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    • pp.1016-1023
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    • 2006
  • The water content of dewatered cake produced from belt press dewatering equipment was about 75 wt% which was some high to handle it, so the equipment contained a limit at the economical and environmental aspect. The thermal dewatering equipment built as an alternative to overcome several problems was set up at the sludge treatment field and estimated some feasibility as comparison with the dewatering performance of belt press. First, dewatering properties of waterworks sludge was analyzed by monthly. The sludge of a water shortage season contained a high organic content which led to be difficult to dewater the cake, the other side the sludge of rainwater season was ease to dewater because of low organic content. According to the results to analysis the water content of dewatered cake produced from two equipments on the base of the seasonal dewatering properties, the water content of dewatered cake produced from thermal dewatering for sludge of water shortage season was $41.6{\sim}48.3$ wt% and $71{\sim}84$ wt% from belt press. In the case of rainwater season, the water content of dewatered cake produced from thermal dewatering was $34{\sim}37.7$ wt% and $57{\sim}70$ wt% from belt press. It was understood that thereduction of water content of cake by thermal dewatering was larger than belt press. The economical aspect for two equipments was evaluated on considering the reduction of cake treatment amount as the decrease of water content of cake. When putting the cost index of thermal dewatering into 100, belt press was 121. This meant that thermal dewater was more economical than belt press by about 20% in the side of construction and operation. In conclusion, thermal dewatering equipment was estimated by producing the low water content dewatered cake as well as being operated with low coat.

Characteristic Analysis of Tropospheric Ozone Sensitivity from the Satellite-Based HCHO/NO2 Ratio in South Korea (위성 기반 HCHO/NO2 비율을 통한 국내 대류권 오존 민감도 특성 분석)

  • Jinah Jang;Yun Gon Lee ;Jeong-Ah Yu;Kyoung-Hee Sung;Sang-Min Kim
    • Korean Journal of Remote Sensing
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    • v.39 no.5_1
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    • pp.563-576
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    • 2023
  • In this study nitrogen dioxide (NO2), formaldehyde (HCHO) from the Ozone Monitoring Instrument (OMI) and TROPOspheric Monitoring Instrument (TROPOMI), OMI/ Microwave Limb Sounder (MLS) tropospheric column ozone (TCO), and Airkorea ground-based O3 data were analyzed to examine the photochemical reaction relationship between tropospheric ozone and its precursors nitrogen oxides (NOx) and volatile organic compounds (VOCs). As a result of analyzing the trend of long-term changes from 2006 to 2020 using OMI satellite data, TCO showed an increasing trend, NO2 steadily decreased, and HCHO continued to increase in Northeast Asia. In addition, formaldehyde nitrogen dioxide ratio (FNR; HCHO/NO2 ratio), an indicator of ozone sensitivity, is gradually increasing, which means that the VOC-limited regime is decreasing. This study conducted a sensitivity analysis of ozone generation using TROPOMI FNR and ground-based ozone (O3) over the recent years (2019~2022) to identify the possible cause for the continuous increase of ozone in Korea. Similar to the previous studies, VOC-limited and transitional regimes appeared in megacities, and VOC-limited regimes also appeared in areas where major power plants were located. In VOC-limited regimes, in other words, areas where NOx is excessively saturated, the reduction in NOx emissions may have weakened the ozone titration and thus led to the increase of ozone. Therefore, VOC emissions should be reduced in the short term rather than NOx emissions to reduce ozone concentrations under the VOC-limited regime.

Classification of Carbon-Based Global Marine Eco-Provinces Using Remote Sensing Data and K-Means Clustering (K-Means Clustering 기법과 원격탐사 자료를 활용한 탄소기반 글로벌 해양 생태구역 분류)

  • Young Jun Kim;Dukwon Bae;Jungho Im ;Sihun Jung;Minki Choo;Daehyeon Han
    • Korean Journal of Remote Sensing
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    • v.39 no.5_3
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    • pp.1043-1060
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    • 2023
  • An acceleration of climate change in recent years has led to increased attention towards 'blue carbon' which refers to the carbon captured by the ocean. However, our comprehension of marine ecosystems is still incomplete. This study classified and analyzed global marine eco-provinces using k-means clustering considering carbon cycling. We utilized five input variables during the past 20 years (2001-2020): Carbon-based Productivity Model (CbPM) Net Primary Production (NPP), particulate inorganic and organic carbon (PIC and POC), sea surface salinity (SSS), and sea surface temperature (SST). A total of nine eco-provinces were classified through an optimization process, and the spatial distribution and environmental characteristics of each province were analyzed. Among them, five provinces showed characteristics of open oceans, while four provinces reflected characteristics of coastal and high-latitude regions. Furthermore, a qualitative comparison was conducted with previous studies regarding marine ecological zones to provide a detailed analysis of the features of nine eco-provinces considering carbon cycling. Finally, we examined the changes in nine eco-provinces for four periods in the past (2001-2005, 2006-2010, 2011-2015, and 2016-2020). Rapid changes in coastal ecosystems were observed, and especially, significant decreases in the eco-provinces having higher productivity by large freshwater inflow were identified. Our findings can serve as valuable reference material for marine ecosystem classification and coastal management, with consideration of carbon cycling and ongoing climate changes. The findings can also be employed in the development of guidelines for the systematic management of vulnerable coastal regions to climate change.

The Growth Characteristics and Germanium Uptake by Water Celery in Soil Treated with Germanium (게르마늄 처리 토양에서 미나리 생육 특성과 게르마늄 흡수)

  • Lee, Seong-Tae;Lee, Young-Han;Heo, Jae-Young;Hong, Kwang-Pyo;Dahlgren, Randy A.;Heo, Jong-Soo
    • Korean Journal of Environmental Agriculture
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    • v.27 no.2
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    • pp.185-190
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    • 2008
  • In order to obtain the basic information for agricultural utilization of Germanium(Ge), the growth characteristics and the germanium uptake by water celery were investigated at different concentration of germanium in soil. This experiment was carried out in the Wagner pot(1 $5,000^{-1}a$). Germanium concentrations in soil for water celery cultivation were maintained at 0.26, 25.0, 62.5, and 125.0 mg $kg^{-1}$, respectively. The treatment of over Ge 25.0 mg $kg^{-1}$ in the soil led to germanium phytotoxicity such as reduction of plant height and fresh weight. The contents of germanium in water celery were increased with the increase of germanium concentration in the soil. When water celery was cultivated from soil maintained with Ge 25.0 and 62.5 mg $kg^{-1}$, its germanium contents in plant were 89.9 and 371.6 mg $kg^{-1}$, respectively. Then, the efficiency of germanium uptake of water celery in Ge 25.0 and 62.5 mg $kg^{-1}$ maintained plots was 1.7 and 2.4%, respectively. When water celery was cultivated from soil maintained with Ge 25.0, 62.5 and 125.0 mg $kg^{-1}$, its content of amino acid was found to be 89.8, 198.4, and 318.2 mg $g^{-1}$, respectively. To investigate the effect of N fertilizer application in uptake of germanium by water celery, these were treated with nontreatment, 1.0, 1.5 and 2.0 times of N application based on soil testing for cultivation of water celery. However, the amount of the N fertilizer application did not affect the contents of germanium in the water celery. When water celery was cultivated from soil maintained with two kinds of inorganic and organic germanium 50 mg $kg^{-1}$, respectively, the content of germanium were 24.2 mg $kg^{-1}$ in the Ge-132 treatment and 11.8 mg $kg^{-1}$ in the $GeO_2$ treatment.

Green Spaces in the Urban Peripheries of Metropole Regions for Sustainable Development - Focused on Berlin, Milano and Seoul - (지속가능한 발전을 위한 대도시 외연부 녹지 활용 사례연구 - 베를린, 밀라노, 서울을 대상으로 -)

  • Hoh, Yun Kyeong;Chae, Jin-Hae
    • Journal of the Korean Institute of Landscape Architecture
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    • v.46 no.1
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    • pp.72-85
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    • 2018
  • This study focused on cases that led sustainable urban development through the construction and utilization of organic greenery systems linking green spaces of urban peripheries with metropolitan areas. To that end, Berlin - Brandenburg's regional parks in Germany and Milan's Raggi Verdi, a radial green axis project, in Italy were selected for analysis as case studies. As frameworks for this analysis, this study has established existing infrastructure accessibility and linkage, recycling and cooperative management. The results of the case study analysis are as follows: First, the specialized spatial strategy based on the individuality of the green space outside the city rather than a uniform landscape was used as the foundation of the sustainable development plan. Second, physical linkage from the center of the city to the periphery contributed to the sustainable development of the overall metropolis by improving the economic value of the surrounding area as well as ecological and environmental values. Third, the central management system was established to reduce the administrative inconvenience caused by multiple administrative districts in the green space of urban periphery. The implications of applying the above results to Seoul, Korea are as follows. First, it is necessary to establish a differentiation strategy by re-establishing the identity of a green landscape in the urban periphery, because the green spaces of Seoul's periphery are dispersed and mostly have a repetitious mountain landscape. Also, it is necessary to actively link peripheral mountains and urban green areas to create ecological value and economic value, and ultimately to help the sustainable development of the city. Finally, building an integrated management system is required to solve fragmented green space management departments in most of the urban periphery's green spaces. In conclusion, this study shows the significant possibility that the sustainable development of a metropolis can be derived from the utilization, linkage, and management of the green space in the urban peripheries, which is extraordinary compared to normal centralized urban development.

Study of Oil Palm Biomass Resources (Part 5) - Torrefaction of Pellets Made from Oil Palm Biomass - (오일팜 바이오매스의 자원화 연구 V - 오일팜 바이오매스 펠릿의 반탄화 연구 -)

  • Lee, Ji-Young;Kim, Chul-Hwan;Sung, Yong Joo;Nam, Hye-Gyeong;Park, Hyeong-Hun;Kwon, Sol;Park, Dong-Hun;Joo, Su-Yeon;Yim, Hyun-Tek;Lee, Min-Seok;Kim, Se-Bin
    • Journal of Korea Technical Association of The Pulp and Paper Industry
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    • v.48 no.2
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    • pp.34-45
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    • 2016
  • Global warming and climate change have been caused by combustion of fossil fuels. The greenhouse gases contributed to the rise of temperature between $0.6^{\circ}C$ and $0.9^{\circ}C$ over the past century. Presently, fossil fuels account for about 88% of the commercial energy sources used. In developing countries, fossil fuels are a very attractive energy source because they are available and relatively inexpensive. The environmental problems with fossil fuels have been aggravating stress from already existing factors including acid deposition, urban air pollution, and climate change. In order to control greenhouse gas emissions, particularly CO2, fossil fuels must be replaced by eco-friendly fuels such as biomass. The use of renewable energy sources is becoming increasingly necessary. The biomass resources are the most common form of renewable energy. The conversion of biomass into energy can be achieved in a number of ways. The most common form of converted biomass is pellet fuels as biofuels made from compressed organic matter or biomass. Pellets from lignocellulosic biomass has compared to conventional fuels with a relatively low bulk and energy density and a low degree of homogeneity. Thermal pretreatment technology like torrefaction is applied to improve fuel efficiency of lignocellulosic biomass, i.e., less moisture and oxygen in the product, preferrable grinding properties, storage properties, etc.. During torrefacton, lignocelluosic biomass such as palm kernell shell (PKS) and empty fruit bunch (EFB) was roasted under an oxygen-depleted enviroment at temperature between 200 and $300^{\circ}C$. Low degree of thermal treatment led to the removal of moisture and low molecular volatile matters with low O/C and H/C elemental ratios. The mechanical characteristics of torrefied biomass have also been altered to a brittle and partly hydrophobic materials. Unfortunately, it was much harder to form pellets from torrefied PKS and EFB due to thermal degradation of lignin as a natural binder during torrefaction compared to non-torrefied ones. For easy pelletization of biomass with torrefaction, pellets from PKS and EFB were manufactured before torrefaction, and thereafter they were torrefied at different temperature. Even after torrefaction of pellets from PKS and EFB, their appearance was well preserved with better fuel efficiency than non-torrefied ones. The physical properties of the torrefied pellets largely depended on the torrefaction condition such as reaction time and reaction temperature. Temperature over $250^{\circ}C$ during torrefaction gave a significant impact on the fuel properties of the pellets. In particular, torrefied EFB pellets displayed much faster development of the fuel properties than did torrefied PKS pellets. During torrefaction, extensive carbonization with the increase of fixed carbons, the behavior of thermal degradation of torrefied biomass became significantly different according to the increase of torrefaction temperature. In conclusion, pelletization of PKS and EFB before torrefaction made it much easier to proceed with torrefaction of pellets from PKS and EFB, leading to excellent eco-friendly fuels.

Antiproliferative Effect of Extracts, Fractions and Compound from Vitex rotundifolia on Human Cancer Cells (순비기 나무(Vitex rotundifolia) 추출.분획물 및 화합물의 인체 암세포 증식억제 효과)

  • Kim, You-Ah;Lee, Jung-Im;Kim, Hae-Jin;Kong, Chang-Suk;Nam, Taek-Jeong;Seo, Young-Wan
    • Journal of Applied Biological Chemistry
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    • v.52 no.4
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    • pp.180-186
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    • 2009
  • Whole plants of Vitex rotundifolia were extracted for 2 days with methylene chloride ($CH_2Cl_2$) followed by extraction of the residue for an additional 2 days. The same procedure was also applied using methanol (MeOH). The two crude extracts were combined and partitioned between $CH_2Cl_2$ and $H_2O$. The organic layer was further partitioned between n-hexane and 85% aq. MeOH, and the aqueous layer was also further fractionated with n-BuOH and $H_2O$, successively. From the 85% aq. MeOH fraction, one compound was isolated through the repeated HPLC. According to the results of physicochemical data including NMR and MS, the chemical structure of the compound was determined as artemetin (1). The antiproliferative effects of the crude extracts, fractions, and compound against HT1080, AGS, MCF-7 and HT-29 human cancer cells were compared with the control by using MTT assay. In the comparative analysis, the 85% aq. MeOH fraction exhibited the strongest antiproliferative effects on human cancer cell lines in a dose-dependent manner (p<0.05). In addition, exposure of compound 1 isolated from 85% aq. MeOH fraction led to strong antiproliferative effect in HT1080 cancer cell lines. These results suggest that the extracts and compound isolated from V. rotundifolia may be used as potential chemopreventive and chemotherapeutic agents.