• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.135-141
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• 2014

Objectives: Fabry disease (FD) is a lysosomal storage disease caused by the inappropriate accumulation of globotriaosylceramide (Gb3) in tissues due to a deficiency in the enzyme ${\alpha}$-galactosidase A. Hypertrophic cardiomyopathy is one of the chronic complications of FD. We tried to evaluate the prevalence of Fabry disease in the Korean patients with left ventricular hypertrophy (LVH). Methods: A total of 257 patients with LVH were recruited and they were 172 males (mean 56 years, range 30-81 years) and 84 females (mean 66 years, range 45-85 years). Urinary Gb3 was used to screen FD by high performance liquid chromatography-tandem mass spectrometry. Confirmatory tests were done by alpha-galactosidaseA activity using fluorometric assay and by GLA mutation analysis using sequencing. Results: Four patients were screening positive by urinary Gb3 analysis (cutoff, 25 ug/mmol creatinine). But, one female patient was diagnosed with FD confirmed by enzyme analysis in leukocytes as well as by genetic analysis (1/257 patients, 0.4%). She showed 54.3 ug/mmoL creatinine of Gb3 and 15.5 nmole/hr/mg protein (reference range, $55.2{\pm}12.7nmole/hr/mg$ protein) of alphagalactosidase A activity. And she had a heterozygous GLA mutation of c.796G>A (p.D266N). Her daughter was found to be a carrier for FD confirmed by GLA mutation analysis. Asymptomatic carrier showed 25.5ug/mmol creatinine of Gb3 and 42.5 nmole/hr/mg protein (reference range, $55.2{\pm}12.7nmole/hr/mg$ protein) of alpha-galactosidase A activity. Conclusions: The prevalence of FD in Koran patients with LVH was detected as 0.4%. Although the prevalence seems to be low, screening studies are of great importance for detecting hidden cases as well as for identifying other effected family members.

• Korean Journal of Agricultural and Forest Meteorology
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• v.12 no.4
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• pp.277-288
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• 2010

The multi-level $H_2O/CO_2$ profile system has been widely used to quantify the storage and advection effects on energy and mass fluxes measured by eddy covariance systems. In this study, we expanded the utility of the profile system by accommodating air sampling devices for isotope analyses of water vapor and $CO_2$. A pre-evacuated 2L glass flask was connected to the discharge of an Infrared Gas Analyzer (IRGA) of the profile system so that airs with known concentration of $H_2O$ and $CO_2$ can be sampled. To test the performance of this sampling system, we sampled airs from 8 levels (from 0.1 to 40 m) at the KoFlux tower of Gwangneung deciduous forest, Korea. Air samples in the 2L flask were separated into its component gases and pure $H_2O$ and $CO_2$ were extracted by using a vacuum extraction line. This novel technique successfully produced vertical profiles of ${\delta}D$ of $H_2O$ and ${\delta}^{13}C$ of $CO_2$ in a mature forest, and estimated ${\delta}D$ of evapotranspiration (${\delta}D_{ET}$) and ${\delta}^{13}C$ of $CO_2$ from ecosystem respiration (${\delta}^{13}C_{resp}$) by using Keeling plots. While technical improvement is still required in various aspects, our sampling system has two major advantages over other proposed techniques. First, it is cost effective since our system uses the existing structure of the profile system. Second, both $CO_2$ and $H_2O$ can be sampled simultaneously so that net ecosystem exchange of $H_2O$ and $CO_2$ can be partitioned at the same temporal resolution, which will improve our understanding of the coupling between water and carbon cycles in terrestrial ecosystems.

• Korean Journal of Air-Conditioning and Refrigeration Engineering
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• v.24 no.6
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• pp.521-537
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• 2012

This article reviews the papers published in the Korean Journal of Air-Conditioning and Refrigeration Engineering during 2011. It is intended to understand the status of current research in the areas of heating, cooling, ventilation, sanitation, and indoor environments of buildings and plant facilities. Conclusions are as follows. (1) Research trends of thermal and fluid engineering have been surveyed as groups of fluid machinery and fluid flow, thermodynamic cycle, and new and renewable energy. Various topics were presented in the field of fluid machinery and fluid flow. Research issues mainly focused on the rankine cycle in the field of thermodynamic cycle. In the new and renewable energy area, researches were presented on geothermal energy, fuel cell, biogas, reformer, solar water heating system, and metane hydration. (2) Research works on heat transfer area have been reviewed in the categories of heat transfer characteristics, pool boiling and condensing heat transfer, nanofluids and industrial heat exchangers. Researches on heat transfer characteristics included heat transfer above liquid helium surface in a cryostat, methane hydrate formation, heat and mass transfer in a liquid desiccant dehumidifier, thermoelectric air-cooling system, heat transfer in multiple slot impinging jet, and heat transfer enhancement by protrusion-in-dimples. In the area of pool boiling and condensing heat transfer, researches on pool boiling of water in low-fin and turbo-B surfaces, pool boiling of R245a, convective boiling two-phase flow in trapezoidal microchannels, condensing of FC-72 on pin-finned surfaces, and natural circulation vertical evaporator were actively performed. In the area of nanofluids, thermal characteristics of heat pipes using water-based MWCNT nanofluids and the thermal conductivity and viscosity were measured. In the area of industrial heat exchangers, researches on fin-tube heat exchangers for waste gas heat recovery and Chevron type plate heat exchanger were implemented. (3) Refrigeration systems with alternative refrigerants such as $CO_2$, hydrocarbons, and mixed refrigerants were studied. Heating performance improvement of heat pump systems were tried applying supplementary components such as a refrigerant heater or a solar collector. The effects of frost growth were studied on the operation characteristic of refrigeration systems and the energy performance of various defrost methods were evaluated. The current situation of the domestic cold storage facilities was analyzed and the future demand was predicted. (4) In building mechanical system fields, a variety of studies were conducted to achieve effective consumption of heat and maximize efficiency of heat in buildings. Various researches were performed to maximize performance of mechanical devices and optimize the operation of HVAC systems. (5) In the fields of architectural environment and energy, diverse purposes of studies were conducted such as indoor environment, building energy, and renewable energy. In particular, renewable energy and building energy-related researches have mainly been studied as reflecting the global interests. In addition, various researches have been performed for reducing cooling load in a building using spot exhaust air, natural ventilation and energy efficiency systems.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.45-52
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• 2007

Purpose : A simple, rapid, and highly sensitive analytical method for Gb3 in plasma was developed without labor-ex tensive pre-treatment by electrospray ionization MS/ MS (ESI-MS/MS). Measurement of globotriaosy lceramide (Gb3, ceramide trihex oside) in plasma has clinical importance for monitoring after enzyme replacement therapy in Fabry disease patients. The disease is an X-linked lipid storage disorder that results from a deficiency of the enzyme ${\alpha}$-galactosidase A (${\alpha}$-Gal A). The lack of ${\alpha}$-Gal A causes an intracellular accumulation of glycosphingolipids, mainly Gb3. Methods : Only simple 50-fold dilution of plasma is necessary for the extraction and isolation of Gb3 in plasma. Gb3 in diluted plasma was dissolved in dioxane containing C17:0 Gb3 as an internal standard. After centrifugation it was directly injected and analyzed through guard column by in combination with multiple reaction monitoring mode of ESI-MS/MS. Results : Eight isoforms of Gb3 were completely resolved from plasma matrix. C16:0 Gb3 occupied 50% of total Gb3 as a major component in plasma. Linear relationship for Gb3 isoforms w as found in the range of 0.001-1.0 ${\mu}g$/mL. The limit of detection (S/N=3) was 0.001 ${\mu}g$/mL and limit of quantification was 0.01 ${\mu}g$/mL for C16:0 Gb3 with acceptable precision and accuracy. Correlation coefficient of calibration curves for 8 Gb3 isoforms ranged from 0.9678 to 0.9982. Conclusion : This quantitative method developed could be useful for rapid and sensitive 1st line Fabry disease screening, monitoring and/or diagnostic tool for Fabry disease.

• Korean Journal of Air-Conditioning and Refrigeration Engineering
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• v.20 no.6
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• pp.427-446
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• 2008

A review on the papers published in the Korean Journal of Air-Conditioning and Refrigeration Engineering in 2006 has been accomplished. Focus has been put on current status of research in the aspect of heating, cooling, ventilation, sanitation and building environments. The conclusions are as follows. (1) The research trends of fluid engineering have been surveyed as groups of general fluid flow, fluid machinery and piping, etc. New research topics include micro heat exchanger and siphon cooling device using nano-fluid. Traditional CFD and flow visualization methods were still popular and widely used in research and development. Studies about diffusers and compressors were performed in fluid machinery. Characteristics of flow and heat transfer and piping optimization were studied in piping systems. (2) The papers on heat transfer have been categorized into heat transfer characteristics, heat exchangers, heat pipes, and two-phase heat transfer. The topics on heat transfer characteristics in general include thermal transport in a cryo-chamber, a LCD panel, a dryer, and heat generating electronics. Heat exchangers investigated include pin-tube type, plate type, ventilation air-to-air type, and heat transfer enhancing tubes. The research on a reversible loop heat pipe, the influence of NCG charging mass on heat transport capacity, and the chilling start-up characteristics in a heat pipe were reported. In two-phase heat transfer area, the studies on frost growth, ice slurry formation and liquid spray cooling were presented. The studies on the boiling of R-290 and the application of carbon nanotubes to enhance boiling were noticeable in this research area. (3) Many studies on refrigeration and air conditioning systems were presented on the practical issues of the performance and reliability enhancement. The air conditioning system with multi indoor units caught attention in several research works. The issues on the refrigerant charge and the control algorithm were treated. The systems with alternative refrigerants were also studied. Carbon dioxide, hydrocarbons and their mixtures were considered and the heat transfer correlations were proposed. (4) Due to high oil prices, energy consumption have been attentioned in mechanical building systems. Research works have been reviewed in this field by grouping into the research on heat and cold sources, air conditioning and cleaning research, ventilation and fire research including tunnel ventilation, and piping system research. The papers involve the promotion of efficient or effective use of energy, which helps to save energy and results in reduced environmental pollution and operating cost. (5) Studies on indoor air quality took a great portion in the field of building environments. Various other subjects such as indoor thermal comfort were also investigated through computer simulation, case study, and field experiment. Studies on energy include not only optimization study and economic analysis of building equipments but also usability of renewable energy in geothermal and solar systems.

• KOREAN JOURNAL OF CROP SCIENCE
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• v.66 no.4
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• pp.383-391
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• 2021

To develop a cultivation method for the mass production of sweetpotato cultivars, 'Juhwangmi' (orange tuber) and 'Sinjami' (puple tuber), the yield of tuber roots and content of various functional substances were analyzed according to planting intervals and growing periods. For 'Juhwangmi, the total yield of tubers was increased by respectively 36% and 54% and the yield of tubers over 300 g was increased by respectively 170% and 221% in the 140-day and 160-day cultivation plots compared with that in the 120-day cultivation plot at the 70×20 cm planting interval. Similarly, the total content of beta-carotene in the tubers increased as the cultivation period was extended. In particular, beta-carotene content at the 70×20 cm planting interval was the highest. For 'Sinjami', at the same planting interval, the total yield of tubers and yield of tubers over 300 g significantly increased as the growing period was extended. Within the same cultivation period, the yield of tubers over 300 g and the total anthocyanin content of 'Sinjami' were higher at the 70×30 and 70×35 cm planting intervals than at the 70×20 and 70×25 cm planting intervals in the 140-day and 160-day cultivation plots. Moreover, the total polyphenol and flavonoid content was significantly higher in 'Sinjami' than in 'Juhwangmi', and the values were the highest in the 160-day cultivation plots. In particular, the content of these two functional substances in tubers over 300 g was the highest at the 70×30 and 70×35 cm planting intervals.

• Korean Chemical Engineering Research
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• v.59 no.3
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• pp.345-358
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• 2021

This study focuses on the development of the liquid air production process that uses LNG (liquefied natural gas) cold energy which usually wasted during the regasification stage. The liquid air can be transported to the LNG exporter, and it can be utilized as the cold source to replace certain amount of refrigerant for the natural gas liquefaction. Therefore, the condition of the liquid air has to satisfy the available pressure of LNG storage tank. To satisfy pressure constraint of the membrane type LNG tank, proposed process is designed to produce liquid air at 1.3bar. In proposed process, the air is precooled by heat exchange with LNG and subcooled by nitrogen refrigeration cycle. When the amount of transported liquid air is as large as the capacity of the LNG carrier, it could be economical in terms of the transportation cost. In addition, larger liquid air can give more cold energy that can be used in natural gas liquefaction plant. To analyze the effect of the liquid air production amount, under the same LNG supply condition, the proposed process is simulated under 3 different air flow rate: 0.50 kg/s, 0.75 kg/s, 1.00 kg/s, correspond to Case1, Case2, and Case3, respectively. Each case was analyzed thermodynamically and economically. It shows a tendency that the more liquid air production, the more energy demanded per same mass of product as Case3 is 0.18kWh higher than Base case. In consequence the production cost per 1 kg liquid air in Case3 was $0.0172 higher. However, as liquid air production increases, the transportation cost per 1 kg liquid air has reduced by $0.0395. In terms of overall cost, Case 3 confirmed that liquid air can be produced and transported with $0.0223 less per kilogram than Base case.