• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Horticultural Science & Technology
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• v.33 no.4
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• pp.501-510
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• 2015

In this study, we investigated the onset and release of endo-dormancy under natural conditions by observing bud break characteristics in 'Fuji' apple trees using water cuttings. Through examinations of bud break rate and days to bud break, we found that the endo-dormancy of 'Fuji' apple tree continues for 70 d from 165 to 255 d after full bloom (DAFB), from late October to early January of the following year. In addition, within 20 d of first bud break, based on a final bud break rate of 60% or more, we able to identify the timing of the changeover from para-dormancy to endo-dormancy, and endo-dormancy to eco-dormancy. Analysis of the chilling requirement during the endo-dormancy period revealed that chilling accumulation up to 255 DAFB to release endo-dormancy amounted to 666 and 517 h based on the CH and Utah models, respectively. Observation of internal changes in the bud during endo-dormancy showed that flower bud differentiation begins from mid-July, and t ime of inflorescence o f the disk f lower is a vailable to f ind. The f lower buds subsequently developed slowly but steadily during endo-dormancy and in the following year in February, the developmental stage of each organ had progressed. Moreover, the flower buds of 'Fuji' apples were mostly healthy during the dormancy period, but some exhibited necrosis of flower primordium, due partial cell damage from the formation of ice crystals rather than a direct effect of the low temperature. Flower buds were formed in both the axillary buds of bourse shoots and terminal buds of spurs, but lower bud differentiation was observed for the terminal buds of spurs at rate of about 65% of total buds, which was directly related to the bud size and shoot diameter.

• International Journal of Highway Engineering
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• v.6 no.3 s.21
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• pp.1-7
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• 2004

Roads, like bikeways, parkways and walks, are to be just capable of supporting light traffic and traveling public, but they are required to be human friendly and environmental-oriented. Lately soil-solidifier mixture, a kind of soil-cement, has developed and has been applied to the recycling and environment-oriented pavement as the surfacing material. Soil-solidifier pavement structure has been designed by only experience. To design this pavement mechanically, it is necessary to find out basic engineering properties of soil-solidifier mixture. This study focuses on finding out mechanical characteristics of the mixture according to mixture proportions and aging. Test molds with various mixture proportions are made, and then unconfined strength tests are performed for test molds with aging of the mixture. As the result of this study, it is found that the strength of the mixture increases with amount of cement and that maximum strength is achieved at 6%$\sim$8% of the ratio of solidifier and water. The strength increase rapidly until 14 days, after then slowly. After 28 days the strength of the mixture approaches to the constant value. The heat of hydration during curing of the mixture is measured no significantly. It also shows that temperature characteristics of the mixture is similar to that of soil. Since this mixture is mixed with soil and is able to improve engineering problems in pavement due to temperature, this mixture is expected to use effectively in the environment-oriented pavement for light traffic.

• The Journal of Korean Academy of Prosthodontics
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• v.48 no.2
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• pp.122-127
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• 2010

Purpose: The objective of this study was to investigate the effects of calcium phosphate coated titanium implant surface on bone response and implant stability at early stage of healing period of 3 weeks and later healing period of 6 weeks. Material and methods: A total of 24 machined, screw-shaped implants (Dentium Co., Ltd., Seoul, Korea) which dimensions were 3.3 mm in diameter and 5.0 mm in length, were used in this research. All implants (n = 24), made of commercially pure (grade IV) titanium, were divided into 2 groups. Twelve implants (n = 12) were machined without any surface modification (control). The test implants (n = 12) were anodized and coated with thin film (150nm) of calcium phosphate by electron-beam deposition. The implants were placed on the proximal surface of the rabbit tibiae. The bone to implant contact (BIC) ratios was evaluated after 3 and 6 weeks of implant insertion. Results: The BIC percentage of calcium phosphate coated implants ($70.8{\pm}18.9%$) was significantly higher than that of machined implants ($44.1{\pm}16.5%$) 3 weeks after implant insertion (P = 0.0264). However, there was no significant difference between the groups after 6 weeks of healing (P > .05). Conclusion: The histomorphometric evaluation of implant surface revealed that; 1. After 3 weeks early healing period, bone to implant contact (BIC) percentage of calcium phosphate coated implants (70.8%) was much greater than that of surface untreated machined implants (44.1%) with P = 0.0264. 2. After 6 weeks healing period, however, BIC percentage of calcium phosphate coated implants group (79.0%) was similar to the machined only implant group (78.6%). There was no statistical difference between two groups (P = 0.8074). 3. We found the significant deference between the control group and experimental group during the early healing period of 3 weeks. But no statistical difference was found between two groups during the later of 6 weeks.

• Journal of Bio-Environment Control
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• v.26 no.3
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• pp.201-207
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• 2017

Objective of this research was to investigate the influence of pre-plant nitrogen levels in root media on plug seedling growth of radish cv. Soksungbommu. To achieve the research purpose, a root medium, the mixture of perlite, coir dust, and peatmoss (volume percentage of 30:35:35) was formulated and the N levels incorporated during mixing were controlled to 0, 100, 250, 500, 750, 1,000, and $1,500mg{\cdot}L^{-1}$. Then, the seeds were sown into 72-cell plug trays in which the root medium was packed. The measurements of growth and analysis of tissue and root media were conducted 2 and 4 weeks after sowing. Elevation of pre-plant N levels raised EC and turned down pH of root media. But, as seedling grew, the pH rose and EC get lowered in all treatments. The EC as well as $NH_4-N$ and $NO_3-N$ concentrations of root media declined gradually until week 2, but those declined sharply between weeks 2 to 4. The seedling growth 2 weeks after sowing showed quadratic response to pre-plant N levels with the highest growth in $250mg{\cdot}L^{-1}$ treatment and lagging growth in the treatments of lower or higher N levels than $250mg{\cdot}L^{-1}$. The seedling growth 4 weeks after sowing showed also quadratic response with the highest growth in $500mg{\cdot}L^{-1}$ treatment. The tissue N contents were get higher and those of K, Ca, and Mg were get lower as pre-plant N levels were elevated. Above results suggest that lower than $250mg{\cdot}L^{-1}$ in pre-plant N levels is optimistic for growth of plug seedling and avoidance of toxic injury in very young stage.

• Journal of Bio-Environment Control
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• v.26 no.3
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• pp.151-157
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• 2017

The objectives of this study were to determine the impact of soil water content on the growth, stomatal conductance, and photosynthesis of Kimchi cabbage and to evaluate proper parameters for development of growth models. There were five levels of irrigation amount treatments (0, 200, 300, 400, and 500 mL/d/plant) and those were commenced at one day after transplanting (DAT). We measured soil water content, stomatal conductance, photosynthesis characteristics, and the A-Ci curve. The growth of Kimchi cabbage as affected by irrigation amount was evaluated at 38 days after transplanting, however, the growth with 0 and 200 mL/d/plant irrigation amount treatments measured at 29 DAT. The relationship between soil water content and stomatal conductance was highly correlated ($r^2=0.999$) and the function represented by y = 6097.4x - 4.2984. The stomatal conductance of Kimchi cabbage leaves showed $300mmol{\cdot}m^{-2}{\cdot}s^{-1}$ when the soil water content was below $0.05m^3/m^3$. The stomatal conductance was rapidly decreased by scarcity of soil moisture. A-Ci curve indicated normal curve in fully irrigation treatment (500 mL/d/plant), however, $CO_2$ couldn't diffuse through the intercellular Kimchi cabbage leaves treated with 0 mL/d/plant. The dry weight of full irrigation treatment was greater approximately 6.8 times than that of deficit irrigation (0 mL/d/plant). In addition, leaf area index showed a logarithmic function (y = 16.573 + 3.398 ln x) with soil water content and that of R-squared represents 0.913. Results indicated that the soil water content was highly correlated with stomatal conductance and leaf area index. Indeed, the scarcity soil moisture reduced photosynthesis and retarded growth.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.215-221
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• 2010

Purpose : Interleukin-17 (IL-17) is produced by activated CD4+T cells and exhibits pleiotropic biological activity on various cell types. IL-17 was reported to be involved in the immunoregulatory response in IgA nephropathy (IgAN). Our aim was to investigate the association between single-nucleotide polymorphisms (SNPs) in IL-17 receptor A (IL-17RA) gene and childhood IgAN. Methods : We analyzed the SNPs in the IL-17RA in 156 children with biopsy-proven IgAN and 245 healthy controls. We divided the IgAN patients into 2 groups and compared them with respect to proteinuria (${\leq}4$ and >$4mg/m^2/h$, ${\leq}40$ and >$40mg/m^2/h$, respectively) and the presence of pathological levels of biomarkers of diseases such as interstitial fibrosis, tubular atrophy, or global sclerosis. Results : No difference was observed between the SNP genotypes rs2895332, rs1468488, and rs4819553 between IgAN patients and control subjects. In addition, no significant difference was observed between allele frequency of SNPs rs2895 332, rs1468488, and rs4819553 between patients in the early and advanced stage of the disease. However, significant difference was observed between the genotype of SNP rs2895332 between patients with proteinuria (>$4mg/m^2/h$) and those without proteinuria (codominant model OR 0.36, 95% CI 0.19-.66, P <0.001; dominant model OR 0.35, 95% CI 0.17-.69 P =0.002; recessive model OR 0.12, 95% CI 0.01-.06 P =0.025). Conclusion : Our results indicate that the SNP in IL-17RA (rs2895332) may be related to the development of proteinuria in IgAN patients.

• Journal of the Korea Organic Resources Recycling Association
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• v.27 no.4
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• pp.15-24
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• 2019

The study was compared for soil chemical and microbial properties as well as growth of the cherry tomato (Lycopersicon esculentum var. cerasiforme) plants environmentally friendly gown for 3 years and 5 years, which had been fertigated with homemade liquid fertilizer (LF) with indigenous microorganism as an additional fertilizer. Treatment included LF with indigenous microorganism for 3 years (3-year IM-LF) and for 5 years (5-year IM-LF), with an effective microorganism for 10 years (EM-LF), which had been applied with 1,000 times of dilution in the farmhouse. IM-LF and EM-LF materials had increased pH pattern for 16 weeks, in particular for increase of 1.2 for EM-LF. IM-LF material contained slightly higher EC but similar level of 0.2 dS/m to EM-LF. For a pot experiment in the greenhouse, IM-LF treatment increased root dry weight of the cherry tomato plants. In the farmhouse experiment, IM-LF treatment increased to 7.5 of soil pH and 8.4 dS/m of EC, indicating high salt accumulation. EM-LF treatment increased to 62 g/kg of soil OM, which would have affected concentrations of macro essential nutrients, including T-N in the soil. However, the optimum soil chemical levels for growth of cherry tomato plants were observed on the IM-LF plots. EM-LF treatment increased number of bacteria and actinobacteria in the soil. EM-LF treatment increased concentrations of macro essential nutrients in the plants, except for P, with similar nutrient concentrations observed between 3-year IM-LF and 5-year IM-LF-treated plants. Leaf SPAD and PS II levels decreased in the plants treated with 3-year IM-LF. EM-LF treatment increased leaf width and length, number of leaves, canopy area, plant height, and stem diameter in the mid-term stage of growth, which were not significantly different between the treatments. EM-LF treated-plants had two times higher leaf dry weight than those of values observed on the IM-LF plants, which was the opposite result observed on the number of fruit.

• Asian-Australasian Journal of Animal Sciences
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• v.31 no.4
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• pp.548-555
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• 2018

Objective: This experiment was conducted to investigate whether asparagine (Asn) could improve liver energy status in weaning pigs when challenged with lipopolysaccharide. Methods: Forty-eight weaned pigs ($Duroc{\times}Large\;White{\times}Landrace$, $8.12{\pm}0.56kg$) were assigned to four treatments: i) CTRL, piglets received a control diet and injected with sterile 0.9% NaCl solution; ii) lipopolysaccharide challenged control (LPSCC), piglets received the same control diet and injected with Escherichia coli LPS; iii) lipopolysaccharide (LPS)+0.5% Asn, piglets received a 0.5% Asn diet and injected with LPS; and iv) LPS+1.0% Asn, piglets received a 1.0% Asn diet and injected with LPS. All piglets were fed the experimental diets for 19 d. On d 20, the pigs were injected intraperitoneally with Escherichia coli LPS at $100{\mu}g/kg$ body weights or the same volume of 0.9% NaCl solution based on the assigned treatments. Then the pigs were slaughtered at 4 h and 24 h after LPS or saline injection, and the liver samples were collected. Results: At 24 h after LPS challenge, dietary supplementation with 0.5% Asn increased ATP concentration (quadratic, p<0.05), and had a tendency to increase adenylate energy charges and reduce AMP/ATP ratio (quadratic, p<0.1) in liver. In addition, Asn increased the liver mRNA expression of pyruvate kinase, pyruvate dehydrogenase, citrate synthase, and isocitrate dehydrogenase ${\beta}$ (linear, p<0.05; quadratic, p<0.05), and had a tendency to increase the mRNA expression of hexokinase 2 (linear, p<0.1). Moreover, Asn increased liver phosphorylated AMP-activated protein kinase (pAMPK)/total AMP-activated protein kinase (tAMPK) ratio (linear, p<0.05; quadratic, p<0.05). However, at 4 h after LPS challenge, Asn supplementation had no effect on these parameters. Conclusion: The present study indicated that Asn could improve the energy metabolism in injured liver at the late stage of LPS challenge.

• Journal of Nutrition and Health
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• v.24 no.6
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• pp.506-515
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• 1991

Serum concentration of selenium were measured ; Studying 36 patients with Hepatoma. 19 with Hepatitis 15 with Liver cirrhosis and 67 Normal control to discover the correlation between serum selenum and cancer. And it have tested also the serum protein and albumin to compare the nutrition status by researching the same persons above The results of those two experiments are as follow : 1) The mean of each group of patients resulting from mearsuing the serum protein content showing 6.91$\pm$0.10g/dl in the patients with hepatoma 7.05$\pm$0.17g/dl in the ones with hepatitis 6.42$\pm$0.13g/dl in the ones with liver cirrhosis and 7.46$\pm$0.06g/dl in the normal control is all being within the normal range such as 6.0-0.8(g/dl) while the distribution is revealin 10.5%, 13.3% in the patients with hepatitis and liver cirrhosis which are below the normal range and 47.2%, 96.9%, 80.0% in the low range of normal one ; therefore in could see that patientsw compared with normal control are in low level in the serum protein content 2) The mean based on the consquence of serum albumin measurement is 3.74$\pm$0.11g/dl. 4.26$\pm$0.13g/dl 4.73$\pm$0.06g/dl in the patients with hepatoma hepatits and normal control which are in between the normal range such as 3.5-5.5(g/dl) except the patients with liver cirrhosis(3/05$\pm$0.12g/dl) while the distribution of the patients with hepatoma an liver cirrhosis is showing 27.8% 73.3% which are below the normal one and 55.5%, 52.6% and 26.7% in the patients with hepatoma hepatits and liver cirrhosis which are in the low area of normal range ; as a result It could find that patients. compared with the normal control are in low level in serum albumin measurement. 3) The mean consulting the serum concentration is 430.86$\pm$26.06$\mu\textrm{g}$/1, 192.37$\pm$18.15$\mu\textrm{g}$/1 186.73$\pm$23.99$\mu\textrm{g}$/1, 196.94$\pm$8.94$\mu\textrm{g}$/1 in the patients with hepatoma hepatitis liver cirrhosis and noramal control ; it shows 2.19 times increasing of serum selenium concentra-tion in the patients with hepatoma 'compared with the normal control and patients with hepatoma spread out at a still higher range in serum selenium concentration test. 4) The serum selenium concentration is not quite dependent on sexes and doesn't show any correlation with age, serum protein, serum albumin. The nutrition status of patients, rested on the results of those experiments, stays in low level, which is caused by that they are in low stage of serum protein and serum albu¬min. In serum selenium concentration, patients with hepatitis and liver cirrhosis, specially, show the condition as good as normal control's; while patients with hepatoma are indicating a significant increasing compared with the three groups, which can be reasoned into that there is a possibility for the patients to take selenium and chemotherapy or radiation treatment, and have high serum selenium concentration by poor excreting of seleno methionine, which is caused by low serum protein content And another thing can be reasoned is that the difference in origination mechanism hetween korean and western people. As a result, It showed that the correlation between serum selenium and seses, age, serum protein, and serum albumin neither exists nor affects on each other.