• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.17 no.3
• /
• pp.85-91
• /
• 2017

Purpose: The aim of this study was to describe the clinical and biochemical features as well as the molecular analysis of a newly diagnosed illustrative case with ML II and to analyze the clinical features of 11 Korean patients with ML II/III. Method: Including a newly diagnosed patient, total 11 patients in 10 families were diagnosed as ML II (n=7) or ML III (n=4) were enrolled in the study. A diagnosis of ML II or III was made by demonstrating increased lysosomal enzyme activities in the plasma and sequence analysis of GNPTAB with characteristic clinical features. Result: A illustrative case of ML II patient was a 17 month-old boy showing characteristic facial appearance, multiple joint contractures with cardiac involvements. The enzyme assay showed increased lysosomal enzyme activities in the plasma. We identified compound heterozygous mutations in GNPTAB sequence analysis, including a frameshift (c.3428dupA [pAsn1143Lysfs*3]) and a nonsense variant c.673C>T (p.Gln225*). In total 11 patients with ML II/III, the patients with ML II showed severe growth retardation (height standard deviation score -3.2 [${\pm}1.5$]), compare to patients with ML III. Furthermore, patients with ML II patients had serious cardiac problem (n=4), hepatomegaly (n=3) and underwent tracheostomy (n=3) with further respiratory support due to respiratory distress. To improve osteoporosis and bone pain, all patients with ML III and four of 7 patients with ML II treated with intravenous pamidronate. Conclusion: Here we showed a newly diagnosed case of ML II and clinical features of 11 Korean patients with ML II or III. These data could be helpful for further diagnosis of mucolipidosis, a rare inherited metabolic disease, in Korea.

• Journal of Life Science
• /
• v.25 no.11
• /
• pp.1214-1222
• /
• 2015

The purpose of this study was to determine the effect of Pueraria lobate-root based combination supplementation containing Rehmannia glutinosa and exercise on histone modification in ovariectomized rat hindlimb skeletal muscle. Sixty rats were fed with high fat diet and randomly assigned into the following groups for 8 weeks: 1)HSV; High fat+Sedentary+Vehicle, 2)HSP; High fat+Sedentary+PR, 3)HSH; High fat+Sedentary+Estradiol, 4)HEV; High fat+Ex+Vehicle, 5)HEP; High fat+Ex+PR, 6)HEH; High fat+Ex+Estradiol. Exercise consisted of low intensity treadmill exercise(1-4th wk:15 m/min for 30 min, 5-8th wk: 18 m/min for 40 min, 5 times/week). The result of this study showed that exercise and Pueraria and Rehmannia glutinosa intake suppressed weight gain. Furthermore, exercise and Pueraria and Rehmannia glutinosa intake increased muscle mass. This study observed H3K9 acetylation and demethylation in plantaris muscle in exercised group, but no difference in soleus muscle. To test whether the decrease in HDAC4, HDAC5 and G9a mRNA levels after exercise and Pueraria/Rehmannia glutinosa intake, HDAC4, HDAC5 and G9a mRNA levels were determined by real-time PCR. Only exercise induced HDAC5 and G9a mRNA reduction in plantaris muscle, but not in soleus muscle. In conclusion, these data demonstrates that exercise and Pueraria/Rehmannia glutinosa intake effect on body compositions. These changes are regulated by epigenetic modifications, such as histone acetylation and methylation. Future studies should focus on gene-specific epigenetics and other epigenetic mechanism for Pueraria/Rehmannia glutinosa intake.

• Journal of Life Science
• /
• v.22 no.1
• /
• pp.16-24
• /
• 2012

Polyamines are essential for cell growth and differentiation; however their precise roles are unclear yet. In the present study, the cytotoxic effect of spermine (spm) on MCF-7 cells was investigated. In the MTT assay of MCF-7 cells treated with spm, cell viability was significantly decreased in a time-and dose-dependent manner. Cell viability measurement was confirmed by trypan blue staining. FACS analysis shows that sub-G1 was increased in a time-and dose-dependent manner too. When the cells were treated with spm, cells started to show morphological changes within 2 hrs. The expression of adhesion proteins (FAK and integrin ${\beta}1$), and cytoskeletal protein (actin) was checked by Western blotting analysis. Integrin ${\beta}1$ levels were slightly decreased, and FAK and actin levels were rapidly decreased with spm treatment. In confocal laser scanning microscopy, the distribution of actin did not change but the expression decreased in a dose-dependent manner with spm treatment. FAK was evenly distributed under the plasma membrane in the untreated control. However, at 10 ${\mu}M$ spm FAK seemed to move toward the cell nucleus. Integrin ${\beta}1$, which was mainly found in the focal point of the plasma membrane in the untreated control, dispersed through the entire plasma membrane in spm treatment. The present results indicate that cytotoxic effects of spm are triggered by the disruption of adhesion proteins and cytoskeletal protein.

• Clinical and Experimental Reproductive Medicine
• /
• v.26 no.2
• /
• pp.163-170
• /
• 1999

Objectives: The aims of this study are 1) to determine if GAST is a better indicator in predicting ovarian response to COH compared with patient's age or basal FSH level and 2) to evaluate its role in detecting abnormal ovarian response. Design: Prospective study in 118 patients undergoing IVF-ET using GnRH-a short protocol during May-September 1995. Materials and Methods: After blood sampling for basal FSH and estradiol $(E_2)$ on cycle day two, 0.5ml (0.525mg) GnRH agonist ($Suprefact^{(r)}$, Hoechst) was injected subcutaneously. Serum $E_2$ was measured 24 hours later. Initial $E_2$ difference $({\Delta}E_2)$ was defined as the change in $E_2$ on day 3 over the baseline day 2 value. Sixteen patients with ovarian cyst or single ovary or incorrect blood collection time were excluded from the analysis. The patients were divided into three groups by ${\Delta}E_2$; group A (n=30):${\Delta}E_2$<40 pg/ml, group B (n=52): 40 pg/ml${\leq}{\Delta}E_2$<100 pg/ml, group C (n=20): ${\Delta}E_2{\leq}100$ pg/ml. COH was done by GnRH agonist/HMG/hCG and IVF-ET was followed. Ratio of $E_2$ on day of hCG injection over the number of ampules of gonadotropins used ($E_2hCGday$/Amp) was regarded as ovarian responsiveness. Poor ovarian response and overstimulation were defined as $E_2$ hCGday less than 600 pg/ml and greater than 5000 pg/ml, respectively. Results: Mean age $({\pm}SEM)$ in group A, B and C were $33.7{\pm}0.8^*,\;31.5{\pm}0.6\;and\;30.6{\pm}0.5^*$, respectively ($^*$: p<0.05). Mean basal FSH level of group $A(11.1{\pm}1.1mlU/ml)$ was significantly higher than those of $B(7.4{\pm}0.2mIU/ml)$ and C $(6.8{\pm}0.4mIU/ml)$ (p<0.001). Mean $E_2hCGday$ of group A was significantly lower than those of group B or C, i.e., $1402.1{\pm}187.7pg/ml,\;3153.2{\pm}240.0pg/ml,\;4078.8{\pm}306.4pg/ml$ respectively (p<0.0001). The number of ampules of gonadotropins used in group A was significantly greater than those in group B or C: $38.6{\pm}2.3,\;24.2{\pm}1.1\;and\;18.5{\pm}1.0$ (p<0.0001). The number of oocytes retrieved in group A was significantly smaller than those in group B or C: $6.4{\pm}1.1,\;15.5{\pm}1.1\;and\;18.6{\pm}1.6$, respectively (p<0.0001). By stepwise multiple regression, only ${\Delta}E_2$ showed a significant correlation (r=0.68, p<0.0001) with $E_2HCGday$/Amp, while age or basal FSH level were not significant. Likewise, only ${\Delta}E_2$ correlated significantly with the number of oocytes retrieved (r=0.57, p<0.001). All four patients whose COH was canceled due to poor ovarian response belonged to group A only (Fisher's exact test, p<0.01). Whereas none of 30 patients in group A (0%) had overstimulation, 14 patients among 72 patients (19.4%) in group B and C had overstimulation (Fisher's exact test, p<0.01). Conclusions: These data suggest that initial $E_2$ difference after GAST may be a better prognostic indicator of ovarian response to COH than age or basal FSH level. Since initial $E_2$ difference demonstrates significant association with abnormal ovarian response such as poor ovarian response necessitating cycle cancellation or overstimulation, GAST may be helpful in monitoring and consultation of patients during COH in IVF-ET cycle.

• Journal of dental hygiene science
• /
• v.14 no.1
• /
• pp.7-14
• /
• 2014

This study aims to evaluate the impact of varying exposure to dental amalgam on urinary mercury levels in children by measuring the number of amalgam-filled teeth and the variance of mercury concentration in urine over a period of 2 years. A total of 317 (male 158, female 159) elementary school children (1st~4th graders) attending 2 schools in urban regions participated in this study. At 6-month intervals, 4 oral examinations were conducted to check any variance in the conditions of dental caries and the status of dental fillings. Also, urine tests were conducted followed by a questionnaire survey. To elucidate the factors potentially affecting the mercury concentration in urine, t-test, ANOVA, chi-square test and a mixed model were used for the analysis. Regarding the status of dental fillings in line with examination time periods, deciduous teeth had more amalgam-filled surfaces than those filled with resin, whereas permanent teeth had more resin-filled surfaces than those filled with amalgam. A significant relevance was found between the exposure to dental amalgam and urinary mercury levels. Specifically, subjects whose teeth surfaces had been filled with dental amalgam showed higher urinary mercury levels than those who had no dental amalgam fillings. Based on the analysis using the mixed model, the increase in the number of teeth surfaces filled with amalgam was found to be the factor affecting the increase in urinary mercury levels. The urinary mercury levels were found to be highly associated with the exposure to dental amalgam. The more the teeth surfaces filled with amalgam, the higher the urinary mercury levels. Hence, even a trace of dental amalgam fillings can liberate mercury, affecting the variance in the urinary mercury levels. These findings suggest that some criteria or measures should be developed to minimize the exposure to dental amalgam. Moreover, relevant further studies are warranted.

• Korean Journal of Ecology and Environment
• /
• v.41 no.3
• /
• pp.283-293
• /
• 2008

The objectives of the study were firstly to provide fundamental data for establishment of total maximum daily load (TMDL) management in Ulsan City, and secondly to make practical application of stream health assessment with tolerance range by each species when physiochemical and fish investigations were carried out together. A total of 44 sites in Taehwa River, Hoiya River, Dong Stream, and Cheongryang Stream were selected to monitor water qualities seasonally and fish investigation was performed in August 2006. Among the parameters of water quality, biological oxygen demand (BOD) and dissolved oxygen (DO) related to respiration, total nitrogen (T-N) and total phosphorus (T-P) related to nutrient and eutrophication, and total suspended solids (TSS) and $NH_4$-N were compared with vertical box plot by 8 dominant species. According to the fish investigation, 12 families 33 species were found including endangered species (Pungitius kaibarae) and introduced species (Lepomis macrochirus, Micropterus salmoides), and appearance rate of Korean endemic species was greater in Taehwa River (29.2%) than others. As the results of tolerance range by species, Zacco koreanus, Rhynchocypris oxycephalus, Iksookimia longicorpa, and Squalidus gracilis majimae had limited low range by water quality parameters indicating preference of good water quality. Whereas, Carassius auratus and Pseudorasbora parva were found downstream and urban-streams which were exposed from frequent inflow of pollutants. It concludes that the results help distinguishing sensitive, intermediate, and tolerant species when we evaluate stream health assessment with fish, and further making practical application for conservation and restoration of aquatic ecosystem.

• Archives of design research
• /
• v.19 no.6 s.68
• /
• pp.9-14
• /
• 2006

There are many paintings describing butterfly in a folk story or old story but the word of Nabby is showed up because korea culture is subject to a Chinese character culture area. the word of Nabby is originated from flying features of nabby. It was expressed to an Hoju(胡蝶:swallowtail) or Hwangjub(黃蝶:yellow butterfly) in old book, Nabby or Naboi in Dusiunhae(杜詩諺解) issued in 1481, Naboi in Hunmonjahoe(訓夢字會) issued in 1527 and Nami in Simongunhaemungmyung(時夢諺解物名) issued in Sookjong dynasty(1675$\sim$1720). After that it was called Nabeui or Nabby and Nabby became the standard language but it is still called Nabbo or Nabbe in some provinces. The butterfly have been called as jewelry spread out through the world and people have been attracted by its meaning (love, pleasure, luck, long life and eternity) and its beautiful figure so that they have collected poems and paintings about it to appreciate its beauty or have made craft works and personal ornaments of it. This research is to analyze the shape and color of the using the application method of the nubi, which is used as expression method in this research, is suitable to express the beauty of butterfly's shape and the nerve of its wings and the basic material, which is light and have good drape, was used to easily express the rhythmical movement of butterfly's flapping. And thus, this research is to present that the above expression method is suitable to express the beautiful expression of butterfly's image and have unlimited potential energy for developing designs. Results showed that the soft outline of butterfly's wings can coincide with the linear shape of human body. It was also found that the characteristics of nubi method could be diversified as material expression method and the Haute Couture luxurious work could be developed by applying the mixed nubi method to costumes.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.11 no.2
• /
• pp.179-186
• /
• 2008

Purpose: Feeding problems are common for typically developing children and have negative effects on physical, behavioral, and cognitive development. The purpose of the current study was to examine factors related to childhood feeding problems for typically developing children via parental reports. Methods: The feeding questionnaire developed by the authors and the Childhood Eating Behavior Inventory (CEBI) were administered to 796 parents of children who recruited from 2 pediatric outpatient clinics (n=379) and community (n=417). Problem eaters (PE) were identified by parental report and frequency analysis was conducted for types of feeding problems and its severity, problem behaviors during mealtime, the feeding methods of the parents, and the need for treatment. Results: The CEBI scores were significantly different between the PE and non-problem eaters (NPE), which suggests that the parental reports were reliable. The younger children had more feeding problems than the older children. The most frequent and severe feeding problems were selective eating and longer mealtimes across all age groups. One-half of the children had more than one problem behaviors during mealtime. Most parents of PE used ineffective methods to deal with children's behaviors during meal time, such as cajoling, which was related to their increased level of stress. Many parents reported their children need professional help for the feeding problems. Conclusion: Feeding problems are prevalent among children, especially younger children. Children with feeding problems showed a number of problem behaviors during mealtime and parental coping methods appeared to be ineffective. The need for treatment was considerable, thus the characteristics of this population must be acknowledged for providing proper treatment and advice.

• Journal of Food Hygiene and Safety
• /
• v.26 no.4
• /
• pp.308-314
• /
• 2011

This study surveyed and compared the temperatures established in display stands and food surfaces for cold and frozen foods in large discount stores in Korea. The temperatures established in display stands for cold food ranged with $3.5{\pm}1.8^{\circ}C$ as mean, minimum and maximum were $0^{\circ}C$ and $7^{\circ}C$. However, the surface temperatures of cold food on sale ranged with $10.7{\pm}2.9^{\circ}C$ as a mean, minimum $4.6^{\circ}C$ and maximum $18.4^{\circ}C$. Totally, the surface temperature of cold food on sale was $7.2^{\circ}C$, as a mean, higher than established in display stands for cold food in large discount stores in Korea. 53% of the surveyed cold foods were more than $10^{\circ}C$ in surface temperature and only 47% was less than $10^{\circ}C$. The differences between temperatures were lowest in fruits, salads and vegetables, but highest in milk products. On the other hand, the temperatures established in display stands for frozen food showed a range with $-20.7{\pm}1^{\circ}C$ as a mean. However, the surface temperatures of frozen food on sale showed a range with $-15.4{\pm}5^{\circ}C$ as a mean, minimum $-28^{\circ}C$ and maximum $-4.6^{\circ}C$ (included defrosting). The surface temperatures of frozen food, frozen meats, frozen processed foods and ice creams were $-13.8^{\circ}C$, $-15.9^{\circ}C$, and $-16.8^{\circ}C$, respectively. Only 32.3% of surveyed frozen foods showed less than $-18^{\circ}C$ in surface temperature. In conclusion, the temperatures established on cold and frozen food display stands were less than those of cold and frozen food surfaces on sale. There was also much variation in food surface temperatures during cold and frozen food storage and sales. Therefore, a temperature management system technology use at the distribution level for cold and frozen foods will be developed.

• Journal of the Korean Institute of Landscape Architecture
• /
• v.42 no.5
• /
• pp.64-72
• /
• 2014

This study quantified the storage and annual uptake of carbon by apple trees in orchards as a production-type greenspace, and computed the annual carbon emissions from apple cultivation. Tree individuals in the study orchards were sampled to include the range of stem diameter sizes. The study measured biomass for each part including the roots of sample trees through a direct harvesting method to compute total carbon storage per tree. Annual carbon uptake per tree was quantified by analyzing the radial growth rates of stem samples at ground level. Annual carbon emissions from management practices such as pruning, mowing, irrigation, fertilization, and use of pesticides and fungicides were estimated based on maintenance data, interviews with managers, and actual measurements. Regression models were developed using stem diameter at ground level (D) as an independent variable to easily estimate storage and annual uptake of the carbon. Storage and annual uptake of carbon per tree increased as D sizes got larger. Apple trees with D sizes of 10 and 15 cm stored 9.1 and 21.0 kg of carbon and annually sequestered 1.0 and 1.6 kg, respectively. Storage and annual uptake of carbon per unit area in study orchards were 3.81 t/ha and 0.42 t/ha/yr, respectively, and annual carbon emissions were 1.30 t/ha/yr. Thus, the carbon emissions were about 3 times greater than the annual carbon uptake. The study identified management practices to reduce the carbon footprint of production-type greenspace, including efficient uses of water, pesticides, fungicides, and fertilizers. It breaks new ground by including measured biomass of roots and a detailed inventory of carbon emissions.