Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.
This study aims to investigate the nutritional status of night eaters using the data from 2005 Korean National Health and Nutrition Examination Survey. A total of 3,903 subjects aged 20 and above were divided into 3 groups by using 24-hr recall data according to the night snack calorie intake: non-night snack, night snack less than 500 kcal and 500 kcal and more. Their data were analyzed to find out the difference on the socio-demographic, anthropometric, blood pressure, blood parameters and dietary characteristics. Among the subjects, non-night eaters were 66.0%, night eaters of less than 500 kcal were 28.4% and 500 kcal and above were 5.6%. Male adults, young-aged, higher educated, higher income earner, breakfast skipper and frequent dine-outer (3 times and more a day) were found more in the night eaters with 500 kcal and above. Night eaters above 500 kcal showed higher waist circumference, Glu-FBS, Glu-PP120 and also showed higher daily intake of fat and alcohol per 1,000 kcal and food groups of meat & eggs, beverages and alcohol drinks (p < 0.05), while they showed the lowest carbohydrate energy ratio of $58.3{\pm}13.7$ among the three groups. These results suggest that habitual night snack intake above 500 kcal could lead to abdominal obesity and diabetes due to higher intake of meat, fat and alcohol.
Koo, Kiwoon;Kweon, Hyeongkeun;Lee, Sang In;Kwon, Semyung;Seo, Jung Il
Journal of Korean Society of Forest Science
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v.110
no.4
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pp.630-647
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2021
We determined the current problem of the restoration deposit-estimation system, stipulated by the Mountainous Districts Management Act, using the Delphi technique. Consequently, we proposed a standard model for forest land restoration to derive a reasonable deposit-estimation system. With the result of the Delphi survey, the inappropriateness of land-use type and slope gradient classifications was shown; the insufficiency of standard works was a significant problem in the current system. A way to solve these problems was devised, to reorganize the current land-use type into the subject of the site. The specific subjects included the following: (i) to permit or report forest land-use change and temporary use of forest land, (ii) to report temporary use of forest land, (iii) to permit stone collection or sale for mineral mining, and (iv) to allow sediment collection. The current slope gradient subdivision into (a) θ<10°, (b) 10°≦θ<15°, (c) 15°≦θ<20°, (d) 20°≦θ<25°, (e) 25°≦θ<30°, and (f) θ≧30° and the reorganization of 17 standard works into 22 standard works were deemed as solutions, along with seven additional works. We developed 24 standard models for the forest land restoration project based on the aforementioned results. The deposits estimated by these models ranged from 34,185,000 (Korean) won to 607,403,000 won. If additional works, premiums, discounts, and supervision fees are added to the models, the deposit increases to an estimated 668,143,000 won subject to permission for stone collection or sale and mineral mining. Experts agree on the distribution of the restoration deposits estimated by these models at a high level in the Delphi survey. Our findings are expected to contribute to securing the appropriateness of the restoration cost deposited for the smooth performance of the vicariously executed restoration project.
We analyzed the distribution area of debris slopes in Mudeungsan (Mt. Mudeung) National Park by comparing aerial photos of the past (1966) and the present (2017) and identified the vegetation characteristics that affect the change in the area of the debris slopes by investigating the vegetation status of the debris slopes and the surrounding areas. The area of debris slopes in Mt. Mudeung appears to have been reduced to a quarter of what it used to be. Debris slopes here have decreased at an average rate of 2.3 ha/yr over 51 years by vegetation covers. Notably, most of the small-area debris slopes in the low-inclination slopes disappeared due to active vegetation coverage. However, there are still west-facing, south-west-facing, south-facing, and large-area debris slopes remaining because the sun's radiant heat rapidly raises the surface temperature of rock blocks and dries moisture, making tree growth unfavorable. Because of these locational characteristics, the small-scale vegetation in the middle of Deoksan Stony Slope, which is the broadest area, showed distinct characteristics from the adjacent forest areas. Sunny places and tree species with excellent drying resistance were observed frequently in Deoksan Stony Slope. However, tree species with high hygropreference that grow well in valleys with good soil conditions also prevailed. In some of these places, the soil layer has been well developed due to the accumulation of fine materials and organic matter between the crevices of the rock blocks, which is likely to have provided favorable conditions for such tree species to settle and grow. At the top of Mt. Mudeung, on the other hand, the forest covered the debris slopes, where Mongolian oaks (Quercus mongolica) and royal azaleas (Rhododendron schlippenbachii), which typically grow in the highlands, prevailed. This area was considered favorable for the development of vegetation for the highlands because the density of rock blocks was lower than in Deoksan Stony Slope, and the soil was exposed. Moreover, ash trees (Fraxinus rhynchophylla) and Korean maple trees (Acer pseudosieboldianum) that commonly appear in the valley areas were dominant here. It is probably due to the increased moisture content in the soil, which resulted from creating a depressive landform with a concave shape that is easy to collect rainwater as rock blocks in some areas fell and piled up in the lower region. In conclusion, the area, density of the rock blocks, and distribution pattern of rock block slopes would have affected the vegetation development and species composition in the debris slope landform.
Seo, Mi-Kyoung;Rhee, MinKyu;Kim, Seung-Hyun;Cho, Sung-Nam;Ko, Young-hun;Lee, Hyuk;Lee, Moon-Soo
Korean Journal of Health Psychology
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v.14
no.3
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pp.579-596
/
2009
This study aimed to develop the Korean tool of competency to consent to psychiatric treatment and to analyze the reliability and validity of this tool. Also the developed tool's efficiency in determining whether a patient possesses treatment consent competence was checked using the Receiver Operating Characteristic curve and the relevant indices. A total of 193 patients with mental illness, who were hospitalized in a mental hospital or were in community mental health center, participated in this study. We administered a questionnaire consisting of 14 questions concerning understanding, appreciation, reasoning ability, and expression of a choice to the subjects. To investigate the validity of the tool, we conducted the K-MMSE, insight test, estimated IQ, and BPRS. The tool's reliability and usefulness were examined via Cronbach's alpha, ICC, and ROC analysis, and criterion related validation was performed. This tool showed that internal consistency and agreement between raters was relatively high(ICC .80~.98, Cronbach's alpha .56~.83)and the confirmatory factor analysis for constructive validation showed that the tool was valid. Also, estimated IQ, and MMSE were significantly correlated to understanding, appreciation, expression of a choice, and reasoning ability. However, the BPRS did not show significant correlation with any subcompetences. In ROC analysis, full scale cutoff score 18.5 was suggested. Subscale cutoff scores were understanding 4.5, appreciation 8.5, reasoning ability 3.5, and expression of a choice 0.5. These results suggest that this assessment tool is reliable, valid and efficient diagnostically. Finally, limitations and implications of this study were discussed.
This study was carried out to determine impacts of burning of barley straw produced from rice-barley double cropping paddy field on air quality by investigating emissions of greenhouse gases ($CO_2$, $CH_4$ and $N_2O$), air pollution gases (CO, $SO_2$, $H_2S$, $NH_3$ and NO) and particulate matters (PM 10 and PM 2.5). When the barley straw at a rate of 4.5 t/ha was burned at open status, the emitted GHGs amounts were $CO_2$ 376.8 kg/l0a, $CH_4$ 1.56 and $N_2O$ 0.06. The amount of CO emission was the largest among air pollution gases. These results showed that the range of $45{\sim}55%$ of total C in barley straw was emitted as $CO_2-C$, followed by CO-C ($6.4{\sim}5.9%$) and $CH_4-C$ ($0.5{\sim}0.7%$). As far as moisture content in barley straw is concerned, the higher moisture content that the barley straw contains, the larger amount of air pollution gases and the higher portion of PM 2.5 in PM 10 were emitted when it burned. In case of harvesting time of barley straw, emission amounts of greenhouse, air pollution gases and PM 2.5 portion in PM 10 had tendency to increase when earlier harvested barley straw was burned.
The dual inoculation response of soybean with rhizobium and mycorrhiza was examined in pot vermiculite and field soils. In order to select a symbiotically compatible mycorrhiza with Bradyrhizobium japonicum, a highly germinating spore among 60 strains from 32 upland soils in southern part of Korea was obtained in Acaulospora sp., Gigaspora sp. and Glomus sp., respectively. As a result of dual inoculation of Glycin max cv. Dajangkong and Eunhakong both with $1{\times}10^8$cells of B. japonicum YCK 213 and 10 spores of each mycorrhiza in vermiculite pot, only Glomus sp. treatment together with the rhizobium showed significant increase ($P{\leqq}0.05$) both in shoot dry wt and nodule mass of not Eunhakong but Dajangkong. In red-yellow soils with pH 5.2($1:5H_2O$) and 203 mg of Lancaster P per kg of soil, in which $10^3$ cells of B. japonicum and $10{\pm}0.2$ spores of mycorrhizae per gram of soil were naturalized, grain yield of G. max cv. Dajangkong was increased to 3.9% by dual inoculation both of $4.8{\times}10^6$cells of B. japonicum and 10 spores of mycorrhizae per two seeds under condition applied with 30 kg $P_2O_5$ and 34 kg $K_2O$ per hectare compared to conventionally fertilized plot (2.75 MT $ha^{-1}$) added with 30 kg N $ha^{-1}$. However, there was not significant.
Volcanic ash soils were classified as Andepts, a suborder of the Inceptisols in Soil Taxonomy. Increased knowledge on the volcanic ash soils necessitated and facilitated considerable improvement in this system. As a result, the new Order of Andisols was incorporated in the 1990 edition of the Keys to Soil Taxonomy. The central concept of an Andisol is that of a soil developing in volcanic ejecta, and/or in volcaniclastic materials, whose colloidal fractions are dominated by short-range-order minerals or Al-humus complexes. Andic propertis of volcanic ash soil in Cheju Island were investigated. For this study, soils of toposequence distributed along the southern slope of Mt. Halla, and the major soil groups such as dark brown soils, very dark brown soils, black soils, and brown forest soils were collected and analyzed for Al, Fe and Si extracted with solutions of pyrophosphate, dithionite-citrate. and oxalate respectively. Weolpyeong and Yongheung soils developed on the lower elevations contain only small amounts of allophane and Al-and Fe-humus complexes. For other soils, allophane content decrease with elevation and increaes with soil depth,whereas Al-humus complexes increase with elevation and decrease with soil depth. Substantial amount of allophane and ferrihydrite are found in all horizons of Ora and Ara soils developed on 150-300m elevations and of Noro and Jeogag soils developed on cinder cones. In the A horizons of Pyeongdae, Tosan and Heugag soils developed on the higher elevations, Al-humus complexes are dominant form of Al reflecting low pH and high organic matter content. However, lower horizons are dominated by allophane.
Woo, Koan Sik;Ko, Jee Yeon;Kim, Jung In;Lee, Jae Saeng;Song, Seuk Bo;Cho, Jae Min;Jung, Tae Wook;Kim, Ki Young;Oh, In Seok
KOREAN JOURNAL OF CROP SCIENCE
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v.58
no.4
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pp.399-407
/
2013
The purpose of this study was to evaluate the physicochemical characteristic of the cooked rice added with glutinous and non-glutinous sorghum. The sorghum cultivars were Sorghum bicolor L. Moench cv. Hwanggeumchal, Nampungchal (glutinous), and Donganme (nonglutinous), and rice cultivar was Ilpum rice. The cooking properties and pasting characteristics of cooking rice adding with sorghum according to varieties and different addition rates evaluated. The cooking properties and pasting characteristics had significant changes with the varieties and different addition rates of sorghum. With increased addition rates of sorghum, the pasting temperature, peak viscosity, trough viscosity, breakdown viscosity, and final viscosity were decreased. With increased addition rates of sorghum, the total polyphenol and flavonoid contents before and after cooked rice were increased. Total polyphenol contents of 30% addition rates before cooking rice with Hwanggeumchal, Nampungchal, and Donganme sorghum were 1,693.30, 1,890.98 and $2,386.11{\mu}g/g$ sample, whereas those after cooking rice with sorghum were 1,189.28, 1,190.42 and $1,397.87{\mu}g/g$ sample, respectively. The high level of DPPH radical scavenging activity before and after cooking rice with sorghum were 126.29 and 70.58 mg TE/100g sample in the Donganme in 30% addition rates. Also, ABTS radical scavenging activity was 135.56 and 83.12 mg TE/100g sample, respectively. The results of this study show that the addition of sorghum can make cooked rice improved antioxidant activity.
Sorghum has been consumed as one of the important staple food in the semiarid tropics of Africa and Asia. Sorghum is rich in starch, protein, essential vitamins and minerals and grows relatively well in dry climate regions when it compared with other staple food crops. Sorghum has taken an increased interest due to several studies that report about the beneficial effects of sorghum on human health. In the present study, we investigated the antioxidative and activity of extract of milling by-products (hull and bran) of Korean sorghum cultivar, 'Hwanggeaumchal' as well as its grain. Hull extract showed the highest total polyphenol contents ($29.7{\pm}0.2mg\;GAE/100g$) and major four pigments content ($322.6{\pm}14.5mg/100g$). From results of 2,2'-azinobis-(3-ethylbenzothiazoline-6-sulfonate (ABTS) radical scavenging activity, hull extract ($IC_{50}$, $6.3{\pm}0.1{\mu}g/mL$) was also showed the strongest antioxidative effects. Bran and grain showed similar polyphenol, pigments contents and antioxidative effects. We determined cell viability by MTT assay and evaluated the anti-inflammatory activity by measuring nitric oxide (NO) of hull, bran and grain methanol extract (0.5% HCl v/v) on RAW 264.7 cells. Hull extract treatment was significantly decreased NO production with dose-dependant manner. Apigeninidin as one of the major pigment of hull was showed inhibitory activity against NO production without cytotoxicitiy. Therefore, sorghum milling by-products can be used as a good source of antioxidative and anti-inflammatory agents.
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