• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.178-181
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• 2014

Hunter syndrome (mucopolysaccharidosis type II, MPS II) is a X-linked lysosomal storage disease caused by a deficiency in the lysosomal enzyme, iduronate-2-sulfatase (IDS), leading to accumulation of glycosaminoglycans within lysosomes of many organs and tissues. Since the enzyme replacement therapy was approved and available in the treatment of MPS I, II, VI, early diagnosis and early therapy can bring the better prognosis of disease and the better quality of life in patients. We described a 2.5 year old child presented with frequent otitis media and developmental delay including speech impairment, who was diagnosed as Hunter syndrome with IDS NM_000202.5:c. 263G>A(p.Arg88His) mutation.

• Journal of mucopolysaccharidosis and rare diseases
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• v.5 no.1
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• pp.17-21
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• 2021

Hunter syndrome or mucopolysaccharidosis type II (MPS-II) (OMIM 309900) is a rare lysosomal storage disorder caused by deficiency in the activity of the enzyme iduronate-2-sulfatase. This enzyme is responsible for the catabolism of the following two different glycosaminoglycans (GAGs): dermatan sulfate and heparan sulfate. The lysosomal accumulation of these GAG molecules results in cell, tissue, and organ dysfunction. Patients can be broadly classified as having one of the following two forms of MPS II: a severe form and an attenuated form. In the severe form of the disease, signs and symptoms (including neurological impairment) develop in early childhood, whereas in the attenuated form, signs and symptoms develop in adolescence or early adulthood, and patients do not experience significant cognitive impairment. The involvement of the skeletal-muscle system is because of essential accumulated GAGs in joints and connective tissue. MPS II has many clinical features and includes two recognized clinical entities (mild and severe) that represent two ends of a wide spectrum of clinical severities. However, enzyme replacement therapy is likely to have only a limited impact on bone and joint disease based on the results of MPS II studies. The aim of this study was to review the involvement of joints in MPS II.

• Journal of The Korean Society of Grassland and Forage Science
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• v.29 no.2
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• pp.95-102
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• 2009

To determine $SO_4^{2-}$ uptake and its assimilation in response to the exogenous $SO_4^{2-}$supply level in forage rape (Brassica napus L.), the concentration of this element in plant tissues and the activity of ATP sulfurylasc and APS reductase was measured after 25 hours of treatment (1.0 mM $SO_4^{2-}$, control; 0.1 mM $SO_4^{2-}$, S deficiency; 0 mM $SO_4^{2-}$, S deprivation). $SO_4^{2-}$ uptake and the concentration in the plant tissues significantly decreased in S-deficient and S-deprived condition, while it maintained at nearly same level in the control. The activity of ATP sulfurylase tended to increase with decreasing the exogenous $SO_4^{2-}$ supply, while that of APS reductase to decrease. A significant change in both enzymes responding to S-deprivation treatment was observed only young and middle leaves. The results indicated that $SO_4^{2-}$ assimilation in young leaf tissues would be much more sensitively responded to S-limited nutrition.

• Journal of Korean Society of Environmental Engineers
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• v.32 no.9
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• pp.851-856
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• 2010

A sulfur utilizing nitrite denitrification process could be placed after the shortcut biological nitrogen removal (SBNR) process. In this study, removal of nitrite using sulfur oxidizing denitrifier was characterized in batch tests with granular elemental sulfur as an electron donor and nitrite as an electro acceptor. At sufficient alkalinity, initial nitrite nitrogen concentration of 100 mg/L was almost completely reduced in the batch reactor within a incubation time of 22 h. Sulfate production with nitrite was 4.8 g ${SO_4}^{2-}/g$ ${NO_2}^-$-N, while with nitrate 13.5 g ${SO_4}^{2-}/g$ ${NO_3}^-$-N. Under the conditions of low alkalinity, nitrite removal was over 95% but 15 h of a lag phase was shown. For nitrate with low alkalinity, no denitrification occurred. Sulfate production was 2.6 g ${SO_4}^{2-}/g$ ${NO_2}^-$-N and alkalinity consumption was 1.2 g $CaCO_3/g$ ${NO_2}^-$. The concentration range of organics used in this experiment did not inhibit autotrophic denitrification at both low and high alkalinity. This kind of method may solve the problems of autotrophic nitrate denitrification, i.e. high sulfate production and alkalinity deficiency, to some extent.

• Asian-Australasian Journal of Animal Sciences
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• v.17 no.10
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• pp.1378-1382
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• 2004

Zinc (Zn) is a micromineral and functions as a cofactor of many enzymes and its deficiency induces retardation of growth and dysfunction of the immune system in animals. This study was conducted to determine lipogenic activity of Zn in bovine intramuscular adipocytes. Preadipocytes were isolated from intramuscular fat depots of 26 month old Korean (Hanwoo) steers and cultured in media containing Zn. At confluence, the cells were treated with insulin, dexamethasone, and 1-methyl-3-isobutyl-xanthine to induce differentiation (accumulation of lipid droplets in cells). The sources of Zn were zinc chloride (${ZnCl}_2$) and zinc sulfate (${ZnSO}_4$), and the final concentrations of both Zn sources were 0, 5, 25, 50 and 100 ${\mu}$M. Glycerol-3-phosphate dehydrogenase (GPDH) activity, an index of adipocyte differentiation, was increased as the concentration of Zn in media increased showing the highest activity (25.74 ng/min/mg protein) at 25 ${\mu}$M of ${ZnSO}_4$. Supplementation of Zn during differentiation of bovine intramuscular adipocytes tended to decrease the production of nitric oxide (NO). Peroxisome proliferator-activated receptor gamma 2(PPAR$\gamma$2) gene expression was increased 10 days after differentiation induction. The current results indicate that Zn has a strong lipogenic activity in cultured bovine intramuscular adipocytes with remarkable suppression of NO production.

• Journal of Nutrition and Health
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• v.16 no.3
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• pp.216-227
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• 1983

As an attempt to improve the nutritional status of Korean rural pre-school children, anthropometry, clinical examination, and hematological parameters(Hb, Ht, and serum albumin) are measured. Six kinds of diet supplemented with 0, 2.5, 5.0, 7.5, 10.0 and 20.0 mg Fe(as ferrous sulfate) were used for 10 weeks. Subjects were 349 pre- school children aged from 1 to 5 years old living in An-gam-my-on and Bong-dang-myon, Hwa-seung-goo, Kyong-gi-duo, and the supplementary diet was a product of the Model Nutritional Enterprise Factory in Wuen-sueng-goon, Gang-won- duo. The results obtained were as follows : 1) Physical development of subject children were within normal range during the feeding trial period of 9 weeks. 2) From the results of clinical examination, no children showed nutritional deficiency signs. 3) Iron free supplementary diet group( control group ) among other subject children also showed some improvement of hemoglobin level ana frequency of anemia. 4) The amount of iron fortified as supplemental diet was effective in 5.0-7.5 mg Fe per child per day, no further effect was observed by adding of more iron. 5) The values of hematocrit and serum albumin also improved by providing the supplementary diet.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.37-40
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• 2016

Mucopolysaccharidosis type III (MPS III) is a rare genetic disorder caused by lysosomal storage of heparan sulfate. MPS IIIB results from a deficiency in the enzyme alpha-N-acetyl-D-glucosaminidase (NAGLU). Affected patients begin showing behavioral changes, progressive profound mental retardation, and severe disability from the age of 2 to 6 years. We report a patient with MPS IIIB with a long-term follow-up duration. He showed normal development until 3 years. Subsequently, he presented behavioral changes, sleep disturbance, and progressive motor dysfunction. He had been hospitalized owing to recurrent pneumonia and epilepsy with severe cognitive dysfunction. The patient had compound heterozygous c.1444C>T (p.R482W) and c.1675G>T (p.D559Y) variants of NAGLU. Considering that individuals with MPS IIIB have less prominent facial features and skeletal changes, evaluation of long-term clinical course is important for diagnosis. Although no effective therapies for MPS IIIB have been developed yet, early and accurate diagnosis can provide important information for family planning in families at risk of the disorder.

• Korean Journal of Environmental Agriculture
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• v.26 no.4
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• pp.294-299
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• 2007

Natural gypsum has been used as a soil amendment in the United States. However, flue gas desulfurization (FGD)-gypsum has not traditionally been used for agricultural purpose although it has potential benefit as a soil amendment. To expand use of FGD-gypsum for agricultural purpose, the effect of FGD-gypsum on soil chemical properties was investigated in the field scales. Application rates for this study were 0 (control), 1.1, and 2.2 Mg ha-1 of FGD-gypsum. After two year application, the soil samples were taken to 110 cm depth and sub-sampled at 10 cm intervals. The heavy metal contents in FGD-gypsum were lower than ceiling levels allowed by regulations for land-applied biosolids. Soil pH was not largely affected by FGD-gypsum application. Although degree of calcium (Ca) saturation in surface horizons increases only slightly with respect to the control, there is a clear decrease in exchangeable aluminum (Al). FGD-gypsum clearly increases the soil electrical conductivity (EC) with increasing application rate. Water-soluble Ca and sulfate is increased with FGD-gypsum application and these ions moved to a depth of at least 80 cm after only 2 years. We conclude that surface application of FGD-gypsum can mitigate toxicity of Al and deficiency of Ca in subsoil of acid soil.

• Korean Journal of Community Nutrition
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• v.6 no.5
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• pp.726-733
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• 2001

This study was designed to investigate the effect of iron supplementation on the iron nutritional status and anemia of high school girls in Korea. One hundred thirty-five female students residing in Ulian metropolitan city in Korea diagnosed as having anemia or iron deficiency participated in this study. One or two tablets of iron medicine(80-160 mg Fe as ferrous sulfate/day) were administered to all participants for 3 months. Subjects were evaluated with a questionaire, measurement of hematological indices before and after iron supplementation. The average height and weight of respondents were 161.62 $\pm$ 4.68 cm and 53.87 $\pm$ 6.10 kg, respectively. Daily intakes of energy were 1597.8 $\pm$ 302.35 kcal(76.0% RDA). Iron intakes were 13.72 $\pm$ 4.17 mg (76.3% of RDA) and calcium intakes were 580.74 $\pm$ 177.21(72.5% of RDA) before iron supp]ementation. At baseline, 63% of all participants had depleted store(serum ferritin 12 ug/ml and/or transferrin saturation(TS) < 14%). After iron supplementation, this proportion declined to 19.3%. 55.6% of subjects had 12 ug/m1 of basal ferritin concentration before iron supplementation, and this proportion declined to 16.3% after iron supplementation. The basal hemoglobin(Hb) concentrations were 12.13 $\pm$ 1.01 g/dl and they increased to 12.79 $\pm$ 0.81 g/dl, which showed significant difference artier iron supplementation(p < 0.001). The basal ferritin and TS(%) were 13.24 $\pm$ 11.66 ng/ml, 18.42 $\pm$ 10.12% and they significantly increased to 32.95 $\pm$ 21.14 ng/ml, 33.53 $\pm$ 16.64%, respectively(p < 0.001). The basal total iron binding protein(TIBC) were 467.81 $\pm$ 97.24 ug/dl and they significantly decreased to 325.05 $\pm$ 48.89 ug/dl(p < 0.001) after iron supplementation. The number of tablets administered was positively correlated with serum iron(t = 0.553, p < 0.01), serum ferritin(t = 0.557, p < 0.01), TS(%)(t = 0.588, p < 0.01) and negatively correlated with TIBC(t= -0.409, p <0.01). The anemia symptoms such as ‘Shortening of breath when going upstairs(p < 0.01)’, ‘Tired out easily(p < 0.01)’, ‘Feeling blue(p < 0.001)’, ‘Decreased ability to concentrate(p < 0.01)’, and ‘Poor memory(p < 0.001)’improved significantly after iron supplementation. In this study, daily iron supplementations were efficacious in improving the iron status and anemic symptoms of female high school students. Regular check-ups and nutrition education for adolescents are necessary because of their vulnerability to iron deficiency. Further studies are needed to determine the minimum effective dose of iron and to examine the adverse effect of long-term iron supplementation.

• Journal of The Korean Society of Grassland and Forage Science
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• v.29 no.2
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• pp.103-110
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• 2009

Rape plants, especially forage type, are known as one of high S-demanding plants. Their productivity and quality have often limited under S-deficient condition. To investigate the effect of S-deficiency on N uptake and its assimilation, $NO_3^-$ absorption, nitrate reducatse (NR) and glutamine synthetase (GS) activity in leaf and root tissues as affected by different S-supplied level was determined. $NO_3^-$ uptake was not significant between control and S-deficient treated plants, while significantly depressed in S-deprived plants for the early 8 h. NR activity decreased as S-availability decreased, especially in young and middle leaves, representing more than 35% of decrease in S-deficient and 70% in S-deprived plants when compared with control. In roots, a significant decrease (-29%) in NR was observed only in S-deprived plants. Relatively higher GS activity was found in young leaves for three all treatments. As a whole leaf tissue, S-limited conditions resulted in a reduction of GS activity. In root which showed the lowest activity, a significant decrease (-30%) was observed only in S-deprived plants.