• Journal of mucopolysaccharidosis and rare diseases
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• 제5권1호
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• pp.12-16
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• 2021

Mucopolysaccharidosis type II (MPS II, Hunter syndrome) is an X-linked lysosomal storage disorder caused by deficiency of the enzyme iduronate-2-sulfatase, leading to the accumulation of glycosaminoglycans (GAGs), which affects multiple organs and systems. Current treatments for MPS II include enzyme replacement therapy (ERT) and hematopoietic cell transplantation (HCT) to reduce the accumulation of GAGs. HCT has the potential advantage that donor-derived enzyme-competent cells can provide a continuous secreting source of the enzyme. However, HCT as a treatment for MPS II remains controversial because its effectiveness is unclear, particularly in terms of neurological symptoms. To date, several clinical experiences with HCT in MPS II have been reported. In this paper, we review post-HCT outcomes in the previously published literature and discuss the effects of HCT on each of the clinical signs and symptoms of MPS II.

• 전력전자학회:학술대회논문집
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• 전력전자학회 2006년도 전력전자학술대회 논문집
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• pp.421-423
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• 2006

In this paper, we propose EDLC's high effective operating techniques. EDLC is energy storage device that has advantages of electrolytic capacitor and accumulation capacitor and that supplements defect such as energy storing accumulation capacity, life time and safety. But, EDLC needs for more complex control scheme because is influenced directly in efficiency and life time by charge and discharge control method. In this paper, DVR system's performance is proposed by EDLC's high effective operating technique.

• Asian-Australasian Journal of Animal Sciences
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• 제19권1호
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• pp.113-118
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• 2006

The effects of dietary Rhus verniciflua Stokes supplementation (0%, 2%, 4% and 6%/feed) on meat color, water-holding capacity (WHC), lipid oxidation and fatty acid composition in m. longissimus from Hanwoo (Korean cattle) beef during refrigerated storage were investigated. The $L^*$, $a^*$, $b^*$ and $C^*$ values of 4% group were significantly (p<0.05) higher than those of the other groups for 7 days of storage. The $a^*$, $b^*$ and $C^*$ values declined gradually during storage in all of the groups and the decline was more rapid in control group. The metmyoglobin (%) was significantly (p<0.05) increased during storage time in all of the groups, but the 4% group had a lower rate of metmyoglobin accumulation during storage. WHC was significantly (p<0.05) higher in the 2% and 4% groups than in the other groups. The TBARS (thiobarbituric acid reactive substances) value of day 0 was not significantly (p>0.05) different among 4 diet conditions, but the TBARS value after 5 days of storage was significantly (p<0.05) higher in the control group than in other groups. The proportions of C18:1, MUFA, UFA and MUFA/SFA ratio were significantly (p<0.05) higher in the meat from Rhus verniciflua Stokes-supplemented Hanwoo than in the control group. Consequently, the meat from Rhus verniciflua Stokes-supplemented Hanwoo showed higher color stability, WHC, unsaturated fatty acids and retarded lipid oxidation compared to the control meat. In particular, dietary Rhus verniciflua Stokes supplementation with 4% extended storage life compared to the other groups.

• 아시안잔디학회지
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• 제10권4호
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• pp.275-283
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• 1996

An investigation was performed to reveal the relation between the storage and decomposition of the titter DNA of a Zoysia japonica grassland on Mt. Kwanak. The loss constant k of litters was 0.167. The times required for the decomposition of half, 95% and 99% of accumulated DNA on the grassland floor were 3.8, 16.6 and 27.6, respectively. The amount of DNA which is turned to living organism in the ecosystem is higher than that of crude protein. In the case of crude protein, the decay constant k was 0.181. The times needed for the decomposition of half, 95% and 99% of accumulated crude protein on the Z. japonica grassland floor were 3.8, 16.6 and 27.6 years, respectively. Key words: Zoysia japonica, Mt. Kwanak, Litter DNA, Crude protein, Decomposition, Accumulation.

• 한국농공학회:학술대회논문집
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• 한국농공학회 2002년도 학술발표회 발표논문집
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• pp.261-264
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• 2002

Yi-dong experimental basin is operated for research on the rural basin characteristics and accumulation of a long term data by hydrological observation equipments. It is basin area 9,440ha, length 14.4km and slope 0.67%. Hydrological observation network is constructed of rainfall meter 4points, reservoir storage level 3points and river water level 2points.

• 한국조경학회지
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• 제27권1호
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• pp.39-53
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• 1999

This study quantified carbon uptake and emissions in urban landscape, and the role of urban greenspace in atmospheric carbon reduction for several cities of Chuncheon and Kangleung in Kangwon province. Mean carbon storage by trees and shrubs was 26.0 t (mertric tons)/ha in Chuncheon and 46.7 t/ha in Kangleung for natural lands, and ranged from 4.7 to 6.3 t/ha for urban lands (all land use types except natural and agricultural lands) in both cities. Mean annual carbon uptake by trees and shrubs ranged from 1.60 to 1.71 t/ha/yr for natural lands, and from 0.56 to 0.71 t/ha/yr for urban lands. There was no significant difference (95% confidence level) between the two cities in the carbon storage and annual carbon uptake per ha, except the carbon storage for natural lands. Organic carbon storage in soils (to a depth of 60 cm) of Chuncheon average 24.8 t/ha for urban lands and 31.6 t/ha for natural lands, 1.3 times greater than for urban lands. Annual carbon accumulation in soils was 1.3 t/hr/yr for natural lands of the study cities. Annual per capita carbon emissions from fossil fuel consumption were 1.3 t/yr in Chunceon and 1.8 t/yr in Kangleung. The principal carbon release in urban landscapes was from transport and industry. Total carbon storage by urban greenspace (trees, shrubs, and soils) equaled 66% of total carbon emissions in Chuncheon and 101% in Kangleung. Carbon uptake by urban greenspace annually offset total carbon emissions by approximately 4% in the study cities. Thus, urban greenspace played a partial important role in reducing atmospheric $CO_2$ concentrations. To increase $CO_2$ uptake and storage by urban greenspace, suggested are conservation of natural lands, minimization of hard surfaces and more plantings, selection of tree species with high growth rate, and proper management for longer healthy tree growth.

• 대한약학회:학술대회논문집
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• 대한약학회 2002년도 Proceedings of the Convention of the Pharmaceutical Society of Korea Vol.2
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• pp.431.2-432
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• 2002

Mucopolysaccharidosis (MPS) is a genetic disorder with deficiency of Iysomal enzymes needed for the degradation of glycosaminoglycans(GAGs). This storage disease is characterized by intra-lysosomal accumulation of GAGs. progressive mental and physical deterioration. multi-organ failure and premature death. Quality of life (QOL) is very low in MPS patients. The MOS 36-ltem Short Form Health Survey (SF-36) was designed to measure the eight (8) dimensions of health in clinical and general population settings. (omitted)

• 한국농공학회:학술대회논문집
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• 한국농공학회 2003년도 학술발표논문집
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• pp.427-430
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• 2003

Yi-dong experimental site is operated for research on the rural basin characteristics and accumulation of a long term data by hydrological observation equipments. This basin area is 9,300ha, length 14.4km and slope 0.67%. Hydrological observation network has 3 rainfall meter3, 3 reservoir storage levels and 2 river water levels.

• Applied Microscopy
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• 제18권2호
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• pp.102-118
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• 1988

This study was carried out to examine in vitro first whether the storage proteins, which the fat bodies of last larvae from Hyphantria cunea secrete into haemolymph, can be uptaked by the fat body cells of prepupa and then how the uptaked storage proteins can be accumulated in the fat body cells, if uptaken. The fat bodies which had been isolated from last instar larvae were cultured in 1 ml of Grace's insect medium containing $50{\mu}l$ of $^{3}H$-leucine (5.0 mCi/mol, Dupont) at $28{\pm}2^{\circ}C$ for 6 hrs. After the homogenates of the cultured fat bodies were centrifuged at 10,000 rpm for 10 minutes, the proteins included in the supernatant were separated by polyacrylamide gel electrophoreses (non-SDS, 6%). The next treatment of the electrophoresed gel was followed by rinsing. A storage protein band of several bands in the rinsed gel was sliced off. With elution of sliced storage protein bands in Tris-glycine buffer, the purification of radioactive storage proteins from fat bodies was finished. After the purified radioactive storage proteins were added in Grace's insect midis containing fat bodies of the prepupae, they were cultured for the randomly following minutes given as 3, 5, 7, 10, 15, 20 and 30 and for the randomly following hours given as 1, 2, 3 and 4 respectively. The double fixations of the cultured fat bodies in aldehyde and $OsO_4$, were followed by preparation of ultrathin sections from Epon-Araldite blocks through dehydration and embedding. The electron microscope autoradiographic treatment of all prepared sections were performed by the dipping method (Kim et al., 1987). The finally prepared specimens were examined with electron microscope. The fat body cells of the prepupa could be found to uptake the storage preteins of the last instar larvae, which were included in the culture medium, mostly by formation of coated vesicles. The in vitro uptake of the storage proteins actively occurred by 30 minutes after the addition of purified storage proteins in the culture medium. After culture for 7 minutes with the storage proteins, the uptaked radioactive storage proteins labelled a number of lysosomal granules. After culture for 20 minutes with the storage proteins, the radioactive storage proteins were finally incorporated and accumulated in lipid droplets and protein granules. The frequency in the fat body cell of radiolabelled lipid droplets occurs approximately 60%, while the frequency, in which the radiolabelled protein granules occurs in a fat body cell, is approximately 40%.

• BMB Reports
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• 제48권8호
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• pp.438-444
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• 2015

Lysosomal storage diseases (LSDs) are a group of inherent diseases characterized by massive accumulation of undigested compounds in lysosomes, which is caused by genetic defects resulting in the deficiency of a lysosomal hydrolase. Currently, enzyme replacement therapy has been successfully used for treatment of 7 LSDs with 10 approved therapeutic enzymes whereas new approaches such as pharmacological chaperones and gene therapy still await evaluation in clinical trials. While therapeutic enzymes for Gaucher disease have N-glycans with terminal mannose residues for targeting to macrophages, the others require N-glycans containing mannose-6-phosphates that are recognized by mannose-6-phosphate receptors on the plasma membrane for cellular uptake and targeting to lysosomes. Due to the fact that efficient lysosomal delivery of therapeutic enzymes is essential for the clearance of accumulated compounds, the suitable glycan structure and its high content are key factors for efficient therapeutic efficacy. Therefore, glycan remodeling strategies to improve lysosomal targeting and tissue distribution have been highlighted. This review describes the glycan structures that are important for lysosomal targeting and provides information on recent glyco-engineering technologies for the development of therapeutic enzymes with improved efficacy. [BMB Reports 2015; 48(8): 438-444]