• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.5-13
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• 2012

I-cell disease (mucolipidosis type II; MIM 252500) and pseudo-Hurler polydystrophy (mucolipidosis type III; MIM 252600) are disorders caused by abnormal lysosomal transport in cells. The presence of numerous inclusion bodies in the cytoplasm of fibroblasts, a lack of mucopolysacchariduria, increased lysosomal enzyme activity in serum, and decreased GlcNAc-phosphotransferase activity are hallmark. Here, we attempted to investigate phenotypical and biochemical characteristics of the knockoutmouse of GlcNAc-phosphotransferase ${\alpha}/{\beta}$ subunits; in addition, we also attempted to determine whether the lysosome enriched fraction derived from placenta can be beneficial to phenotype and biochemistry of the knockout mouse.We found that the knockout mouse failed to thrive and had low bone density, as is the case in human. In addition, skin fibroblasts from the animal had the same biochemical characteristics, including increased lysosomal enzyme activity in the culture media, in contrast to the relatively low enzyme activity within the cells. Intravenous injection of the lysosome rich fraction derived from placenta into the tail vein of the animal resulted in a gain of weight, while saline injected animals didn't.In conclusion, our study demonstrated the phenotypical and biochemical similarities of the knockout mouse to a mucolipidosis type II patient and showed the therapeutic potential of the lysosome enriched fraction. We admit that a larger scale animal study will be needed; however, the disease model and the therapeutic potential of the lysosome enriched fraction will highlight the hope for a novel treatment approach to mucopolipidosis type II, for which no therapeutic modality is available.

• Journal of the Korean Applied Science and Technology
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• v.37 no.2
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• pp.153-161
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• 2020

Arthrospira platensis is the oldest marine microalgae on the planet, is said to contain most of the nutrients needed by the human body. It's components are reported to contain a large amount of various substances such as phycocyanin, chlorophyll and β-carotene, and are known to have an aging and whitening effect. In this study, UVB-induced reactive oxygen species reduction efficacy and antioxidant activity of spirulina purified water extract were investigated. effect was confirmed by measuring DPPH radical scavenging activity, FRAP reducing power and ABTS⁺ radical scavenging activity of 0.05, 0.10, 0.50 1.0 mg/mL of spirulina purified water extract. The coagulation rate, hatching rate and heart rate toxicity were measured by treating spirulina purified water extract with 0.05, 0.10, 0.50 mg/mL concentration using Zebrafish, an alternative experimental animal model. UVB-induced ROS measurement was treated with spirulina extract at 0.05, 0.10, 0.50 mg/mL concentration, and then stained with DCFH-DA to confirm the inhibitory effect of ROS. As a result of measuring antioxidant effect, DPPH, FRAP and ABTS⁺ showed concentration-dependent antioxidant effects in comparison with ascorbic acid. and measuring the coagulation rate, hatching rate, and heart rate using Zebrafish, an alternative experimental animal, it was confirmed that there was no toxicity in 0.05 and 0.10 mg/mL except 0.5 mg/mL compared to the control group. The ROS scavenging activity of UVB-induced zebrafish showed higher ROS reduction than the positive control. The results of this study suggest that spirulina and purified water extracts are valuable for UV and skin protection cosmetics.

• The Journal of Korean Society for Radiation Therapy
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• v.26 no.1
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• pp.77-81
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• 2014

Purpose : In the current whole brain Radiation Therapy, Optimold was used to immobilize the head. However, skin dose was increased about 22% due to the scattering radiation by the Optimold. Since the minimum dose causing cataracts was 2 Gy, it could be seen that the effects were large especially on the lens. Therefore, in the whole brain Radiation Therapy, it was to compare and to evaluate the lens absorbed dose according to the presence of Optimold in the eyeball part. Materials and Methods : In order to compare and to evaluate the lens absorbed dose according to the presence of Optimold in the eyeball part, the Optimold mask was made ??up to 5mm bolus on the part of the eye lens in the human model phantom (Anderson Rando Phantom, USA). In the practice treatment, to measure the lens dose, the simulation therapy was processed by placing the GafChromic EBT3 film under bolus, and after the treatment plan was set up through the treatment planning system (Pinnacle, PHILIPS, USA), the treatments were measured repeatedly three times in the same way. After removing the Optimold mask in the eyeball part, it was measured in the same way as above. After scanning the film and measuring the dose by using the Digital Flatbed Scanner (Expression 10000XL, EPSON, USA), the doses were compared and evaluated according to the presence of Optimold mask in the eyeball part. Results : When there was the Optimold mask in the eyeball part, it was measured at $10.2cGy{\pm}1.5$ in the simulation therapy, and at $24.8cGy{\pm}2.7$ in the treatment, and when the Optimold mask was removed in the eye part, it was measured at $12.9cGy{\pm}2.2$ in the simulation therapy, and at $17.6cGy{\pm}1.5$ in the treatment. Conclusion : In case of removing the Optimold mask in the eyeball part, the dose was increased approximately 3 cGy in the simulation therapy and was reduced approximately 7 cGy in the treatment in comparison to the case that the Optimold mask was not removed. During the whole treatment, since the lens absorbed dose was reduced about 27%, the chance to cause cataracts and side effects was considered to be reduced due to decrease of the absorbed dose to the eye lens which had the high sensitivity on the radiation.

• Journal of Life Science
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• v.21 no.9
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• pp.1214-1218
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• 2011

We investigated the fruits of Rubus crataegifolius Bge, a plant which has been traditionally used in Korea in phytotherapy, to describe antioxidant materials from plant sources. R. crataegifolius fruits were extracted with methanol and further fractionated into n-hexane, diethyl ether, and ethyl acetate. The antioxidant activity of each fraction and the residue was assessed using a 1,1-diphenyl-2-picrylhydrazyl (DPPH), $H_2O_2$ radical scavenging method, and their cytotoxicity on human primary kerationcyte (HK) was determined by an MTS assay. The R. crataegifolius fruit methanol extract showed strong antioxidant activity (75.04%, 50%) compared with vitamin C (79.9%, 54.1%) by the DPPH, and $H_2O_2$ method, respectively. The measured activity from the subsequent extracts of the methanol extract were 20.3% for n-hexane fraction (HF), 68.8% for diethyl ether fraction (DF), 67.1% for ethyl acetate fraction (EF), and 67.1% for the residue fraction (RE) by DPPH and 2.2% for HF, 1.6% for DF, 10% for EF, and 50% for the RE by $H_2O_2$ assay. An oxidative stress model of HK was established under a suitable concentration (1 mM). The cell viability of the RE treated group increased and the percentage of apoptotic cells decreased at concentrations of 0.005-0.02% RE compared with the $H_2O_2$ treated group. Fruit extracts of the medicinal plant R. crataegifolius showed potent antioxidant activity and the ability to relieve cell damage from $H_2O_2$ induced injury to HK.