• 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.

• The Korean Journal of Air & Space Law and Policy
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• v.30 no.2
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• pp.279-310
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• 2015

The Montreal Convention is an agreement that governs the liability of air carriers for injury and death to passengers travelling internationally by air. The Montreal Convention serves as the exclusive legal framework for victims and survivors seeking compensation for injuries or death arising from accidents involving international air travel. The Montreal Convention sets monetary liability caps on damages in order to promote the financial stability of the international airline transport industry and protect the industry from exorbitant damages awards in courts that would inevitably bankrupt an airline. The Convention allows a litigant suing under the Convention to avoid the liability caps in instances where the airline's culpability for the injury or death is the direct result of negligence, another wrongful act, or an omission of the airline or its agents. The Montreal Convention identifies specific locations as appropriate venues to advance claims for litigants seeking compensation. These venues are closely tied to either the carrier's business operations or the passenger's domicile. In March 2015, in an act of suicide stemming from reactive depression, the co-pilot of Germanwings flight 9525 intentionally crashed the aircraft into the French Alps killing the passengers and the remaining crew. Subsequent to the crash, there were media reports that Lufthansa made varying settlement offers to families of the passengers who died aboard the flight ranging from $8,300 USD to $4.5 Million USD depending on the passengers' citizenship. The unverified offers by Lufthansa prompted outcries from the families of the decedent passengers that they would institute suit against the airline in a more plaintiff friendly jurisdiction such as the United States. The first part of this article accomplishes two goals. First, it examines the Montreal Convention's venue requirement along with an overview of the recoverable damages from countries comprising the citizenship of the passengers who were not American. The intentional crash of Germanwings flight 9525 by its First Officer encompasses the possibility that Lufthansa may be exposed to unlimited compensatory damages beyond the liability caps contained in the Convention. The second part of this article explores the application of the Convention's liability limits to the Germanwings flight to demonstrate that the likelihood of escaping the liability limits is slim.

• Journal of Broadcast Engineering
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• v.24 no.1
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• pp.118-131
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• 2019

In order to provide 8K UHD contents of terrestrial broadcasting with a large capacity, the terrestrial broadcasting system has various problems such as limited bandwidth and so on. To solve these problems, UHD contents transmission technology has been actively studied, and an 8K UHD broadcasting system using terrestrial broadcasting network and communication network has been proposed. The proposed technique is to solve the limited bandwidth problem of terrestrial broadcasting network by segmenting 8K UHD contents and transmitting them to heterogeneous networks through hierarchical separation. Through the terrestrial broadcasting network, the base layer corresponding to FHD and the additional enhancement layer data for 4K UHD are transmitted, and the additional enhancement layer data corresponding to 8K UHD is transmitted through the communication network. When 8K UHD contents are provided in such a way, user can receive up to 4K UHD broadcasting by terrestrial channels, and also can receive up to 8K UHD additional communication networks. However, in order to transmit the 4K UHD contents within the allocated bit rate of the domestic terrestrial UHD broadcasting, the compression rate is increased, so a certain level of image deterioration occurs inevitably. Due to the nature of UHD contents, video quality should be considered as a top priority over other factors, so that video quality should be guaranteed even within a limited bit rate. This requires packet scheduling of content generators in the broadcasting system. Since the multiplexer sends out the packets received from the content generator in order, it is very important to make the transmission time and the transmission rate of the process from the content generator to the multiplexer constant and accurate. Therefore, we propose a variable transmission scheduler between the content generator and the multiplexer to guarantee the image quality of a certain level of UHD contents in this paper.