• 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 Computational Design and Engineering
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• v.10 no.2
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• pp.133-142
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• 2005

This paper proposes an object-oriented conceptual information model of mechanical assemblies, named open assembly model (OAM). The proposed assembly model primarily defines hierarchical relationships between parts and subassemblies. Together with the assembly hierarchy. the model also provides a way to represent tolerances, kinematic information, and parametric assembly constraints. Relational information such as mating conditions and degree of freedom between parts and subassemblies is captured via assembly features and relationships thereof. The information model is described using class diagrams of the Unified Modeling Language (UML), and instance diagrams are used to exemplify the proposed information model. The conceptual model presented in this paper is an integrated information model for assembly representation, which could supply necessary information for tolerance analysis and synthesis, kinematic simulation, and assembly simulation. Such a conceptual information model plays an important role for the exchange of information between modeling, analysis and planning systems. Hence, the proposed model could serve as a framework for developing data exchange standards of mechanical assemblies. The proposed model is demonstrated through a case study of a planetary gear assembly.

• Journal of Navigation and Port Research
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• v.38 no.6
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• pp.673-681
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• 2014

Due to the increased demand for safety and security requirements on the port infrastructure, the harbor tranquility is one of the important parameter in the mooring basin of harbor. It relates keenly to berthing/unberthing and cargo handling works but also it is an important indicator to get the minimum water area as the safe refuge. Hupo harbor is a national coastal harbor located in east coast of Korea and a development plan for a new marina near the entrance is being carried out including berth layouts, breakwater extensions, 300m marina berths, dredging and land reclamation works. The new plan will impact on calmness of the existing port. Therefore, it is necessary to analyze in complex the variation of wave height and direction caused by wave refraction, diffraction, shoaling and reflection from the incident waves from outside the harbor. In order to check the calmness inside a harbor, the numerical models are being used currently need fundamental reviews according to the difference of results which depend on their respective features. In this study, hence, it was introduced the validity of numerical models by comparing the computational results with the hydraulic model experiment. The current investigations contribute to the existing development recommendations and provide further solutions for port planning.

• Transactions of the Korean Society of Mechanical Engineers
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• v.17 no.1
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• pp.38-45
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• 1993

The feature based modeling approach is useful for post-CAD related works such as process planning and NC part programming. This paper describes the development of 'FeaTURN' system which is feature based NC part programming system for turning operation. The programming task in 'FeaTURN' system becomes easy and effective with the assistance of feature icons. The manufacturing attributes can be handled toghther with the features during input procedure. The cutter location data (CLD) is determined by the processor module. The post process module converts the CL data to machine control data (MCD). Also, the system graphically displays the tool path.

• Journal of the Korean Institute of Landscape Architecture
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• v.28 no.4
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• pp.132-137
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• 2000

In June, the City of Seoul held a design competition for $\ulcorner$Seoul Park$\lrcorner$in Paris to promote friendly relations with its sister city. The purpose of this paper is to articulate the design concept of a scheme submitted by the author. The author interpreted the object of this design competition as follows: if we regards a park not as one of urban planning facilities but as a space for expressing a culture, $\ulcorner$Seoul Park$\lrcorner$in Paris is a space expressing Korean culture, or a culture of the City of Seoul in Paris, France. Three points were emphasized in this scheme: 1. Physical and non-physical aspects of Korean culture, or a culture of the City of Seoul were expressed separately. In physical part, a traditional Korean garden was reappeared to express its authenticity compared to its counterpart, French classical garden - its formal and grand style. In nonphysical part, Seoul's features and its citizen's facial expression were engraved on 'free standing walls' named 'Seoul Expression'. In addition, Korean traditional and modern performing arts will be performed in a square named as 'Seoul madang' surrounded by the free standing walls. 2. A space clearly divided by the fence was necessary to distinguish a traditional Korean garden from the place which looks like an amusement park. Traditional wall, mounding and pine tree groves were included. 3. Bamboo grove with the way taking a walk was introduced. The author expected that Parisian feels oriental mystery, the sound of wind, and the time lag of past and present in this sounding bamboo grove.

• Korean Institute of Interior Design Journal
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• v.22 no.1
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• pp.299-306
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• 2013

While artists and architects both recognize the importance of well-designed exhibition spaces, they have rarely shared, through direct and open communication, their thoughts on what constitutes an ideal architectural context for art. Remy Zaugg's text offers a rare and valuable insight on artists' conception of an ideal space for art, and his words and diagrams have directly influenced some of the successful architectural projects for art in the recent past. By analyzing the original content of Zaugg's writings, and highlighting Zaugg's key design elements from three sample projects, all of which mention Zaugg's text as a direct reference, we can compile a list of core design elements for constructing an ideal space for art. Three museums closely incorporated all but two of Zaugg's seven core planning elements, and the remaining two recommendations on the luminous ceiling and gallery distribuition were more subjectively interpreted. These deviant components in turn become unique features to each project, suggesting proper areas of intervention for architects as authors.

• Journal of Astronomy and Space Sciences
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• v.29 no.4
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• pp.375-380
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• 2012

It is a crucial matter to select a landing site for landers or rovers in planning the Mars exploration. The landing site must have not only a scientific value as a landing site, but also geographical features to lead a safe landing for Mars probes. In this regard, this study analyzed landing site of Mars probes and rovers in previous studies and discussed the adequacy of the landing site to scientific missions. Moreover, this study also examined domestic studies on the Mars. The frameworks of these studies will guide the selection of exploration sites and a landing site when sending Mars probe to the Mars through our own efforts. Additionally, this paper will be used as the preliminary data for selection of exploration site and a landing site.

• The Plant Pathology Journal
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• v.30 no.2
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• pp.220-227
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• 2014

The GacS/GacA system in the root colonizer Pseudomonas chlororaphis O6 is a key regulator of many traits relevant to the biocontrol function of this bacterium. Proteomic analysis revealed 12 proteins were down-regulated in a gacS mutant of P. chlororaphis O6. These GacS-regulated proteins functioned in combating oxidative stress, cell signaling, biosynthesis of secondary metabolism, and secretion. The extent of regulation was shown by real-time RT-PCR to vary between the genes. Mutants of P. chlororaphis O6 were generated in two GacS-regulated genes, trpE, encoding a protein involved in tryptophan synthesis, and prnA, required for conversion of tryptophan to the antimicrobial compound, pyrrolitrin. Failure of the trpE mutant to induce systemic resistance in tobacco against a foliar pathogen causing soft rot, Pectobacterium carotovorum SCCI, correlated with reduced colonization of root surfaces implying an inadequate supply of tryptophan to support growth. Although colonization was not affected by mutation in the prnA gene, induction of systemic resistance was reduced, suggesting that pyrrolnitrin was an activator of plant resistance as well as an antifungal agent. Study of mutants in the other GacS-regulated proteins will indicate further the features required for biocontrol-activity in this rhizobacterium.

• Journal of muscle and joint health
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• v.13 no.2
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• pp.119-129
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• 2006

Purpose: The purpose of this study was to analyze current treatments and characteristics of patients with hemophilia. Method: research design is a descriptive study exploring the current treatments, accompanied disorders, features related to the disease and subjects' social-demographic characteristics. The sample consisted of 218 hemophilia patients registered at Korea Hemophilia Foundation. Results: 80.3% of the subjects were diagnosed of this disease before the age of 10. 64.7% of them had a hemophilia familial history. 74.3% of them had disabilities. Main characters related to the disease were lack of the 8th factor and bleeding tendencies. Levels of severity had significant relationship with the number of hemorrhagic experiences. A common comorbidity found was hepatitis C. A treatment agent frequently use was GREENMONO INJ. and most patients injected it by themselves. 47.7% of the subjects used a dosage of 100 unit, and 45.4% of them injected to once a week. Conclusion: These data will be used as valuable knowledge for planning a patient care and managing their symptoms.

• Fashion & Textile Research Journal
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• v.14 no.1
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• pp.75-82
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• 2012

Compared to off-line, on-line word-of-mouth has fast dissemination speed and extensive effects based on interactive features. Moreover, in the age of Web 2.0, on-line media has made consumers evolve from simple purchasers to producers, who intervene into product manufacturing through on-line WOM. According to this, this study is conducted to clarify how consumer's prosumer propensity affects WOM in detail when purchasing fashion products on-line through website interactivity and perceived usefulness of WOM. The results are as follows. 1. Consumer's prosumer propensity is classified in four dimensions: the propensity to participate, the propensity to relate, the propensity to amuse and the propensity to create. 2. The sample has shown low prosumer propensity overall, and there were no gender differences. 3. Testing structural equation model, it was clarified that the higher the consumer's prosumer propensity, the higher the consumer's evaluation of website interactivity and thus the greater the WOM effect through its perception of usefulness. 4. There were some differences in the path of structural equation model according to consumer's prosumer propensity. From the results, it can be concluded that consumer's prosumer propensity is a key factor in the on-line WOM. Therefore fashion businesses should actively utilize consumer's prosumer propensity to apply their opinion in the product planning stage or use it as the means of company-friendly viral marketing.