• Asia pacific journal of information systems
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• v.22 no.4
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• pp.101-123
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• 2012

Currently, Social Media, it has widely a renown keyword and its related social trends and businesses have been fastly applied into various contexts. Social media has become an important research area for scholars interested in online technologies and cyber space and their social impacts. Social media is not only including web-based services but also mobile-based application services that allow people to share various style information and knowledge through online connection. Social media users have tendency to common identity- and bond-attachment through interactions such as 'thumbs up', 'reply note', 'forwarding', which may have driven from various factors and may result in delivering information, sharing knowledge, and specific experiences et al. Even further, almost of all social media sites provide and connect unknown strangers depending on shared interests, political views, or enjoyable activities, and other stuffs incorporating the creation of contents, which provides benefits to users. As fast developing digital devices including smartphone, tablet PC, internet based blogging, and photo and video clips, scholars desperately have began to study regarding diverse issues connecting human beings' motivations and the behavioral results which may be articulated by the format of antecedents as well as consequences related to contents that people create via social media. Social media such as Facebook, Twitter, or Cyworld users are more and more getting close each other and build up their relationships by a different style. In this sense, people use social media as tools for maintain pre-existing network, creating new people socially, and at the same time, explicitly find some business opportunities using personal and unlimited public networks. In terms of theory in explaining this phenomenon, social capital is a concept that describes the benefits one receives from one's relationship with others. Thereby, social media use is closely related to the form and connected of people, which is a bridge that can be able to achieve informational benefits of a heterogeneous network of people and common identity- and bonding-attachment which emphasizes emotional benefits from community members or friend group. Social capital would be resources accumulated through the relationships among people, which can be considered as an investment in social relations with expected returns and may achieve benefits from the greater access to and use of resources embedded in social networks. Social media using for their social capital has vastly been adopted in a cyber world, however, there has been little explaining the phenomenon theoretically how people may take advantages or opportunities through interaction among people, why people may interactively give willingness to help or their answers. The individual consciously express themselves in an online space, so called, common identity- or bonding-attachments. Common-identity attachment is the focus of the weak ties, which are loose connections between individuals who may provide useful information or new perspectives for one another but typically not emotional support, whereas common-bonding attachment is explained that between individuals in tightly-knit, emotionally close relationship such as family and close friends. The common identify- and bonding-attachment are mainly studying on-offline setting, which individual convey an impression to others that are expressed to own interest to others. Thus, individuals expect to meet other people and are trying to behave self-presentation engaging in opposite partners accordingly. As developing social media, individuals are motivated to disclose self-disclosures of open and honest using diverse cues such as verbal and nonverbal and pictorial and video files to their friends as well as passing strangers. Social media context, common identity- and bond-attachment for self-presentation seems different compared with face-to-face context. In the realm of social media, social users look for self-impression by posting text messages, pictures, video files. Under the digital environments, people interact to work, shop, learn, entertain, and be played. Social media provides increasingly the kinds of intention and behavior in online. Typically, identity and bond social capital through self-presentation is the intentional and tangible component of identity. At social media, people try to engage in others via a desired impression, which can maintain through performing coherent and complementary communications including displaying signs, symbols, brands made of digital stuffs(information, interest, pictures, etc,). In marketing area, consumers traditionally show common-identity as they select clothes, hairstyles, automobiles, logos, and so on, to impress others in any given context in a shopping mall or opera. To examine these social capital and attachment, we combined a social capital theory with an attachment theory into our research model. Our research model focuses on the common identity- and bond-attachment how they are formulated through social capitals: cognitive capital, structural capital, relational capital, and individual characteristics. Thus, we examined that individual online kindness, self-rated expertise, and social relation influence to build common identity- and bond-attachment, and the attachment effects make an impact on both the willingness to help, however, common bond seems not to show directly impact on information sharing. As a result, we discover that the social capital and attachment theories are mainly applicable to the context of social media and usage in the individual networks. We collected sample data of 256 who are using social media such as Facebook, Twitter, and Cyworld and analyzed the suggested hypotheses through the Structural Equation Model by AMOS. This study analyzes the direct and indirect relationship between the social network service usage and outcomes. Antecedents of kindness, confidence of knowledge, social relations are significantly affected to the mediators common identity-and bond attachments, however, interestingly, network externality does not impact, which we assumed that a size of network was a negative because group members would not significantly contribute if the members do not intend to actively interact with each other. The mediating variables had a positive effect on toward willingness to help. Further, common identity attachment has stronger significant on shared information.

• Journal of the Korean Institute of Landscape Architecture
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• v.41 no.6
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• pp.20-28
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• 2013

The purpose of this study was to compare the ultra-violet(UV) radiation under the landscaping shade facilities and tree with natural solar UV of the outdoor space at summer middays. The UVA+B and UVB were recorded every minute from the $20^{th}$ of June to the $26^{th}$ of September 2012 at a height of 1.1m above in the four different shading conditions, with fours same measuring system consisting of two couple of analog UVA+B sensor(220~370nm, Genicom's GUVA-T21GH) and UVB sensor(220~320nm, Genicom's GUVA-T21GH) and data acquisition systems(Comfile Tech.'s Moacon). Four different shading conditions were under an wooden shelter($W4.2m{\times}L4.2m{\times}H2.5m$), a polyester membrane structure ($W4.9m{\times}L4.9m{\times}H2.6m$), a Salix koreensis($H11{\times}B30$), and a brick-paved plot without any shading material. Based on the 648 records of 17 sunny days, the time serial difference of natural solar UVA+B and UVB for midday periods were analysed and compared, and statistical analysis about the difference between the four shading conditions was done based on the 2,052 records of daytime period from 10 A.M. to 4 P.M.. The major findings were as follows; 1. The average UVA+B under the wooden shelter, the membrane and the tree were $39{\mu}W/cm^2$(3.4%), $74{\mu}W/cm^2$(6.4%), $87{\mu}W/cm^2$(7.6%) respectively, while the solar UVA+B was $1.148{\mu}W/cm^2$. Which means those facilities and tree screened at least 93% of solar UV+B. 2. The average UVB under the wooden shelter, the membrane and the tree were $12{\mu}W/cm^2$(5.8%), $26{\mu}W/cm^2$(13%), $17{\mu}W/cm^2$(8.2%) respectively, while the solar UVB was $207{\mu}W/cm^2$. The membrane showed the highest level and the wooden shelter lowest. 3. According to the results of time serial analysis, the difference between the three shaded conditions around noon was very small, but the differences of early morning and late afternoon were apparently big. Which seems caused by the matter of the formal and structural characteristics of the shading facilities and tree, not by the shading materials itself. In summary, the performance of the four landscaping shade facilities and tree were very good at screening the solar UV at outdoor of summer middays, but poor at screening the lateral UV during early morning and late afternoon. Therefore, it can be apparently said that the more delicate design of shading facilities and big tree or forest to block the additional lateral UV, the more effective in conditioning the outdoor space reducing the useless or even harmful radiation for human activities.

• Journal of the Korean Institute of Traditional Landscape Architecture
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• v.34 no.1
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• pp.40-52
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• 2016

Buyongjeong, a pavilion in the Rear Garden of Changdeok Palace, was appointed as Treasure No. 1763 on March 2, 2012, by the South Korea government since it shows significant symmetry and proportion on its unique planar shape, spatial configuration, building decoration, and so forth. However, the designation of Treasure selection was mainly evaluated by concrete science, in that the selection has not clearly articulated how and why Buoungjeong was constructed as a present unique form. Therefore, this study aims to clarify the identity of Buyongjeong at the time of construction by considering its historical, ideological, philosophical background and building intention. Summary are as follows: First, Construction backgrounds and characters of Buyongjeong: Right after the enthronement, King Jeongjo had founded Kyujanggak(奎章閣), and sponsored civil ministers who were elected by the national examination, as a part of political reform. In addition, he established his own political system by respecting "Kaksin(閣臣)", Kyujanggak's officials as much as "Kain(家人)", internal family members. King Jeongjo's aggressive political reform finally enabled King's lieges to visit King's Rear Garden. In the reign of King Jeongjo's 16th year(1792), Naekaksangjohoe(內閣賞釣會) based on "Kaksin" was officially launched and the Rear Garden visitation became a regular meeting. The Rear Garden visitation consisted of "Sanghwajoeoyeon(賞花釣魚宴)" - enjoying flowers and fishing, and activities of "Nanjeongsugye". Afterward, it eventually became a huge national event since high rank government officials participated the event. King Jeongjo shared the cultural activities with government officials together to Buyongjeong as a place to fulfill his royal politics. Second, The geographical location and spatial characteristics of Buyongjeong: On the enthronement of King Jeongjo(1776), he renovated Taeksujae. Above all, aligning and linking Gaeyuwa - Taeksujae - a cicular island - Eosumun - Kyujangkak along with the construction axis is an evidence for King Jeongjo to determine how the current Kyujangkak zone was prepared and designed to fulfill King Jeonjo's political ideals. In 17th year(1793) of the reign of King Jeongjo, Taeksujae, originally a square shaped pavilion, was modified and expanded with ranks to provide a place to get along with the King and officials. The northern part of Buyongjeong, placed on pond, was designed for the King's place and constructed one rank higher than others. Discernment on windows and doors were made with "Ajasal" - a special pattern for the King. The western and eastern parts were for government officials. The center part was prepared for a place where government officials were granted an audience with the King, who was located in the nortern part of Buyongjeong. Government officials from the western and eastern parts of Buyongjeong, could enter the central part of the Buyongjeong from the southern part by detouring the corner of Buyongjeong. After all, Buyongjeong is a specially designed garden building, which was constructed to be a royal palace utilizing its minimal space. Third, Cultural Values of Buyongjeong: The Buyongjeong area exhibits a trait that it had been continuously developed and it had reflected complex King's private garden cultures from King Sejo, Injo, Hyunjong, Sukjong, Jeongjo and so forth. In particular, King Jeongjo had succeded physical, social and imaginary environments established by former kings and invited their government officials for his royal politics. As a central place for his royal politics, King Jeongjo completed Buyongjeong. Therefore, the value of Buyongjeong, as a garden building reflecting permanency of the Joseon Dynasty, can be highly evaluated. In addition, as it reflects Confucianism in the pavilion - represented by distinguishing hierarchical ranks, it is a unique example to exhibit its distinctiveness in a royal garden.

• Journal of Korean Society of Forest Science
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• v.77 no.3
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• pp.303-314
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• 1988

Tong-ri beach has not enough vegetation to be enjoyed by the sea bathers and to be satisfied with preventing the disaster-damages, but mixed forest near the beach can work its funtions and the old forest of Pirus thunbergii $P_{ARL}$. near the beach do a Little. Therefore it is very urgent to plant more trees near the beach for bathers and disaster-damage prevention. This study was carried out for planning an afforestation, with reporting upon the crown amounts and soil properties of disaster-damage prevention forests of P. thunbergii $P_{ARL}$. planted on the coast sand dunes in 1970 and 1976, and with reporting upon the valuation on soil properties of the lands near the beach in order to set the afforestation site. The results are as follows : 1. In disaster-damage prevention forests, crown surface area and crown volume became increasingly greater in proportion to the height. To D.B.H., crown volume also became increasingly greater in proportion, but crown surface area was directly proportional. 2. In comparison to sail characteristics of sand dune, those of the forests were in large quantity in OM, T-N and avail. $SiO_2$, and almost in the same in avail. $P_2O_5$, but in small quantity in exchangeable canons : K, Ca, Mg and Na. 3. EC, Cl and pH were in small value in the forest soils, but CEC was in large value in those soils. 4. Above facts showed that the forests fulfill their functions for preventing disaster-damages and improve their soil properties. 5. The forests have naturally been thinned up to 34% in 17 years and 39% in 11 years, and one can easily pass through the forest(planted in 1970), because of its sufficient clear-length(2.71m) and its space to pass. 6. A plan for afforestation was oracle nut after judging several sites by the evaluation on the soil properties and considering the best relaxation and the prevention of the various disaster-damages upon which were reported in the last issue. 7. Afforestation should be kept for maintaining its appropriate density for best relaxation and disaster-damage prevention.

• Journal of Korea Entertainment Industry Association
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• v.15 no.8
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• pp.19-31
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• 2021

This study tried to identify the characteristics of college students' entrepreneurial intentions in the entertainment industry, focusing on existing literature studies. Based on this, it was intended to suggest realistic educational alternatives for university student start-ups and implications for start-up management to university start-up officials and those in charge of national start-up support policy. Therefore, the implications of this study are as follows. First, technology(item) for idea creation education, which is an essential element in the entertainment industry, how to connect ideas and products, technology methods that can increase content value, and user characteristics education within the entertainment industry will need to be continued. In addition, along with the idea education, it is necessary to increase the understanding of start-up business management such as financing, human resource management, marketing, and operation management, and furthermore, confidence education should be provided so that the possibility of success in an entertainment start-up and a sense of adventure in a new job can be developed. Second, the space and equipment necessary for start-up (club room, student start-up room, entertainment-related equipment, etc.) should be provided centering on the opinion survey of students who are interested in starting a business, and various regulations of universities and government for student start-up should be relaxed. will have to In addition, education for the formation of entrepreneurial knowledge inside and outside of the school, special lectures and consultations by experts, and on-the-spot education, etc., should be made to create more practical entrepreneurial knowledge. something to do. Third, for students wishing to start a business in the entertainment industry, it is necessary to inform their families about the field situation of the entertainment industry accurately so that their children can develop a positive perception rather than a negative perception when choosing a business field. In addition, by promoting various successful cases of college students to their families after starting a business, families should be encouraged so that their children can develop a challenging spirit about starting a business. Fourth, it should be possible to form continuous clubs or gatherings with friends who wish to start a business in the entertainment industry, and furthermore, an opportunity to listen to the opinions of friends who actually started a business through these meetings should be provided. In addition, the meeting and the formation of friends should create a place for discussion about writing a business plan, how to succeed in starting a business, and management of startups, and psychological stimulation activities should be conducted so that each other's will to start a business arises. Fifth, various knowledge related to start-up (methods for securing funds, management of start-up organizations, grasping information about the market in which they want to start a business, etc.) should be cultivated, and how to write a business plan for the various entertainment industry fields they want to start up. You will also need to train them to be practical. Also, based on this knowledge formation, students themselves should be able to respond to risks and changes that may occur in entrepreneurship. Lastly, it is necessary to increase the understanding of business start-up management, and various psychological stimulation activities are needed to make the confidence and fear of starting a business disappear.

• (The) Research of the performance art and culture
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• no.38
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• pp.349-383
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• 2019

This discussion is focused on Donghaeanbyulsingut's 'gutchum-pan to pray'. 'Gutchum-pan to pray' is repeated in almost all of the geori in Byulsingut, so it is a crucial chum-pan that can never be disregarded in understanding Byulsingutchum. Meanwhile, it supposes that Donghaeanbyulsingut is grounded on the activity of producing 'praying (words) and dance (motions)' within its relationship with the structure of capitalistic society along with the context of traditional rituals. The motion that is newly generated as a response to the concrete expression of 'praying' conducted by a mudang (a shaman), that is, the expression coming from the inside associated with the praying is seen as gutchum. This dance is bound to be in competition and interest among shaman groups, and they tend to influence one another. If praying leads to dance, a mudang can gain profits from capital as well as the value of labor. When the mudang succeeds in forming a bigger bond of sympathy with her praying, the object of praying gets more eager to select byulbi and dances a heoteunchum (impromptu dance) more vigorously. This means that a mudang's ability to perform a ritual is associated with the object of praying's consumption. With his impromptu motions, the object of praying comes to go into 'the field of consumption' within the structure of capitalistic competition before he is aware of it. Behind the communication that praying leads to dance, a lot of things are associated with one another organically. 'Gutchum-pan to pray' is generated by the continuous movement of diversity and unity that the time has within the ritual of the mudang and the object of praying. It continues to create the future 'self' that is different from the present 'self', and it means that he expects variability from the present 'self' through 'gutchum-pan to pray'. The mudang also prays for him arranging the variability of the other (the object of praying) inside her labor. In a big picture, of course, the mudang expects the variability of herself, too, which is connected to the value of her labor. The variability that they expect forms a crucial axis that determines where the flow of time and space that the 'gutchum-pan to pray' has is directed to. The contents of praying are directly related with the villagers' lives, and what leads to dance is mostly related with their jobs. This implies that what the mudang experiences in her everyday consuming activity is directly associated with the villagers' activity for earning money. In other words, the contents of that praying change constantly according to the flow of capitalistic economy. Also, those striving to respond to it before anyone else also expect better life for them by substituting their self to the 'gutchum-pan to pray' eagerly. If so, who are the ones that generate 'gutchum-pan to pray'? This can be understood through relationship among mudangs, relationship between the mudang and villagers, and also relationship among villagers. Their relationships can never be free from the concepts like labor in capitalistic society, consumption and expenditure, or time; therefore, they come to compete with the other, the present self, or the better self within the diverse relationships. This gets to be expressed in any ways, words or motions. And the range that covers the creation of either group or individual 'gutchum-pan to pray' in the village is the village community. Outside the range, it is upsized to the competition of the village unit, so individual praying may become diminished more easily. Although mudangs pray in each geori, it does not mean all praying leads to dance. Within various relationships between mudangs and villagers, 'gutchum-pan to pray' comes to be generated, repeated, and extinct. As it is mitigated to more positive competition, it does not lead to gutchum any longer. In other words, repeating 'gutchum-pan to pray' previously created has turned the object of praying into the state different from the former. Also, the two groups both have experienced the last step of Byulsingut, and at that point, praying does no longer lead to dance. In other words, from the position of the shaman group, it is the finish of their labor time and ritual performance, and from the perspective of the villagers, it means the finish of consuming activity and participation in a ritual. The characteristics of 'gutchum-pan to pray' can be summarized as follows. First, it goes through the following process: competition in the village group → competition in the group → competition among individuals. Second, repeated praying does not lead to 'gutchum'. Third, in the cases of praying for each of the occupation groups, the mudang can induce a bond of sympathy from the objects of praying directly, and this lead to dance. Fourth, the group that fails in being included in the category of praying gets to be alienated from 'gutchum-pan to pray' repeatedly.

• The Korean Journal of Pesticide Science
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• v.5 no.3
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• pp.1-11
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• 2001

New technologies will have a large impact on the discovery of new herbicide site of action. Genomics, combinatorial chemistry, and bioinformatics help take advantage of serendipity through tile sequencing of huge numbers of genes or the synthesis of large numbers of chemical compounds. There are approximately $10^{30}\;to\;10^{50}$ possible molecules in molecular space of which only a fraction have been synthesized. Combining this potential with having access to 50,000 plant genes in the future elevates tile probability of discovering flew herbicidal site of actions. If 0.1, 1.0 or 10% of total genes in a typical plant are valid for herbicide target, a plant with 50,000 genes would provide about 50, 500, and 5,000 targets, respectively. However, only 11 herbicide targets have been identified and commercialized. The successful design of novel herbicides depends on careful consideration of a number of factors including target enzyme selections and validations, inhibitor designs, and the metabolic fates. Biochemical information can be used to identify enzymes which produce lethal phenotypes. The identification of a lethal target site is an important step to this approach. An examination of the characteristics of known targets provides of crucial insight as to the definition of a lethal target. Recently, antisense RNA suppression of an enzyme translation has been used to determine the genes required for toxicity and offers a strategy for identifying lethal target sites. After the identification of a lethal target, detailed knowledge such as the enzyme kinetics and the protein structure may be used to design potent inhibitors. Various types of inhibitors may be designed for a given enzyme. Strategies for the selection of new enzyme targets giving the desired physiological response upon partial inhibition include identification of chemical leads, lethal mutants and the use of antisense technology. Enzyme inhibitors having agrochemical utility can be categorized into six major groups: ground-state analogues, group specific reagents, affinity labels, suicide substrates, reaction intermediate analogues, and extraneous site inhibitors. In this review, examples of each category, and their advantages and disadvantages, will be discussed. The target identification and construction of a potent inhibitor, in itself, may not lead to develop an effective herbicide. The desired in vivo activity, uptake and translocation, and metabolism of the inhibitor should be studied in detail to assess the full potential of the target. Strategies for delivery of the compound to the target enzyme and avoidance of premature detoxification may include a proherbicidal approach, especially when inhibitors are highly charged or when selective detoxification or activation can be exploited. Utilization of differences in detoxification or activation between weeds and crops may lead to enhance selectivity. Without a full appreciation of each of these facets of herbicide design, the chances for success with the target or enzyme-driven approach are reduced.

• Korean Journal of Environment and Ecology
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• v.21 no.6
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• pp.487-493
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• 2007

This study was carried out to evaluate soil property between campsites located at Joongsanri, Daewonsa old, Daewonsa new, Baekmudong and their adjoining forest areas in the eastern area of Jirisan National Park. The survey results showed that there existed a significant difference(p<0.05) between the two areas in soil property, such as soil bulk density, soil hardness, soil pH, water infiltration and soil respiration rates. However, there was no difference in soil property by depth between surface soil($0{\sim}15cm$) and subsurface soil($15{\sim}30cm$) for the two areas(p>0.05). In case of soil bulk density, its values in campsites were significantly higher ($1.29{\sim}1.44g/cm^3$) than in forest areas($0.95g/cm^3$), while soil hardness was significantly lower(p<0.05) in forest areas($1.44kg/cm^2$) than in campsites($2.9{\sim}4.0kg/cm^2$). Soil pore space was significantly lower in campsites($45.7{\sim}51.4%$) than in forest areas(64.3%), and soil pH in forest areas indicated pH 5.46 and that of the campsites was distributed at the range from pH 6.49 to pH 6.38. In addition, water infiltration was significantly lower in campsites($0.79{\sim}2.06cc/sec$) than in forest areas(18.7cc/sec), while soil respiration rates were significantly higher in forest areas($0.58gCO_2/m^2/h$) than in campsites($0.13{\sim}0.34gCO_2/m^2/h$).

• Proceedings of the Korean Geotechical Society Conference
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• 1991.10a
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• pp.87-102
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• 1991

It has been reported that the failure of Carsington Dam in Eng1and occured due to the existence of a thin yellow clay layer which was not identified during the design work, and due to pre-existing shears of the clay layer. The slope stability analyses during the design work, which utilized traditional circular arc type failure method and neglected the existence of the clay layer, showed a safety factor of 1.4. However, the post-failure analyses which utilized translational failure mode considering the clay layer and the pre-existing shear deformation revealed the reduction of safety factor to unity. The post-failure analysis assumed 10。 inclination of the horizontal forces onto each slice based on the results of finite element analyses. In this paper, Bishop's simplified method, Janbu method, and Morgenstern-Price method were used for the comparison of both circular and translational failure analysis methods. The effects of the pre-existing shears and subsquent movement were also considered by varying the soil strength parameters and the pore pressure ratio according to the given soi1 parameters. The results showed factor of safefy 1.387 by Bishop's simplified method(STABL) which assumed circular arc failure surface and disregarding yellow clay layer and pre-failure material properties. Also the results showed factor of safety 1.093 by Janbu method(STABL) and 0.969 by Morgenstern-Price method(MALE) which assumed wedge failure surface and considerd yellow clay layer using post failure material properties. In addition, dam behavior was simulated by Cam-Clay model FEM program. The effects of pore pressure changes with loading and consolidation, and strength reduction near or at failure were also considered based on properly assumed stress-strain relationship and pore pressure characteristics. The results showed that the failure was initiated at the yellow clay layer and propagated through other zones by showing that stress and displacement were concentrated at the yel1ow clay layer.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.93-101
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• 2016

Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.