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Characterization of Mutations in Bruton's Tyrosine Kinase(Btk) Gene from Unrelated 3 X-linked Agammaglobulinemia(XLA) Families in Korea (국내 X-관련성 범저감마글로불린혈증 세가족에 대한 Bruton's Tyrosine Kinase 단백질 발현 및 유전자 변이 분석)

  • Song, Chang-Hwa;Jo, Eun-Kyeong;Park, Jeong-Kyu;Kim, Jung-Soo;Hong, Soo-Jong;Lee, Jae-Ho
    • Clinical and Experimental Pediatrics
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    • v.45 no.3
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    • pp.302-310
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    • 2002
  • Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells(PBMC) from three XLA families in Korea. Methods : Heparinized venous blood samples were collected from four XLA patients and additional family members in three unrelated XLA families. Mononuclear cells were separated from their blood and the intracellular Btk protein was characterized by a flow cytometry. The mutation analysis was performed using direct sequencing. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in the patients with XLA. We observed a novel deletion and two point mutations within introns(intron 1 and intron 18) resulting in alternative splicings. In XLA family 2, a 980 bp deletion(from intron 9+191 T to intron 10-215 C) including exon 10 was found in patient P2. He was the only sporadic case in this study, because his mother and brother showed a normal Btk expression by flow cytometry. Conclusion : These identified genetic alterations support the molecular heterogeneity of Btk gene in XLA disease. Additionally, by means of flow cytometric analysis, we diagnosed three hypogammaglobulinemia patients as XLA. Advancements in diagnostic methods has facilitated a prompt and definite diagnosis of this disease.

A Study on the Characteristics of Museum Projects by Richard Meier (리챠드 마이어의 미술관 특성에 관한 연구)

  • 김용립
    • Archives of design research
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    • v.12 no.4
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    • pp.231-241
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    • 1999
  • This study propose to analyze the design method and the form elements of museums, of works by Richard Meier, and through the analysis, spacial characteristics of museums will be understood. The museum works of Richard Meier, as an exhibition space, not only display the art works efficiently, but they also offer visitors the opportunity to experience the art of architecture, as a cultural space. Richard Meier, when working on the projects, has utilized the design methods and the architectural language, learned from Mies van der Rohe and Le Corbusier, resourcefully. Having the structural grid as basis, the rational rectangular forms were intended for exhibition space, while the circular and partial circular forms of geometry were utilized in formative space. This was able to maintain the balance between functional and formative space. In the museums of his works, the ramp and the glass wall separated from the structure become very important means of expression. The ramps, not only make people to enjoy the interior and exterior of museum, but also able them to see the works of art from different distances and angles repeatedly and the large glass wall reveals the various shapes of interior to exterior. In comparing with the design method and language of two masters mentioned, the design principles and elements, developed by Meier were applied to the site plans, exhibition space planning and elevations to manifest its originality. The design concept, derived from the urban fabric and historical buildings around, gave harmony to the museum with its surroundings, and employing the deformed axis brought variation and the effect of diversion to the site plan. The exhibition space is much vitalized by the well arrangement of various exhibition fixtures in the museum. The exhibion fixtures, which the partitions, shelves, miches, and stages were put together in flexibility, play multiple roles as partitions dividing spaces, as furniture displaying art works, and as elements creating forms. The systematically arranged fixtures, also produce several visual axes and centers, which have visitors appreciate the works of art in various perspectives, hence create a unique environment.

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Income Level Necessary for Old-age Living by Income Status (노령계층의 소득계층별 필요소득수준 연구)

  • 석재은
    • Korea journal of population studies
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    • v.26 no.1
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    • pp.79-113
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    • 2003
  • This study is intended to explore an income level necessart for the old-age by income Status. Firstly, it is verified whether there is significant difference between old-age and working age or not. Secondly, if their difference is found, it will be identified that it is determined by certain factors. Thirdly, it is brought out needful income level for the old-age living through analysing old-age consumption expenditure level over working age. The results in this research are as follows. Firstly, there is a significant difference between old-age and working age. Secondly, the major factors which generated difference between old-age and working age consumption expenditure are income, household size, and age. Thirdly, the income level necessary for oldage living is on average 61 %. By income status, it is 90-100% for low income status, 60-70% for middle income status, 50-60% for high income status.

Elementary School Teachers' Concept of Combustion - Focus on Change of Gases - (연소에 대한 초등교사의 개념 - 기체변화를 중심으로 -)

  • Shin, Ae-Kyung;Moon, Hyun-Sook;Kang, Min-Seog
    • Journal of The Korean Association For Science Education
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    • v.31 no.6
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    • pp.942-957
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    • 2011
  • The purpose of this research was to examine the concept of elementary school teachers of combustion. The participants were selected from the elementary school teachers who had various career experiences, 6th grade science teaching experiences, and academic backgrounds on science. For the purpose of this study, 12 elementary school teachers took the concept-test formed five questions on combustion and were interviewed. The concept-test was composed with 'The definition of combustion', 'The reason that the candle was blown out when glass was closed.', 'The existence of oxygen and carbon dioxide of before and after combustion in glass', 'Combustion of iron', 'Combustion products'. And the collected data by semi-structured interviews based on responses to the concept-test. During the analysis of the data, additional interviews by phone, e-mail and Internet messenger were conducted if necessary. The answers to each question were classified into three levels: (Scientific-concept(S), Partial-concept(P), Misconcept(M)). The research results showed that all teachers had misconceptions or partial-concept of more than 50 percent of each question. Teachers who had the 6th grade science teaching experience acquired scientific concepts of the combustion more than teachers who did not have the 6th grade science teaching experience. We should develop visualization materials about the change of gases during combustion and use these materials for implementation of the scientific concept.

The Management of Artworks in the Collection of Ihwajang House after the April 19 Student Revolution: An Examination of the "Property Ledger of Dr. Rhee" (1970) in the Collection of the Presidential Archives (이화장 소장 미술품의 4·19 이후 처리경위 -대통령기록관 소장 「이박사(李博士) 재산태장(財産台帳)」(1970)의 검토)

  • Yoon, Insu
    • MISULJARYO - National Museum of Korea Art Journal
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    • v.101
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    • pp.66-86
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    • 2022
  • This paper focuses on the document "Property Ledger of Dr. Rhee," which was a supplement to the Disposal of the Late Dr. Rhee's Property written by the secretarial office of the Cheongwadae (the Korean presidential residence) in 1970. The document contains a list of 311 items, including furnishings and artworks, once owned by President Syngman Rhee. The items had been in the collection of the Cheongwadae, but in February 1970 they were transferred to the family of the late President Rhee. The background for creating this list is as follows. After the April 19 Student Revolution in 1960, a survey was conducted of Rhee's movable assets, including artworks in his private residence Ihwajang. Their transfer to the National Museum was discussed at one point, but it was decided to use them at the Cheongwadae instead. In 1963, however, the transfer of these assets to the National Museum was ordered, and some duplications of the private possessions were sent to Ihwajang House. In 1970, furnishings and artworks stored at the Cheongwadae were also returned to Ihwajang House, and the "Property Ledger of Dr. Rhee" document was produced. This ledger lists works by artists from the Joseon period through the 1960s. It is difficult to identify each of these artworks since no photos are attached and the details provided are minimal. Nevertheless, this ledger is valuable in that the scale of artworks in the collection of the Gyeongmudae (the former name for the Cheongwadae) and Ihwajang House can be partially identified and in that it helps us understand how the issues surrounding the reversion of ownership of these artworks were resolved.

Development of a Practical Field Data Acquisition Device for Civil and Environmental Engineering Applications (토목환경공학분야의 응용을 위한 실용적인 현장자료수집기의 개발)

  • 여운광;윤병만;이종국;허정호
    • Journal of Korean Society of Coastal and Ocean Engineers
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    • v.9 no.4
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    • pp.176-181
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    • 1997
  • A practical, easy-to-use data acquisition device has been developed for use in civil and envirionmental engineering applications. It uses the PC parallel port for the acquisition and data transfer and is simple enough to be setup and run in laboratory and field experiment conditions. The usual limitations of data storage capacity of several data loggers was solved by using PC resources. Also versatile programming capability using high level languages of the PC programming environment such as C and Visual Basic enables the data acquisition device to be configured as a general and practical data acquisition system. An example of effective uses of the device is. provided for the water environment measurement at the Bangwha bridge construction site. The in site. test proved the device to be practical in civil and environmental engineering applications.

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A Study on the Store Hold Design for Fashion Sales Environment as Store Identity - With a focus of the brand "A" named - (SI작업의 일환으로 판매환경 집기디자인에 관한 연구 - A 브랜드를 중심으로 -)

  • 이미란;권석준;박우장
    • Journal of the Korea Furniture Society
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    • v.10 no.1
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    • pp.33-42
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    • 1999
  • This study aims to organize the research ways of interior design for clothing stores as a management strategy in the market changing rapidly. The public met their needs in a mass production system. However, the brand-name companies began to create the conditions of consumption for people, there appeared people with new lifestyles and new way of thoughts so that their propensity to consume resulted in consumption activities, and it become varied, individual, and highly qualified. Therefore, in view of consumers' priority, based on SI sales environment this paper aims to create a new image by re-designing the interior and furniture of shops which will help to enhance the image of brand name. Therefore, the brand-name companies will expect a large sale for the new product and it will apply to sales environments of other products and influence them. The purpose of this paper is to find a desirable way to increase sales efficiency for the companies. In order to get a concept and propriety of sales environment design, examined mutual relation to each study of interior design, marketing, and fashion design. Through referring to the documents research of these fields I could get a conception and basic structure for them. Based on the theoretical knowledge, this study was made to show to examine and generalize the existing sales environments and situations and set up a new concept for this. It will apply to interior design and store holds and present a new design alternative for improvement.

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The Indoor Propagation Modeling for Indoor Wireless LAN Service (실내 무선 랜 서비스를 위한 실내 전파 모델링)

  • 김진웅;김기홍;윤영중;석재호;임재우;신용섭
    • The Journal of Korean Institute of Electromagnetic Engineering and Science
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    • v.13 no.5
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    • pp.426-435
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    • 2002
  • In this paper we present an indoor propagation model for indoor wireless LAN service in the ISM band. We primarily use a 3D ray tracing as well as a patch scattering model in order to take into account the indoor fixtures. Therefore input parameters such as indoor environment parameters and antenna's types, polarizations are considered. As the results, we present fading characteristics and rms delay spread from time delay spread. In order to investigate the accuracy of the presented model, comparisons of predictions with measurement and simulations are performed in indoor wireless LAN service environments. The results show that measurements and simulations are very similar. Therefore in this paper, the effect of presented indoor propagation model is confirmed.

Design of prototype that SVG editing system (구조화된 벡터 그래픽 표현을 위한 SVG 편집시스템의 프로토타입 설계)

  • 김철순;김창수;정회경
    • Proceedings of the Korean Institute of Information and Commucation Sciences Conference
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    • 2003.05a
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    • pp.281-284
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    • 2003
  • Development of internet had centered on that offer universality of expression. But, graphic processing of expression center has many inconveniences because of method that is not structural in World Wide Web. Announced SVG that is XML application to permit efficient expression and storage of vector graphic expression, share in W3C to supplement these things. Therefore, because using SVG document in this treatise, edit vector graphic through interface that could edit expressed graphic object directly structural edit and screen that is offered in graphic edit system form and designed SVG edit system to express. This system may use usefully to efficient XML graphics processing on the GIS system and Mobile service environment and so on.

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A Design and Implementation of the Tree-based Document Editing System for XML Application (XML 어플리케이션을 위한 트리 기반 문서 편집 시스템의 설계 및 구현)

  • Kim, Young-Chul;Kang, Chun-Kil
    • The KIPS Transactions:PartD
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    • v.11D no.4
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    • pp.959-966
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    • 2004
  • This paper describes a design and implementation of the tree-based document editing system for XML application, available at the structure-oriented environment. This system converts DTD to ASTD( Syntax Tree Definition) to support syntax-directed editing for valid document, considers the extensibility to add new tools and supports multiple entry parser for real-time document validation. It is expected that this paper contributes related XML application document editing system development model.