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A New Treatment Option for Gaucher Disease Type 1: Substrate Reduction Therapy (제1형 고셔병 환자의 새로운 치료로서의 기질 감소 치료)

  • Sohn, Young Bae
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.3
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    • pp.117-122
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    • 2016
  • 제1형 고셔병(Gaucher disease type 1)은 리소좀 효소인 산성 ${\beta}$-글루코시다아제(acid ${\beta}-glucosidase$)의 결핍으로 인한 리소솜 축적 질환이다. 효소 활성도가 감소되어 기질이 축적되어, 간비종대, 빈혈, 혈소판감소증 및 골질환을 포함한 전신 증상이 발생한다. 재조합 효소 단백을 정맥 주입하는 효소 대체요법(Enzyme replacement therapy)는 지난 20년 넘게 고셔병의 표준 치료법이었다. 그러나 성공적인 효소 대체요법에도 불구하고, 심각한 폐증상과 골격 증상 등 고셔병 치료에 여전히 해결되지 않는 문제들이 남아 있다. 기질 감소 치료(Substrate reduction therapy)는 기질의 생합성을 억제하여 축적을 감소시킨다. 최근 새로운 경구용 기질감소 치료제인 엘리글루스타트(eliglustat)가 적합한 CYP2D6 대사 표현형을 가진 고셔병 성인 환자를 위한 1차 치료제로 미국과 유럽에서 승인되었다. 엘리글루스타트가 아직 한국에서는 쓰이지 않고 있지만, 본 종설에서는 문헌 검토를 통해 고셔병의 새로운 치료로서의 효소 대체요법을 소개하고자 한다. 아직 확고한 결론을 도출하기에는 연구 결과가 제한적이기는 하지만, 현재까지의 데이터에 따르면 엘리글루스타트는 임상 효능에 있어서 효소 보충 요법에 비열등성을 보인다. 장기 결과에 대한 추가 연구가 필요하지만, 엘리글루스타트의 승인은 해당 1형 고셔병 성인 환자들에게 경구 치료제라는 새로운 선택을 가능하게 하였다. 향후 국내에서 엘리글루스타트가 처방 가능해 지면, 각 환자 마다 철저한 평가를 통해 치료법을 선택할 수 있도록 해야 할 것이다. 나아가, 국내 1형 고셔병 환자들을 위해 엘리글루스타트의 사용에 관한 임상적 지침 또한 조만간 개발될 필요가 있다.

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JTAG fault injection methodology for reliability verification of defense embedded systems (국방용 임베디드 시스템의 고신뢰성 검증을 위한 JTAG 결함주입 방법론 연구)

  • Lee, Hak-Jae;Park, Jang-Won
    • Journal of the Korea Academia-Industrial cooperation Society
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    • v.14 no.10
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    • pp.5123-5129
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    • 2013
  • In this paper, it is proposed that JTAG fault injection environment and the results of the classification techniques that the reliability of embedded systems can be tested. As applying these, this is possible to quantitative analysis of vulnerable factor for system. The quantitative analysis for the degree of vulnerability of system is evaluated by faults errors, and failures classification schemes. When applying these schemes, it is possible to verify process and classify for fault that might occur in the system.

A Study on the Development of Stability Charts for Reinforced Embankments by Geotextile (Geotelrtile 보강사면의 안정도표개발에 관한 연구)

  • 서인식
    • Geotechnical Engineering
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    • v.13 no.4
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    • pp.67-74
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    • 1997
  • This paper presents the deterministic model to evaluate the two dimensional stability of geotextile-reinforced embankments on soft foundations. The potential failure surfaces in this study are assumed as the logarithmic spiral curves refracted at the boundary of layers. To facilitate the iterative calculations, we developed a program that determines the geotextile tensile force for an geoteztile-reinforced embankments. This program can be used for situations with a varying number of soil layers and soil types. A series of calculations have been made for a schematised situation. The results of these series are collected in stability charts, which are compared with those by circular potential failure surfaces. Using these charts in an early stage of the design provides a reasonable estimate of the stability of geotextile-reinforced embankments. In a later stage a more detailed calculation can be made by the developed programs.

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Properties of Fire Resistant Finishing Mortar Using Fly Ash and Glass Forming Light Weight Aggregate (플라이애시와 유리 발포 경량골재를 사용한 내화 마감모르타르의 특성)

  • Song, Hun
    • Journal of the Korean Recycled Construction Resources Institute
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    • v.3 no.4
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    • pp.374-381
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    • 2015
  • This study is investigating the fire resistant finishing materials composed of fly ash and glass forming light weight aggregate has the high temperature thermal stability. High temperatures such as a fire, cementitious materials beget dehydration and micro crack of cement matrix. From the test result, developed fire resistant finishing materials showed good stability in high temperatures. These high temperature stability is caused by the ceramic binding and low thermal conductivity of glass forming light weight aggregate. Also, alkali activation reaction of fly ash and meta kaolin not showing the decomposition of calcium hydrates. Thus, this result indicates that it is possible to fire resistant finishing light weight mortars.

Manual and Automatic Steering System Using Pulley and Electrical Clutch for Manned and Unmanned Electric Vehicle (풀리 및 전자클러치를 이용한 유무인 전기자동차용 수동 및 자동조향장치)

  • Lee, Yong-Jun;Ryoo, Young-Jae
    • Journal of the Korean Institute of Intelligent Systems
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    • v.22 no.5
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    • pp.597-602
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    • 2012
  • In this paper, a manual and automatic steering system for electric vehicles capable of manned and unmanned driving is proposed. The automatic steering systems, EPS, MDPS, used in conventional engine based car includes the problem of handle lock phenomenon while driving of overloading, therefore it has a drawback to apply to manned and unmanned electric vehicles. By using electronic clutch and pulleys, the proposed manual and automatic steering mechanism was designed so that it is possible to convert from manual to automatic steering mode. To experiment the performance of the proposed steering system, we made an experimental setup of an electric vehicle. We confirmed that the proposed manual and automatic steering system was useful for manned and unmanned electric vehicles.

Trend analysis of mobile gift certificate market (모바일 상품권 시장 동향 분석)

  • Bae, Yu-Mi;Jung, Sung-Jae;Lee, Kwang-Yong;Jang, Rae-Young;Lee, Jae-Ung
    • Proceedings of the Korean Institute of Information and Commucation Sciences Conference
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    • 2016.05a
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    • pp.189-192
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    • 2016
  • Gift card certificate law was abolished in February 1999. If the stamp duty is paid gift certificate can be issued without registration or authorization by everyone. Recently, the use of mobile devices and mobile apps became widespread as explosive increase in mobile card market. Due to the advantage of being easily available this gift, Including foods such as cakes and coffee are released in various fields, such as mobile vouchers surrogate operation, matchmaking services. The future of the voucher market is considered to be reorganized into mobile gift certificate from vouchers of paper.

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An Ultra-Lightweight RFID Authentication Protocol Using Index (인덱스를 사용한 초경량 RFID 인증 프로토콜)

  • Lee, Jae-Kang;Oh, Se-Jin;Yun, Tae-Jin;Chung, Kyung-Ho;Ahn, Kwang-Seon
    • The Journal of Korean Institute of Communications and Information Sciences
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    • v.37 no.1C
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    • pp.24-33
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    • 2012
  • Recently, the ultra-lightweight authentication RFID protocol that can actually implement on the RFID Tag is one among authentication protocols getting a concern, but recently many problems were clarified of the feature becase of the protocol which doesn't use the security algorithm. In this paper, we analyzed the problem of the ultra-lightweight authentication protocols and propose the design of ultra-lightweight RFID authentic ation protocols improving the index processing techniques. Because of improving the index processing technique in the method sending the Server authentication message to the authenticated tag, the proposed protocol is strong against the active attack which Li presents. Besides, the proposed protocol has the buffer storage of the keys and index and is strong against the asynchronous attack.

A Study of Implementation for SCORM based Learning Management System (SCORM기반 교수 학습 시스템 구현에 대한 연구)

  • Park, Hea-Sook
    • Journal of Digital Contents Society
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    • v.9 no.3
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    • pp.499-507
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    • 2008
  • This paper aims at studying the new SCORM based e-Learning system and self-course design method. To construct this aims, we have researched the merits, shortcomings and characteristics of the previous LMS(Learning Management System) and we have researched the merits, shortcomings and characteristics of SCORM(Sharable Content Object Reference Model). SCORM was suggested ADL (Advanced Distributed Learning) to elevate the reusability of learning contents. Also we have researched the related researches of SCORM, SCORM based LMS and case studies. This paper suggests the level based self learning and course design and the system based on SCORM. This system has elevated the effectiveness and satisfaction of the learners.

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EMC design for TDX-1B subscriber Board (전전자 교환기 가입자회로의 EMI 대책 설계연구)

  • 윤현보;임계재
    • The Proceeding of the Korean Institute of Electromagnetic Engineering and Science
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    • v.6 no.1
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    • pp.48-65
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    • 1995
  • This paper deals with the analysis of the unintentional radiation from the TDX-1B subscriber Board. According to the demand of digital systems and developement of high speed switching devices, electro- magnetic noise intensity and it's bandwidth occupancy in spectrum are increasing and so there is a serious possibility causing the various EMI phenomena. More accurate radiation model was established by using the Finite Difference Time Domain (FDTD) analysis, and the EMI probabilistic analysis of the digital switching system was performed. For validating the proposed model and its calculated results, on-promises test was performed and compared with the calculated results. These results may be applicable to be used for EMI model in large digital system, and prediction of EMI effect from a large digital system, and the EMI control design when designing digital system to meet inter- national EMI regulation.

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Design and Implementation of Deep Learning based System for Object Identification of Multimedia Data (멀티미디어 데이터에서 객체 식별을 위한 딥러닝 기반의 시스템 설계 및 구현)

  • Ko, Sang-Gyun;Kim, Bongjae;Kim, Jeong-Dong
    • Annual Conference of KIPS
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    • 2018.10a
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    • pp.606-608
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    • 2018
  • 최근 CCTV나 블랙박스 등 멀티미디어 데이터를 생성해내는 장치의 사용이 늘어나고 있다. 이러한 대용량 멀티미디어 데이터가 증가함에 따라 사용자가 동영상과 같은 멀티미디어 데이터 내의 객체를 식별하기 위해서는 많은 시간을 할애하여 매뉴얼하게 일일이 찾아야 하는 한계점이 있다. 본 논문에서는 사용자가 동영상 및 이미지에서와 같은 멀티미디어 데이터에서 객체를 자동으로 식별할 수 있 수 있는 딥러닝 기반의 객체 식별 및 검색 모델을 제안한다. 제안하는 객체 식별 검색은 이미지 검색과 동영상 검색을 지원한다. 이미지 검색에서는 이미지에 존재하는 동일한 객체를 검색 대상 이미지들에서 객체를 식별하고, 이미지에 존재하는 객체를 검색하여 결과로 반환한다. 또한 동영상 검색에서는 동영상에서 검색하고자 하는 객체를 식별하고 객체가 출현하는 시간을 전처리과정을 통해 기록하며, 검색하고자 하는 동영상 내에 존재하는 객체의 검색이 가능하다. 따라서 사용자가 동영상에서 객체의 검색 시 키워드 검색이 가능하여 동영상을 모두 재생하서 객체를 식별해야 하는 번거로움을 해결할 수 있다.