Pseudomonas fluorescens Malate Synthase 의 Acetyl - CoA 결합부위에 있는 필수 Cysteine 잔기에 대한 입증

조성윤,김유삼 ( Sung Yoon Cho,Yu Sam Kim ) 생화학분자생물학회 1991 BMB Reports Vol.24 No.2

Malate synthase from Pseudomonas fluorescens was inhibited by iodoacetamide and N-ethylmaleimide with second-order rate constant of 43.9 M^(-1) and of 1,784 M^(-1), respectively. A double logarithmic plot of the reciprocal of the half-time of inactivation against concentration yield reaction order of 1.275 and 0.94 with respect to iodoacetamide and N-ethylmaleimide, respectively, indicating that there may be one essential residue per active enzyme. The enzyme was also rapidly inhibited by 5,5`-dithiobis (2-nitro-benzoic acid) and by 2-nitro-5-(thiocyanato)benzoate. pK_a of the functional group was determined to be 8.3 by using iodoacetamide. The inactivation by iodoacetamide and by N-ethylmaleimide was protected by acetyl-CoA but not by glyoxylate. The total number of thiol groups in the enzyme was determined to be five. These results strongly suggest that there is one essential group at the acetyl-CoA binding region among five thiol groups of the malate synthase.

Conventional한 방식과 CAD/CAM System을 이용한 완전 무치악 환자 동시 수복 증례

조성윤,이준석,Cho, Sungyoon,Lee, Joonseok 대한치과보철학회 2020 대한치과보철학회지 Vol.58 No.1

무치악 환자에게서 의치의 제작은 통상적이고 전통적인 방법을 따를 경우 최종 의치 장착까지 여러 단계를 거쳐야 하며 이로 인한 환자의 잦은 내원이 불가피하다. 또한 기공 과정 상에서 나타나는 중합수축에 의한 오차나, 의치의 파절이나 분실 시 빠른 재제작의 어려움 등의 단점이 존재하였다. 하지만 최근 치과 분야에서 computer-aided design and computer-aided manufacturing (CAD/CAM) System이 사용되기 시작하였고, 의치 제작에까지 그 영역을 넓히기 시작하였다. CAD/CAM System을 이용하여 의치를 제작하면 첫 번째 내원 시에 인상채득과 악간관계의 기록, 인공치의 선택 등이 동시에 이루어지고 두 번째 내원 시 의치의 장착이 가능하기 때문에 환자의 내원 횟수와 진료시간을 감소시킬 수 있다. 또한 3D-Printing 기술을 이용하기 때문에 중합수축에 의한 기공 오차를 감소시킬 수 있다. 현재 상용화 되어 있는 CAD/CAM System을 사용한 의치로는 DENTCA CAD/CAM denture (DENTCA Inc., Los Angeles, CA, USA)가 있다. 본 증례에서는 상, 하악 완전 무치악 환자에게서 conventional 한 방법과 DENTCA CAD/CAM Denture System을 사용하는 방법을 동시에 사용하여 두 쌍의 총의치로 수복함으로써 발음, 저작, 연하 등의 기능적인 측면뿐만 아니라 심미적인 측면에서도 양호한 결과를 얻었기에 이를 보고하고자 한다. In patients with fully edentulous jaw, treatment of complete dentures should be carried out in many stages when following the conventional methods. Therefore there were disadvantages such as multiple visits to dental clinic is inevitable. In addition, errors caused by polymerization shrinkage, which happens during the fabrication of denture, and difficulties in reproduction of damaged or lost denture were considered as disadvantages. But nowadays, computer-aided design and computer-aided manufacturing (CAD/CAM) system is widely used in dentistry and it has begun to expand its spectrum in manufacturing complete dentures. Using CAD/CAM system to fabricate complete dentures can reduce the number of patient's visit and clinical chair time, since taking impression, recording jaw relation, and selection of artificial teeth are performed at the same time during the first visit, and delivering of dentures during the second visit is possible. In addition, because 3D-Printing technology is used, errors by polymerization shrinkage can be reduced. Among the companies that fabricate complete dentures using CAD/CAM system, DENTCA CAD/CAM denture (DENTCA Inc., Los Angeles, CA, USA) is the most commercialized company. In this case, we treated patients of complete dentures using conventional complete denture method and DENTCA CAD/CAM denture system in the same patient. We would like to report this case because we have achieved good results not only in functional aspects of pronunciation, chewing, and swallowing but also in aesthetic aspects.

와이파이 수신신호세기를 사용하는 실내위치추정의 성능 향상을 위한 수정된 잔차 기반 확장 칼만 필터

조성윤,Cho, Seong Yun 제어로봇시스템학회 2015 제어·로봇·시스템학회 논문지 Vol.21 No.7

This paper presents a modified residual-based EKF (Extended Kalman Filter) for performance improvement of indoor positioning using WiFi RSSI (Received Signal Strength Indicator) measurement. Radio signal strength in indoor environments may have irregular attenuation characteristics due to obstacles such as walls, furniture, etc. Therefore, the performance of the RSSI-based positioning with the conventional trilateration method or Kalman filter is insufficient to provide location-based accurate information services. In order to enhance the performance of indoor positioning, in this paper, error analysis of the distance calculated by using the WiFi RSSI measurement is performed based on the radio propagation model. Then, an IARM (Irregularly Attenuated RSSI Measurement) error is defined. Also, it shows that the IARM error is included in the residual of the positioning filter. The IARM error is always positive. So, it is presented that the IARM error can be estimated by taking the absolute value of the residual. Consequently, accurate positioning can be achieved based on the IEM (IARM Error Mitigated) EKF with the residual modified by using the estimated IARM error. The performance of the presented IEM EKF is verified experimentally.

Development and Validation of Pediatric-Youth Hyperphagia Assessment for Prader-Willi Syndrome

조성윤,강단비,임민지,Yang Aram,김민선,김지연,노은선,권은경,최유진,한선주,박영아,곽민정,Kim Youngha,Cho Juhee,진동규 한국역학회 2022 Epidemiology and Health Vol.44 No.-

OBJECTIVES: Hyperphagia is a highly stressful, life-threatening feature of Prader-Willi syndrome (PWS). It is important to assess this complex behavior accurately over time. This study aimed to develop and validate the Pediatric-Youth Hyperphagia Assessment for Prader-Willi syndrome (PYHAP) as a tool targeting children and adolescents. METHODS: After an extensive literature review and qualitative interviews, the final version of the PYHAP with 14 questions in 3 domains (verbal [5], behavior [4], and social [5]) was developed and tested at Samsung Medical Center in Seoul, Korea from July 2018 to September 2019. Exploratory factor analysis and confirmatory factor analysis (CFA) were performed to confirm construct validity. The correlations between the PYHAP and the Korean Children’s Eating Behavior Questionnaire (K-CEBQ) were calculated to evaluate convergent and discriminant validity. Criterion validity and the validity of the response categories were also tested. RESULTS: Cronbach’s alpha coefficient of the PYHAP was 0.91. The fit indices for CFA were good (comparative fit index, 0.87; standardized root mean squared residual, 0.08). The domains of the PYHAP were closely correlated with the relevant domains of the K-CEBQ. The accuracy of the PYHAP score for predicting uncontrolled hyperphagia was good (area under the curve, 0.75; 95% confidence interval, 0.65 to 0.85). CONCLUSIONS: The PYHAP was confirmed to be a reliable and valid tool to evaluate hyperphagia in children and adolescents with PWS via caregivers’ assessments. It is recommended to use the PYHAP to communicate with parents or caregivers about patients’ hyperphagia or to monitor and manage extreme behaviors in children with PWS.

알람정보와 인접환경 분석을 통한 지하시설물 상황인식 시스템의 프로토타입 구현

조성윤,홍상기,장석우,Cho, Sung-Youn,Hong, Sang-Ki,Jang, Seok-Woo 대한공간정보학회 2011 한국공간정보학회지 Vol.19 No.3

도시의 기반 시설은 국가 또는 도시의 기술적인 발전 정도를 표현하는 지표적인 의미를 가지므로 이들을 효과적으로 관리하고 운영하는 것은 매우 중요하다. 본 논문에서는 u-City에서 지능적으로 도시의 지하 시설물을 관리하기 위한 상황인식 시스템을 제안하고, 이에 대한 알고리즘 및 프로토타입(prototype)을 개발한다. 제안된 상황인식 시스템의 알고리즘 작성은 USN(ubiquitous sensor network)환경에서 시설물을 관리하고 있는 각종 센서가 이상 신호를 감지하여 알람을 발생시킨 시점부터 상황인식 시스템이 발생한 알람의 원인을 찾아낸 후 그 결과를 도시공간정보 통합 플랫폼과 주변의 모듈에 전송해 주는 시점까지이다. 그리고 이 알고리즘을 기반으로 동작하는 상황인식 시스템의 프로토타입을 구현한 후 단위 테스트를 수행하여 구현된 프로토타입의 유효성을 검증하였다. 본 논문에서 구현된 지하시설물의 관리를 위한 알고리즘과 프로토타입은 검증 및 수정 작업을 거친 후 실제적인 시스템 개발로 연결될 것이며, 도시의 여러 가지 시설물을 관리하는 관련 시스템들의 참조모델로서 유용하게 사용될 것이라 기대한다. Since urban facilities have a signigicant meaning that represents the degree of development of nations and cities, it is very important to effectively manage and operate the facilities. In this paper, we propose a context-awareness system for managing urban underground facilities intelligently and develop its algorithm and prototype. The algorithm of the suggested system includes the period from the point when various sensors set up in USN environments sense abnormal signals and make alarms to the point when the context-awareness system analyzes the alarm and sends the analysis results to integrated platform and related modules. We then implement the prototype of the proposed context awareness system and verify the effectiveness of the system by performing unit tests. Our developed prototype will become the basis of actual system development. We expect that the suggested system will be used as a good reference model of related systems managing various types of urban facilities.

선천성 부신 과형성증(21-hydroxylase 결핍)의 신생아 선별 검사 후 진단 알고리즘

조성윤,고정민,이경아,Cho, Sung Yoon,Ko, Jung Min,Lee, Kyung-A 대한유전성대사질환학회 2016 대한유전성대사질환학회지 Vol.16 No.2

21-hydroxylase deficiency (21-OHD), most common form of congenial adrenal hyperplasia, is categorized into classical forms, including the salt-wasting (SW) and the simple virilizing (SV) types, and nonclassical (NC) forms based on the severity of the disease. Newborn screening for 21-OHD has been performed in Korea since 2006. $17{\alpha}$-hydroxyprogesterone (17-OHP) is a marker for 21-OHD and is measured using a radioimmunoassay or a fluoroimmunoassay. Premature and low birth weight infants are likely to give false positive 17-OHP findings, therefore, cutoff values for these infants should be determined based on gestational weeks or birth weight. ACTH simulation test is helpful when the 17-OHP shows equivocal increase, and it is gold standard for diagnosis of NC type. Recently, liquid chromatography linked with tandem mass spectrometry was developed for rapid, highly specific, and sensitive analysis of multiple analytes. Molecular analysis of CYP21A2 is useful for confirming diagnosis of mild SV or NC type, predicting prognoses, and genetic counseling. In order to make newborn screening for 21-OHD more efficient, early detection of boy with SW type, early determination of girl with ambiguous genitalia, detection of NC type, and overcoming of false positive in premature and low birth weight infants should be considered. Above all, early treatment should be started when the patient is suspected as having 21- OHD clinically before confirming the diagnosis to prevent adrenal crisis. Here, author reviewed recent articles of guideline and proposed guideline for 21-OHD. 선천성 부신 과형성증 중 가장 흔한 21-hydroxy-lase deficiency (21-OHD)는 신생아 선별검사에서 17-hydroxyprogesterone (17-OHP)의 증가로 조기 진단이 가능하다. 17-OHP가 애매하게 증가되는 경우에는 ACTH 자극 검사가 필요하며, 이 검사는 nonclassical (NC형) 21-OHD 진단의 gold standard이다. 전형적인 임상 증상이 없는 경우, 예를 들어 남성화가 심하지 않은 여아, 경한 simple virilizing (SV)형 남아나 신생아 선별 검사에서 발견되지 않을 수 있는 NC형 환자의 경우, 분자유전학적 검사가 진단에 도움을 줄 수 있으며, 이는 예후 에측 및 유전 상담에도 도움이 된다. 미숙아와 저체중 출생아의 경우는 17-OHP가 위양성을 보이기 쉬우므로 출생 주수나 출생 체중에 따른 cutoff 값 설정이 필요하다. 높은 위양성률을 극복하기 위해 기존 RIA방법에 비해 최근 LC-MS/MS가 민감도와 특이도를 높이는 검사로 주목 받고 있다. 21-OHD 신생아 선별 검사의 효율성을 높이기 위해서는 SW형 남아를 조기에 발견하고, 여아에서 성별 결정을 조기에 올바르게 하고, NC형 환자를 찾아내고, 미숙아/저체중 출생아/아픈 신생아에서 위양성률을 낮추어서 불필요한 재검 및 경제적/심리적 부담을 최소화 하기 위한 노력이 필요하다. 무엇보다 21-OHD가 임상적으로 확실하게 의심되는 경우에는 확진 검사에 앞서 적절한 치료가 조기에 시작되어야 한다. 저자들은 본 종설에서 21-OHD의 신생아 선별 검사 후 진단 알고리즘에 대해 최신 문헌들에 근거하여 가이드라인을 제시하는 바이다.

Skeletal Manifestations of Inborn Errors of Metabolism: A Comprehensive Retrospect

조성윤,Sung Yoon Cho The Korea Society of Inherited Metabolic Disease 2023 대한유전성대사질환학회지 Vol.23 No.1

Inborn errors of metabolism encompass a wide variety of disorders, frequently affecting bone. This review presents a comprehensive retrospect on the primary involvement of bone in inborn errors of metabolism. Primary involvement of bone in inborn errors of metabolism includes entities that primarily affect the bone marrow, mineral component or cartilage. These include lysosomal storage disorders, hypophosphatasia, and hereditary hypophosphatemic rickets. In this review, we discuss the primary involvement of bone in inborn errors of metabolism (hypophosphatasia, X-linked hypophosphatemic rickets, Gaucher disease, and mucopolysaccharidoses) along with the therapeutic agents used in clinical settings, diagnostic strategies, and general management. With the development of disease-specific targeted therapies and supportive care, more number of patients with these disorders live longer and survive into adulthood. Moreover, skeletal symptoms have become a more prominent feature of these disorders. This makes the awareness of these skeletal symptoms more important.

Characterization of a Mucolipidosis Type II Mouse Model and Therapeutic Implication of Lysosomal Enzyme Enriched Fraction Derived from Placenta

조성윤,김기용,김수진,손영배,맹세현,김치화,고아라,송정한,여성희,김경효,진동규,Cho, Sung Yoon,Kim, Ki-Yong,Kim, Su Jin,Sohn, Young Bae,Maeng, Se Hyun,Kim, Chi Hwa,Ko, Ah-Ra,Song, Junghan,Yeau, Sung-Hee,Kim, Kyung-Hyo,Jin, Dong-Kyu The Korean Society of Inherited Metabolic Disease 2012 대한유전성대사질환학회지 Vol.12 No.1

I 세포 질환(뮤코지방증 2형; MIM 252500)과 pseudo-Hurler polydystrophy (뮤코지방증 3형; MIM 252600)는 세포내 비정상적인 리소좀 관련 운송으로 인해 발병한다. 특징적인 소견으로는 섬유아 세포의 세포질에 다수의 봉입체, 뮤코다당뇨의 부재, 혈청 내 리소좀 효소 활성도의 증가, GlcNAc-phosphotransferase 활성도의 감소를 보인다. 이 연구에서는 GlcNAc-phosphotransferase 알파/베타 아형에 대한 knockout 마우스의 표현형과 생화학적 특징을 조사하였다. 또한, 태반으로부터 추출한 리소좀 농축 분획을 knockout 마우스에 투여하였을 때 체중 증가에 대한 효과를 볼 수 있는지에 대해 알아보고자 하였다. knockout 마우스는 뮤코지방증 2형 환자에서 그렇듯이 정상적인 체중 증가를 보이지 않았고 낮은 골밀도를 보였다. 게다가 knockout 마우스의 피부 섬유 아세포의 배양액에서는 리소좀 효소 활성도가 증가한 반면, 세포 내에서는 리소좀 효소 활성도가 감소되어 있는 것을 확인할 수 있었으며 이러한 특징은 뮤코지방증 2형 환자에서 볼 수 있는 특징과 일치한다. knockout 마우스의 꼬리 정맥내로 태반에서 추출한 리소좀 농축 분획을 투여한 결과, 체중이 증가하는 것을 확인할 수 있었고, 반면 생리식염수를 투여한 knockout 마우스의 경우는 체중이 증가하지 않았다. 결론적으로, knockout 마우스의 표현형과 생화학적 특징이 뮤코지방증 2형 환자와 유사하다는 것을 확인하였으며, 리소좀 농축 분획의 치료적 가능성을 증명하였다. 더 큰 범위의 동물 실험을 진행할 필요가 있으나, 이 연구는 질병에 대한 동물 모델을 개발하고 리조솜 농축 분획의 치료적 가능성을 제시하는 것을 통해 현재까지 치료가 불가능한 뮤코지방증 2형의 새로운 치료 방법의 가능성을 열었다고 볼 수 있다. 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.

Multi-model Switching for Car Navigation Containing Low-Grade IMU and GPS Receiver

조성윤,Byung Doo Kim,Young Su Cho,Wan Sik Choi 한국전자통신연구원 2007 ETRI Journal Vol.29 No.5

This letter presents a filter for a car navigation system integrating a low-grade inertial measurements unit (IMU) and a global positioning system receiver. The filter is designed according to the state variables to be estimated and the usable measurements. The usable measurements change from case to case, and the estimative state variables also change due to the measurements; therefore, multiple models must be used for real environmental maneuvers. In this letter, four models for land navigation are chosen and switched by rearranging the system matrix and resetting the error covariance matrices.

동아시아 지역 뮤코다당증 환자의 분포와 치료에 대한 현황과 전망

조성윤,진동규,Cho, Sung Yoon,Jin, Dong-Kyu 대한유전성대사질환학회 2013 대한유전성대사질환학회지 Vol.13 No.2