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β-ureidopropionase Deficiency

  • Jun Hwa Lee
    • Journal of Interdisciplinary Genomics
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    • v.5 no.1
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    • pp.5-11
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    • 2023
  • β-ureidopropionase (β-UP) is an enzyme that catalyzes the final step in the pyrimidine degradation pathway, which converts β-ureidopropionate and β-ureidoisobutyrate into β-alanine and β-aminoisobutyrate, respectively. β-UP deficiency (UPB1D; OMIM # 613161) is an extremely rare autosomal recessive inborn error disease caused by a mutation in the UPB1 gene on chromosome 22q11. To date, approximately 40 cases of UPB1D have been reported worldwide, including one case in Korea. The clinical manifestations of patients with UPB1D are known to be diverse, with a very wide range of manifestations being previously reported; these manifestations include completely asymptomatic, urogenital and colorectal anomalies, or severe neurological involvement, including global developmental delay, microcephaly, early onset psychomotor retardation with dysmorphic features, epilepsy, optic atrophy, retinitis pigmentosa, severely delayed myelination, and cerebellar hypoplasia. Currently, diagnosis of UPB1D is challenging as neurological manifestations, MRI abnormalities, and biochemical analysis for pyrimidine metabolites in the urine, plasma, and cerebrospinal fluid also need to be confirmed by UPB1 gene mutations. Overall, treatment of patients with UPB1D is palliative as there is still no definitive curative treatment available.

Analysis of Quality Change Based on Storage Conditions to Set the Use-by date of Bakery Breads (베이커리 식빵의 소비기한 설정을 위한 보관 방법에 따른 품질 변화 연구)

  • Sun Hye Hwang;Ji Yeon Choi;Min Joo Kim;Yong Sun Cho
    • Journal of Food Hygiene and Safety
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    • v.38 no.4
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    • pp.236-245
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    • 2023
  • In this study, the quality safety limit period of seven types of bakery bread was analyzed, and their use-by date was calculated. For evaluating product quality, storage conditions were set as 5, 15, 25, and 35℃ for 50 days, and moisture, microorganisms, sensory characteristics, and dominant bacteria were examined. The quality and safety standards followed the Korea Food Code and Korean industrial standards (KS). The results showed that all products stored at 5℃ satisfied the standard for bacterial count for day 50, but the sensory quality was below the standard level. Samples stored at 15℃ showed high variability from 3-39 days. At 25℃, a quality safety limit period of 2-20 days was set, and one sample was found to have the same shelf life. Bread stored at 35 ℃ had the shortest quality safety limit period. Considering a safety factor of 0.87, a use-by date period of 1.7-13.1 days was calculated. Therefore, setting the use-by date according to the product type is necessary, even for the same product category. Among the bread products sold in bakeries, those managed as room temperature products (1-35℃) can be distributed and stored in a temperature range of up to 35℃. Overall, this study demonstrates the importance of setting a quality retention period based on the product characteristics and carefully considering the safety factor.

A Study on the Shape of the Upper Arm and the Armscye Line (상완부 및 진동형태에 관한 연구)

  • 김순분
    • Journal of the Korean Society of Costume
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    • v.30
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    • pp.201-210
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    • 1996
  • The perpose of this study is to compare and to analyze the characters of the shape of up-per arm and armscye line between young and aged women. The subjects were measured with the Mar-tin's anthropometer and with free curve ruler. 10 items in the shape of upper arm were measured and 16 items in the shape of armscye line measured indirectly based on the measured shape taken from the subjects with free curve ruler and both date were analyzed. Mean standard deviation f-test factor analysis were performed for the statistical analysis of the date. The main results were as follows: 1) The shape of the upper arm is long and narrow in youg women compared with aged. 2) The length of the axilla of the aged women were longer than that of young women. 3) There are significant differences young and aged women on the shape of armscye line. THe length of from the front point of axilla to shoulder point width and depth of the shape of the armscye line length of axilla of aged women were longer than that of young. but the length of from the back point of axilla to shoulder point of yyoung women was longer than aged. 4.) The front point of axilla was existed lower than back point of axilla in aged women while front point of axilla was existed higher than the back point in young. 5) Through the factor analysis two factors were obtained from the shape of the upper arm and five factors from the shape on armmscye line in both groups. Factor compornents in the shape of the up per arm were length width and girth of that and in the shape of the armscye line were width and length of upper and lower back and front part of the shape of the armscye line depth of the axilla. he items in the same factor were resemble each other between two groups.

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Ultra Precision Machining of Optical Pick-up Aspheric Glass Objective Lens Molding Press Core for Optical Information Storing (I) (광정보저장용 광픽업 대물렌즈 성형용 코어 초정밀 형상가공 (I))

  • Kim, Min-Jae;Lee, Jun-Key;Hwang, Yeon;Kim, Hye-Jeong;Kim, Jeong-Ho
    • Proceedings of the Korean Institute of Electrical and Electronic Material Engineers Conference
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    • 2009.06a
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    • pp.290-290
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    • 2009
  • Blu-ray Disk, the next generation optical information storing equipment used Blu-ray is the next generation leading storing equipment that has capacity is about from six times to thirty-five times bigger than the existing CDs, DVDs. Especially, we need elaborate optical pick-Up equipment to record and recognize detailed date. Moreover, Blu-ray disk has so narrow track-pitch so it is used high NA(Numerical Aperture) aspheric glass objective lens. In this research, we processed optical pick-up aspheric glass objective lens molding press core by parallel grinding method with ultra precision machining and mold core surface measured form accuracy(PV), surface roughness(Ra).

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Financing Problem for an Early Retirement Scheme (조기은퇴제도를 위한 자금조달문제)

  • Lee, Sang-Un
    • The Journal of the Institute of Internet, Broadcasting and Communication
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    • v.21 no.3
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    • pp.151-157
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    • 2021
  • This paper deals with financing an early retirement scheme problem(FERSP) with minimum initial cash and filling up maximum financial interest. For this problem, Guéret et al. programming the Mosel, and Edvall merely realize the CPLEX Branch-and-Cut MIP Solver program to get the optimal solution. But there is no clear rule to finding the solution. This paper suggests calculation formula of bond number decision-making that the reverse from long arrival due date to short. Then we optimize and confirm the bonds number in accordance with continuative effect of the arrival due date. The shortage prepare with the principal and interest of one year deposit(saving) reversely calculation formula.

Satellite Link Simulator Development in 100 MHz Bandwidth to Simulate Satellite Communication Environment in the Geostationary Orbit (정지궤도 위성통신 환경모의를 위한 100 MHz 대역폭의 위성링크 시뮬레이터 개발)

  • Lee, Sung-Jae;Kim, Yong-Sun;Han, Tae-Kyun
    • Journal of the Korea Institute of Military Science and Technology
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    • v.14 no.5
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    • pp.842-849
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    • 2011
  • The transponder simulator designed to simulate the transponder of military satellite communication systems in the geostationary orbit is required to have time delay function, because of 250 ms delay time, when a radio wave transmits the distance of 36,000 km in free space. But, it is very difficult to develop 250 ms time delay device in the transponder simulator of 100 MHz bandwidth, due to unstable operation of FPGA, loss of memory data for the high speed rate signal processing. Up to date, bandwidth of the time delay device is limited to 45 MHz bandwidth. To solve this problem, we propose the new time delay techniques up to 100 MHz bandwidth without data loss. Proposed techniques are the low speed down scaling and high speed up scaling methods to read and write the external memory, and the matrix structure design of FPGA memory to treat data as high speed rate. We developed the satellite link simulator in 100 MHz bandwidth using the proposed new time delay techniques, implemented to the transponder simulator and verified the function of 265 ms time delay device in 100 MHz bandwidth.

Design of a Smart Application for Remote Diagnosis in Ubiquitous Computing Environment (유비쿼터스 컴퓨팅 환경에서 원격진단을 위한 스마트 응용의 설계)

  • Oh, Sun-Jin
    • The Journal of the Institute of Internet, Broadcasting and Communication
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    • v.16 no.4
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    • pp.81-87
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    • 2016
  • With the rapid growth of the up-to-date smartphone and wireless network technologies, huge and various types of smart applications using these technologies are actively developed recently. Especially, multiplex types of smart applications using smartphone are developed and diffused with the rapid development of the ubiquitous sensor network technology using various sensors and the mobile computing technology that enables us to get network services at any time in any places. In this paper, we design a smart application that can accurately diagnose and process the current state of the local environment, objects, and persons remotely based on the context information such as local ecology, circumstances, medical or healthcare records and realtime sound or motion pictures using up-to-date samrtphone technology on the USN based mobile computing environment.

Female Adolescents' Clothing Behavior as Related to Self-Efficacy and Sensation Seeking (여고생의 의복행동과 자기효능감 및 감각추구성향과의 관련 연구)

  • 고애란;이수경
    • Journal of the Korean Society of Clothing and Textiles
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    • v.22 no.7
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    • pp.931-941
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    • 1998
  • The purpose of this study were 1) to identify the effects of the self-efficacy, sensation seeking and delinquency on female adolescents' clothing behavior, and 2) to identify the differences in self-efficacy, sensation seeking, deliquency and clothing behavior in relation to the demographic variables. The data were collected from 435 high school girls living in Seoul, via a self-administered questionnaires, and were analyzed by factor analysis, Peason's correlation, multiple regression, one-way ANOVA and SNK test. The result of this study were as follows: As the result of analyzing the effects of self-efficacy, efficacy, sensating seeking and delinquency on each of clothing behavior variables, fashion leadership was explained by the factors such as social self-efficacy, delinquency and boredom susceptibility, and psychological clothing dependence by boredom susceptibility, social self-efficacy, and thrill and adventure, clothing exhibition by boredom susceptibility, delinquency, and general self-efficacy, social approval by boredom susceptibility, and general self-efficacy, preference for up-to-data style by delinquency, boredom susceptibility by delinquency, boredom susceptibility. There were significant differences between the two types of school in general self-efficacy, thrill and adventure, experience seeking, delinquency, preference for up-to-data style, and appearance related delinquency. Location of school had the effects on experience seeking, delinquency, fashion leadership, and appearance related delinquency, and socioeconomic status on fashion leadership.

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The Profit Analysis of Straddle Sell by Entry-Time and Delta at System Trading (시스템 트레이딩에서 진입시점과 델타에 따른 스트래들 매도의 성능 분석)

  • Ko, Young Hoon;Kim, Yoon Sang
    • Journal of Korea Society of Digital Industry and Information Management
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    • v.6 no.1
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    • pp.151-157
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    • 2010
  • This paper proposes the Pyramid strategy which is based on the straddle sell. The Pyamid strategy has multi-entry features with starting date and delta parameters. And It is hedged against a loss by mutual trades and dynamic ripples. This paper analyzes the profit and MDD(maximum draw down) of the Pyramid strategy on system trading. The portfolio tool is used for the experiment which is one of the Multicharts' package. The Multicharts is a good trading system of recent years. For the experiment, three call options and three put options are used at october in 2009. Two parameters are used which are the starting date from first October to twentieth October in 2009 and delta from eight percent to fifty percent. As a result, the profit of composite option is about 3 million won. If the strategy starts before the beginning of option month, investors feel uncomfortable because of a large MDD. If a delta belows 20%, it shows high profit and the ratio of profit and MDD builds up a low value. However a low delta makes frequent trades and results in a loss unless increasing entry levels which mean more amount of investment. This work provides a safer trade system than native option trades. It is important how much levels of multi-entry are acceptable. And an amount of investment with appropriate levels of multi-entry is a subject of a future study.

A Study On D-Shortage Control Hyper System Using MRP and JIT (MRP와 JIT를 융합한 D-결품관리 시스템에 관한 연구)

  • 조동수;이근희
    • Journal of Korean Society of Industrial and Systems Engineering
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    • v.15 no.25
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    • pp.63-74
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    • 1992
  • This study proposes D- shortage control system which is a convenient tool for cooperative companies to reduce shortages which frequently break out between the manufacturing companies and the cooperative companies. On the ground of theoretical analysis of MRP and JIT system, D- shortage control system sets up a schedule that secures the delivery date by precedent scheduling( D-) comparing with MRP It also syncronizes business, production and release, and builds the pull system comparing with JIT. The factors causing shotages are the scheduling absurdity and the controlling absurdity. The scheduling absurdity can be settled by the calculating required quantity method of MRP and the controlling absurdity can be settled by daily control of business, production and pruchasing fuctions by the pull system of JIT. And the inventory and the WIP can be reduced by the operating of PULL system and by the settlement of D- shortage control practices. The Application of D- shortage control system, therefore, enables the rationalization of logistics and reduces the inventory And it leads to the reinforced competitiveness and the security of subsistence of manufacturer by the cost ruduction, the reduction of financial difficulty, and the insurance of the delivery date.

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