• Title/Summary/Keyword: CPS1

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A Fatal Case of Neonatal Onset Carbamoyl Phosphate Synthetase I Deficiency with Homozygous CPS1 Mutation (동종 접합자 CPS1 돌연 변이를 동반한 신생아 발병형 Carbamoyl Phosphate Synthetase 1 결핍증의 치명적 사례)

  • Yun, Jung Ha;Shin, Seung Han;Ko, Jung Min;Kim, Ee-Kyung;Kim, Han-Suk
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.1
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    • pp.18-22
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    • 2018
  • Carbamoyl phosphate synthetase I (CPS1) deficiency is a rare autosomal recessive urea cycle disorder that causes hyperammonemic crisis. CPS1 is the first enzyme encoded by the CPS1 gene, which catalyzes the first step of the urea cycle. In CPS1 deficiency, ammonia, the toxic metabolite produced by the interruption of the urea cycle, is accumulated in the blood and brain, leading to hyperammonemic encephalopathy and irreversible brain damage. Here, we report a fatal case of neonatal-onset CPS1 deficiency in a 4-day-old girl presenting with recurrent seizures, who was revealed to be homozygous for c.1529delG ($p.Gly510Alafs^*5$).

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The brand-new compound CPS219 exhibits anti-oxidant and estrogen-like actions

  • Kyung-Min, Jeong;Yu-Jin, Choi;Seung-Hwan, Kim;Hyun-Ja, Jeong
    • CELLMED
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    • v.13 no.2
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    • pp.2.1-2.9
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    • 2023
  • CPS219, a new concept compound composed of coffee (CO), Pueraria Tomsonii flower (PF), and Sambou bamboo saltTM (BS), was developed as a coffee beverage to alleviate menopausal symptoms. The purpose of this study is to evaluate the anti-oxidant and menopausal alleviating effects of CPS219 combined as the optimal ratio of each component selected through sensory evaluation and blind consumer test. For CPS219, the optimal ratio of CO, PF, and BS was determined to be 1:0.1:0.017 through various sensory evaluations and blind consumer tests. CPS219 significantly enhanced the superoxide dismutase-like activity compared to the CO or CO plus PF (CP). The proliferation of MCF-7 cells was considerably increased after 24 hours by treatment with CO, CP, or CPS219, but only CPS219 significantly boosted the proliferation of MCF-7 cells after 48 hours. Moreover, CPS219 had an estrogen-like effect by dramatically increasing the expression of estrogen receptor-β mRNA in MCF-7 cells but not CO and CP. Treatment of MCF-7 cells with CO, CP, or CPS219 did not cause any cytotoxicity. In conclusion, these findings imply that anti-oxidant and estrogen-like properties of CPS219 can be used to prevent and cure postmenopausal symptoms.

The Association of Intra-Annual Cambial Activities of Pinus koraiensis and Chamaecyparis pisifera planted in Mt. Worak with Climatic Factors (월악산에 식재된 잣나무와 화백나무의 형성층 활동과 기후인자와의 관계)

  • Seo, Jeong-Wook;Choi, En-Bi;Ju, Jeong-Deok;Shin, Chang-Seop
    • Journal of the Korean Wood Science and Technology
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    • v.45 no.1
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    • pp.43-52
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    • 2017
  • This study was fulfilled to verify the durations of cambial activity and analyze the associations of degree days and precipitation with the initiation of cambial activity and intra-annual wood formation for Pinus koraiensis and Chamaecyparis pisifera planted at Mt. Worak, respectively, by monitoring of their intra-annual cambial activities. And more, the reason was also analyzed why the DBH of Chamaecyparis pisifera known as planted in the same year could be classified as two groups (CPL: ${\phi}30cm$, CPS: ${\phi}15cm$). The intra-annual cambial activity was monitored using mini-cores (${\phi}2mm$) and they were collected in 2-week interval between April and October. However, between April and May and between middle September and October expected as the initiation and cessation of the cambial activity, respectively, it was fulfilled in 1-week interval. The average number of tree rings for PK (30) was less than CPS (37) and CPL (38), whereas the average ring width of PK (4.12 mm) was wider than CPS (1.84 mm) and CPL (3.97 mm). In the comparison of ring widths between CPL and CPS, CPL was 2.13 mm wider than CPS, however, excepting CPS 1 (0.83 mm), the average ring widths of CPS 2 (2.42 mm) and CPS 3 (2.73 mm) in the last 3 years were close to the average of CPL (2.71 mm). The initiation of cambial activity for PK was between 1 and 21 April, which was 1 week earlier than CPL and CPS (excepting CPS 1) and the cessation was between 1 and 22 September. The longest growing season therefore was 157.3 days (${\pm}3.3$) and it was longer than CPL ($145.7{\pm}6.6days$) and CPS ($148.0{\pm}15.1days$). In CP groups there were wide variations for the cessation of cambial activity and also there were the meaningful linear relationship between the growing seasons and the ring widths (r = 0.69, p < 0.064). The cambial activity in PK was initiated when degree days were between 99 and 134 and in CPS (excepting PCS 1) and CPL between 134 and 200. Excepting CPS 3, the false ring was observed in all samples collected on 21 July when drought stress was high due to low precipitation from June to the beginning of July.

Feasibility Analysis on the Attack Graph Applicability in Selected Domains

  • Junho Jang;Saehee Jun;Huiju Lee;Jaegwan Yu;SungJin Park;Su-Youn Hong;Huy Kang Kim
    • Journal of the Korea Society of Computer and Information
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    • v.28 no.5
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    • pp.57-66
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    • 2023
  • In this paper, a research trend of attack graph studies for Cyber-Physical System (CPS) environments is surveyed, and we analyse the limitations of previous works and prospect the future directions. 35 among around 150 attack graph studies conducted within 5 years target CPS, and we inspect key features of CPS environment in the security aspect. Also, we categorize and analyze target studies in the aspect of modelling physical systems and considering air gaps, which are derived as key features of the security aspects of CPS. Half of 20 research that we surveyed do not reflect those two features, and other studies only consider one of the two features. In this circumstance, we examine challenges that attack graph studies on CPS environment face. Finally, we expect state-led studies or studies targeting open-spec commercial CPS will dominate.

The Effect of Supplemental Cardioplegia Infusion before Anastomosis in Patients Undergoing Heart Transplantation with Long Ischemic Times

  • Kim, Hong Rae;Jung, Sung-Ho;Yang, Junho;Kim, Min Su;Yun, Tae-Jin;Kim, Jae-Joong;Lee, Jae Won
    • Journal of Chest Surgery
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    • v.53 no.6
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    • pp.375-380
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    • 2020
  • Background: Prolonged ischemic time is a risk factor for primary graft dysfunction in patients who undergo heart transplantation. We investigated the effect of a supplemental cardioplegia infusion before anastomosis in patients with long ischemic times. Methods: We identified 236 consecutive patients who underwent orthotopic heart transplantation between February 2010 and December 2014. Among them, the patients with total ischemic times of longer than 3 hours (n=59) were categorized based on whether they were administered a complementary cardioplegia solution (CPS) immediately before implantation (CPS+, n=30; CPS-, n=29). Results: The mean total ischemic times in the CPS+ and CPS- groups were 238.1±30.1 minutes and 230.1±28.2 minutes, respectively (p=0.3). The incidence of left ventricular primary graft dysfunction (CPS+, n=6 [20.0%]; CPS-, n=5 [17.2%]; p=0.79) was comparable between the groups. In the Kaplan-Meier survival analysis, no significant difference in overall survival at 5 years was observed between the CPS+ and CPS- groups (83.1%±6.9% vs. 89.7%±5.7%, respectively; log-rank p=0.7). No inter-group differences in early mortality (CPS+, n=0; CPS-, n=1 [3.4%]; p=0.98) or complications were observed. Conclusion: The additional infusion of a cardioplegia solution immediately before implantation in patients with longer ischemic times is a simple, reproducible, and safe procedure. However, we did not observe benefits of this strategy in the present study.

Analysis of Research Trends of Cyber Physical System(CPS) in the Manufacturing Industry (제조 분야 사이버 물리 시스템(CPS) 연구 동향 분석)

  • Kang, Hyung-Muck;Hwang, Kyung-Tae
    • Informatization Policy
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    • v.25 no.3
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    • pp.3-28
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    • 2018
  • The purpose of this study is to analyze the research trends and present future research directions in the field of Cyber Physical System (CPS), a key element in the 4th Industrial Revolution, Industry 4.0, and Smart Manufacturing that are currently promoted as important innovation agenda both at home and abroad. In this study, (1) the concepts of industry 4.0, smart manufacturing and CPS are summarized; (2) analysis criteria of these fields are established; and 3) analysis results are presented and future research direction is proposed. 74 overseas and 8 domestic literature on manufacturing CPS from 2013 to 2017 are identified through 'Google Scholar Search'. Major results of the analysis are summarized as follows: (1) research on a common methodology and framework for the manufacturing CPS needs to be done based on the analysis of the existing methodologies and frameworks of various perspectives; (2) in order to improve the maturity of the manufacturing CPS, it is necessary to study actual deployment and operations of CPS, including the existing systems; (3) it is necessary to study the diagnostic methodology that can evaluate manufacturing CPS and suggest improvement strategy; and (4) as for the detailed model and tool, it is necessary to reinforce research on SCM production planning and human-machine collaboration while considering the characteristics of CPS.

FMI based Real-time CPS Distributed Simulation Framework using OMG DDS middleware (OMG DDS 미들웨어를 이용한 FMI기반 실시간 CPS 분산 시뮬레이션 프레임워크)

  • Hong, Seokjoon;Joe, Inwhee;Kim, Wontae
    • Journal of IKEEE
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    • v.22 no.1
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    • pp.6-13
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    • 2018
  • To develop highly dependable CPS, M&S(modeling and simulation) is very important. It is not easy to model any CPS whole system in a single simulation tool because each simulation tool is optimized for modeling each different part of the CPS. The FMI is the standard for M&S between different simulation tools. The DDS is a communication middleware suitable for large-scale real-time data transmission. In this paper, we proposed FMI based CPS real-time distributed simulaton framework using DDS. To evaluate the performance of the proposed framework, we performed distributed simulation using IEEE HLA/RTI and OMG DDS middleware and measured and compared the execution time of the entire simulation. From the simulation results, we can confirm that the simulation execution time using DDS is at least 1.14 times faster compared to execution time using HLA/RTI.

A Case of Lethal Neonatal Type Carbamoyl Phosphate Synthetase 1 Deficiency with Novel Mutation of CPS1 (새로운 CPS1 유전자 돌연변이에 의한 신생아형 carbamoyl phosphate synthetase 1 결핍 1례)

  • Suh, Seung-hyun;Kim, Yoo-Mi;Byun, Shin Yun;Son, Seung Kook;Kim, Seong Heon;Kim, Hyung Tae;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.2
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    • pp.109-114
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    • 2016
  • Carbamoyl phosphate synthetase 1 (CPS1) deficiency is an autosomal recessive urea cycle disorder which causes hyperammonemia. CPS1 is the first enzyme step in the urea cycle and almost patients present their symptoms during neonatal period. We report a case of CPS1 deficiency in a boy who developed symptoms including lethargy and seizure at 3 days of age. The ammonia level was up to $2,325{\mu}mol/L$, sodium benzoate (250 mg/kg/d) and high calories of both dextrose and lipid was promptly administered. Central access by experienced pediatric surgeon and emergent continuous hemodialysis by pediatric nephrologist was performed within 3 hours and ammonia was less than $100{\mu}mol/L$ at 5 days of age. Currently, he has showed excellent response to treatments including scavenging drugs and a low-protein diet. Despite of diffuse increasing signal intensity on cerebral white matters and basal ganglia on brain MRI, his development and weight gain were good at the last follow-up at 11 months of age. Molecular assay of the CPS1 gene demonstrated that patient had compound heterozygous for c.1529del ($p.Gly510Alafs^*5$) in exon 14 and c.3142-1G>C (IVS25(-1)G>C) in intron 25 and exon 26 boundary. The splicing mutation was novel mutation and inherited from patient's mother. Here, we report a neonatal lethal type CPS1 deficiency patient having novel mutation.

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Development of Chollian Bipropellant Propulsion System (천리안위성 이원추진시스템 개발)

  • Han, Cho-Young;Lim, Cheol-Ho
    • Journal of the Korean Society for Aviation and Aeronautics
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    • v.19 no.1
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    • pp.94-100
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    • 2011
  • This paper gives a brief overview of the COMS CPS development process from start to finish. The manufacturing techniques used for CPS were founded on established generic processes that have been developed and proven on previous satellite programs, and have used the expertise and facilities in the framework of international collaboration. Manufacture and testing of the CPS were successfully accomplished, and COMS CPS demonstrated good performance in the launch phase.