• Journal of The Korean Society of Inherited Metabolic disease
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• v.13 no.2
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• pp.98-103
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• 2013

Purpose: MAT-I/III deficiency by MAT1A gene mutation causes isolated hypermethioninemia, which is considered to be a clinically benign disease. But in some patients, mental retardation, developmental delay, myelination disorder may be shown. This study was performed to find out the clinical manifestations and genetic characteristics of patients with isolated hypermethioninemia. Methods: Clinical, biochemical and genetic analysis were done to 10 patients with isolated hypermethioninemia who were referred to department of pediatrics, Soonchunhyang University Hospital from March 1999 to March 2012. Results: At first visit, all patients' mean plasma methionine level was 5.5 mg/dL (2.1-14.6) and there were no increase of amino acid levels including homocystine in all patients. Serum homocysteine level was evaluated in seven patients who visited after year 2003, and ranged from 4.96 to $11.15{\mu}mol/L$ (normal < $25{\mu}mol/L$). Methionine restricted diet was started to all patients. Nine patients who managed regularly showed normal development, but one patient whose initial plasma methionine level was 14.6 mg/dL showed language delay at 1 year of age and was diagnosed as mild mental retardation (IQ=66) at 6 years of age. Genetic analysis was done to eight patients, R264H mutation was identified in seven patients. Also, both R299C and R356Q mutation were identified in one patient. Conclusion: Clinical findings in patients with isolated hypermethioninemia were generally good, but one patient showed mental retardation and language difficulty. R264H mutation which usually inherits as an autosomal dominant trait was most frequently found in our patients, and R299C/R356Q mutation were also identified.

• Clinical and Experimental Pediatrics
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• v.61 no.12
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• pp.403-406
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• 2018

Floating-Harbor syndrome is a rare autosomal dominant genetic disorder associated with SRCAP mutation. To date, approximately 50 cases of Floating-Harbor syndrome have been reported, but none have been reported in Korea yet. Floating-Harbor syndrome is characterized by delayed bony maturation, unique facial features, and language impairment. Here, we present a 6-year-old boy with a triangular face, deep-set protruding eyes, low-set ears, wide nose with narrow nasal bridge, short philtrum, long thin lips, clinodactyly, and developmental delay that was transferred to our pediatric clinic for genetic evaluation. He showed progressive delay in the area of language and cognition-adaption as he grew. He had previously undergone chromosomal analysis at another hospital due to his language delay, but his karyotype was normal. We performed targeted exome sequencing, considering several syndromes with similar phenotypes. Library preparation was performed with the TruSight One sequencing panel, which enriches the sample for about 4,800 genes of clinical relevance. Massively parallel sequencing was conducted with NextSeq. An identified variant was confirmed by Sanger sequencing of the patient and his parents. Finally, the patient was confirmed as the first Korean case of Floating-Harbor syndrome with a novel SRCAP (Snf2 related CREBBP activator protein) mutation (c.7732dupT, p.Ser2578Phefs*6), resulting in early termination of the protein; it was not found in either of his healthy parents or a control population. To our knowledge, this is the first study to describe a boy with Floating-Harbor syndrome with a novel SRCAP mutation diagnosed by targeted exome sequencing in Korea.

• Journal of the Korean Institute of Navigation
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• v.15 no.2
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• pp.39-47
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• 1991

As per the rapid development of world economics the marine traffic volume was increased accordingly and caused frequent disasters in human lives and natural environment in the consequence of accidents. As the result of the above they started to establish Vessel Traffic System(VTS) and separation scheme in waterway from 1960' to prevent the marin traffic accident but the problem of safety at sea appears now as neither fully defined nor sufficiently analysed. At the present, the dominant factor in establishing the strategy of marine traffic has been safety of navigation concerning only with the ship, but the risk of society derives almost wholly from the nature of cargo. To measure the degree of danger for each ship there is suggested concept of safety factor numbers denoting the level of latent danger in connection with ship and her cargo. In this paper, where the strategy of VTS is put on controlling density of safety factor for control area. it suggested algorithms how to assign the vessels and also to get optimal sequence of vessels located to a sector in the sense of minimizing the passage delay. For the formulation of problem, min max and 0-1 programming methods are applied and developed heuristic algorithm is presented with numerical example to improve the efficiency of calculation.

• The Journal of Korea Institute of Information, Electronics, and Communication Technology
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• v.2 no.1
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• pp.37-42
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• 2009

The digital filter is essential element in digital signal processing area. It needs a high computational burden caused by multiplying and adding. The multiplier in digital filter is a dominant element, which occupies an wide area at the field of VLSI design, needs high power-consuming and also decides critical path that affects to filter performance. In this paper we proposed the simultaneous transform method which is represented 2's complementary representation to CSD and MSD representation to solve a complexity problem and to improve a computational speed. The performance of proposed method was implemented in VHDL and applied to an digital filters, was evaluated the decreasing of critical path delay.

• Transactions of the Korean Society of Automotive Engineers
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• v.9 no.3
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• pp.60-67
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• 2001

As a method of variable valve train(VVT), Electro-Mechanical Valve(EMV) has been studied. Compared with conventional VVT system, the EMV system has a relatively simple structure. The system has two electromagnets, springs and an armature. The system can be operated by reciprocal action between armature and two electromagnets. And, the operating event can be controlled by electrical signal from controller. Therefore, reduction of emission and fuel consumption can be achieved through valve event control at each engine operating condition. In this study, characteristics of EMV system were investigated by simulations and experiments. The results of simulation and experiment show that the core shape and material characteristics are dominant parameters on magnetic force and delay time. In order to apply the system to commercial engine, it has a compact size and high stiffness springs(50N/mm) to increase the valve speed. Because of high valve seating velocity, loud noise and high impact force generated, which can lead to reduction of actuator durability. Therefore, further research is required to reduce valve seating velocity.

• Nuclear Engineering and Technology
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• v.48 no.3
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• pp.684-701
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• 2016

The Gaussian process model (GPM) is a flexible surrogate model that can be used for nonparametric regression for multivariate problems. A unique feature of the GPM is that a prediction variance is automatically provided with the regression function. In this paper, we estimate the safety margin of a nuclear power plant by performing regression on the output of best-estimate simulations of a large-break loss-of-coolant accident with sampling of safety system configuration, sequence timing, technical specifications, and thermal hydraulic parameter uncertainties. The key aspect of our approach is that the GPM regression is only performed on the dominant input variables, the safety injection flow rate and the delay time for AC powered pumps to start representing sequence timing uncertainty, providing a predictive model for the peak clad temperature during a reflood phase. Other uncertainties are interpreted as contributors to the measurement noise of the code output and are implicitly treated in the GPM in the noise variance term, providing local uncertainty bounds for the peak clad temperature. We discuss the applicability of the foregoing method to reduce the use of conservative assumptions in best estimate plus uncertainty (BEPU) and Level 1 probabilistic safety assessment (PSA) success criteria definitions while dealing with a large number of uncertainties.

• Journal of Institute of Control, Robotics and Systems
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• v.16 no.10
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• pp.1018-1023
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• 2010

The CAN (Controller Area Network) system is the most dominant protocol for in-vehicle networking system because it provides bounded transmission delay among ECUs (Electronic Control Units) at data rates between 125Kbps and 1Mbps. And, many automotive companies have chosen the CAN protocol for their in-vehicle networking system such as chassis network system because of its excellent communication characteristics. However, the increasing number of ECUs and the need for more intelligent functions such as ADASs (Advanced Driver Assistance Systems) or IVISs (In-Vehicle Information Systems) require a network with more network capacity and the real-time QoS (Quality-of-Service). As one approach to enhancing the network capacity of a CAN system, this paper introduces a CAN system with dual communication channel. And, this paper presents a distributed message allocation method that allocates messages to the more appropriate channel using forecast traffic of each channel. Finally, an experimental testbed using commercial off-the-shelf microcontrollers with two CAN protocol controllers was used to demonstrate the feasibility of the CAN system with dual communication channel using the distributed message allocation method.

• Transactions of the Korean Society for Noise and Vibration Engineering
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• v.17 no.1 s.118
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• pp.33-40
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• 2007

Rotatory two-phase transverse flux machine(TFM) is a relatively new type of motor with high power density, high torque, and low speed in comparison to conventional electrical motors. However, it has some shortcomings,.i.e. complex construction and high possibility of the magnetically induced nitration due to its inherent structure. This Paper investigates the characteristics of the magnetic force and the torque in the rotatory two-phase TFM by using the 3-D finite element method and the spectral analysis. This research shows that the average torque decreases and that the torque ripple increases as the phase delay increases. It also shows that the unbalanced magnetic force is one of the dominant excitation forces in this machine. And it proposes a new topology of rotatory two-phase TFM to eliminate the unbalanced magnetic force.

• Korean System Dynamics Review
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• v.14 no.3
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• pp.135-163
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• 2013

This study aims to analyze the feedback loops and policy simulation of price fluctuation of Korean Cattle. The Korean Cattle market shows the 'Cycle of Beef' since 1970. In general, the market for agricultural commodities exhibit repeated cycles of prices and production. Why Beef products market in Korea shows the fluctuation of cattle and beef price repeatedly for forty years? To find an answer, this paper explores the feedback structure of the dynamics of the beef market by the systems thinking and build a stock-flow diagram model for the simulation of future behavior of the market sector of the Cattle. The dynamic simulation model was developed to identify and analyze the cyclical behavior among many variables, which is the number of cattle (calves, cow, etc.), the price of cattle, the demand for beef, the desirable number of cattle, slaughter, etc. The results of this study demonstrate that dominant feedback loops between the number of cattle and livestock prices. The demand for Beef and slaughter with time delay, also the results of the simulation to explain the persistence of future price fluctuations and actions meat market until 2025.

• Journal of the Korea Institute of Information Security & Cryptology
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• v.12 no.2
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• pp.53-64
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• 2002

This paper describes a design of cryptographic processor that implements the AES (Advanced Encryption Standard) block cipher algorithm, "Rijndael". An iterative looping architecture using a single round block is adopted to minimize the hardware required. To achieve high throughput rate, a sub-pipeline stage is added by dividing the round function into two blocks, resulting that the second half of current round function and the first half of next round function are being simultaneously operated. The round block is implemented using 32-bit data path, so each sub-pipeline stage is executed for four clock cycles. The S-box, which is the dominant element of the round block in terms of required hardware resources, is designed using arithmetic circuit computing multiplicative inverse in GF($2^8$) rather than look-up table method, so that encryption and decryption can share the S-boxes. The round keys are generated by on-the-fly key scheduler. The crypto-processor designed in Verilog-HDL and synthesized using 0.25-$\mu\textrm{m}$ CMOS cell library consists of about 23,000 gates. Simulation results show that the critical path delay is about 8-ns and it can operate up to 120-MHz clock Sequency at 2.5-V supply. The designed core was verified using Xilinx FPGA board and test system.