• Title/Summary/Keyword: Common-Gate

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A novel variant of PHEX in a Korean family with X-linked hypophosphatemic rickets

  • Kim, Sejin;Kim, Sungsoo;Kim, Namhee
    • Journal of Genetic Medicine
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    • v.19 no.1
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    • pp.27-31
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    • 2022
  • X-linked dominant hypophosphatemic rickets are the most common form of familial hypophosphatemic rickets resulting from hypophosphatemia caused by renal phosphate wasting, which in turn is a result of loss-of-function mutations in PHEX. Herein, we report a 39-year-old female with short stature and skeletal deformities and 12-month-old asymptomatic daughter. The female has a history of multiple surgical treatments because of lower limb deformities. Her biochemical findings revealed low serum phosphorus levels with elevated serum alkaline phosphatase activity and normal serum calcium levels, suggesting presence of hypophosphatemic rickets. To identify the molecular causes, we used a multigene testing panel and found a mutation, c.667dup (p.Asp223GlyfsTer15), in PHEX gene. To the best of our knowledge, this is a novel mutation. A heterozygous form of the same variant was detected in daughter, who showed no typical symptoms such as bow legs, frontal bossing, or waddling gate, but presented early signs of impaired mineralization in both X-ray and biochemical findings. The daughter was initiated onto early medical treatment with oral phosphate supplementation and an active vitamin D analog. Because the daughter was genetically diagnosed based on a family history before the onset of symptoms, appropriate medical management was possible from early infancy.

A Study on the Double Gap Blocking Device for the Improvement of Fire Resistance and Airtightness of Steel Door (강철재 도어의 내화, 기밀성 향상을 위한 이중틈새 차단장치에 관한 연구)

  • Lee, Joo-Won;Lim, Bo-Hyuk;Cho, Sung-Kwon;Lee, Hae-Yeol
    • Proceedings of the Korean Institute of Building Construction Conference
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    • 2023.05a
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    • pp.147-148
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    • 2023
  • Steel doors, which are common in general buildings, do not seal the gap between the door and the floor, so drafts, noise, dust, and lights flow from the outside, and shielding devices are installed in various materials and methods, such as adding magnetic gate paper to the side of the door or installing a gasket under the door, but performance is limited. Accordingly, in order to fundamentally solve these problems, we researched and developed a double gap blocking device that can improve fire resistance and airtightness performance in steel doors. Unlike general products, the double gap blocking device has the advantage of maximizing airtight performance by forming an air layer in the center when the door is closed, as well as greatly improving the fire resistance performance, which is the basic performance of the fire door.

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A Study on the Digital Restoration Policy Implementation Process of Donuimun Gate (돈의문의 디지털 복원 정책집행 과정에 관한 연구)

  • CHOE Yoosun
    • Korean Journal of Heritage: History & Science
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    • v.56 no.2
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    • pp.246-262
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    • 2023
  • This study analyzed policy implementation factors focusing on how Donuimun, a demolished cultural heritage, was digitally restored and the policy implementation process of Donuimun Gate restoration. Through this, the characteristics of the implementation process of the digital Donuimun Gate restoration policy promoted by public-private multilateral collaboration were examined and implications were sought for how institutions with different interests solved problems and collaborated in the implementation process. The research method was focused on policy implementation factors including policy executive factors, policy content factors, policy resource factors, and policy environment factors, and the process was analyzed for each detailed component. Along with literature analysis, in-depth interviews were conducted with participants in policy implementation. As a result of the study, first, it was found in the policy executive factor that the quick decision-making leadership of the policy manager and the flexible attitude of the person in charge of the government agency had a positive effect on preventing conflicts between different interest groups. Second, in terms of policy content, establishing a common goal that everyone can accept and moving forward consistently gave trust and created synergy. Third, in the policy implementation resource factor, the importance of the budget was emphasized. Finally, as an environmental factor for policy implementation, the opening of 5G mobile communication for the first time along with the emergence of the Fourth Industrial Revolution at the time of policy implementation acted as a timely factor. The digital Donuimun Gate was the first case of restoring a lost cultural heritage with AR and VR, and received attention and support from the mass media and the public. This also shows that digital restoration can be a model case that can be a solution without conflicts with local residents where cultural heritages are located or conflicts between stakeholders in the preservation and restoration of real objects.

Research on Broadband Millimeter-wave Cascode Amplifier using MHEMT (MHEMT를 이용한 광대역 특성의 밀리미터파 Cascode 증폭기 연구)

  • Baek, Yong-Hyun;Lee, Sang-Jin;Baek, Tae-Jong;Choi, Seok-Gyu;Yoon, Jin-Seob;Rhee, Jin-Koo
    • Journal of the Institute of Electronics Engineers of Korea SD
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    • v.45 no.4
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    • pp.1-6
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    • 2008
  • In this paper, millimeter-wave broadband MHEMT (Metamorphic High Electron Mobility Transistor) cascode amplifiers were designed and fabricated. The $0.1{\mu}m$ InGaAs/InAlAs/GaAs MHEMT was fabricated for cascode amplifiers. The DC characteristics of MHEMT are 670 mA/mm of drain current density, 588 mS/mm of maximum transconductance. The current gain cut-off frequency($f_T$) is 139 GHz and the maximum oscillation frequency($f_{max}$) is 266 GHz. To prevent oscillation of the designed cascode amplifiers, a parallel resistor and capacitor were connected to the drain of common gate device. By using the CPW (Coplanar Waveguide) transmission line, the cascode amplifier was designed and matched for the broadband characteristics. The designed amplifier was fabricated by the MHEMT MMIC process that was developed through this research. As the results of measurement, the amplifier was obtained 3 dB bandwidth of 50.37 GHz between 20.76 to 71.13 GHz. Also, this amplifier represents the S21 gain with the average 7.07 dB gain in bandwidth and the maximum gain of 10.3 dB at 30 GHz.

A Study on Characteristics of Jinsatak(陳士鐸)'s Clinic Theory (진사탁(陳士鐸) 임상 이론의 특징에 관한 연구)

  • Jeong, Kyung-Ho;Kim, Ki-Wook;Park, Hyun-Guk
    • Journal of Korean Medical classics
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    • v.22 no.3
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    • pp.31-51
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    • 2009
  • The characteristics of Jin's ideas on clinic theory can be arranged as follows. 1. Jin emphasized warming and tonifying[溫補] in treatment and the part that shows this the best is the taking care of[調理] the Vital gate[命門], kidney, liver, and spleen. His ideas were based on his understanding of a human life's origin, and was influenced by Seolgi(薛己), Joheon-ga(趙獻可) and Janggaebin(張介賓)'s Vital gate and source Gi theory(元氣說) so scholastically, he has that in common with them but was later criticized by later doctors such as Oksamjon(玉三尊) as an 'literary doctor(文字醫)' who followed the ideas of "Uigwan(醫貫)". 2. The warming and tonifying school[溫補學派], who were influenced by Taoism, said in their theory of disease outbreak[發病學說] that since one must not hurt one's Yin essence and Yang fire [陰精陽火] there is more deficiency than excess, so that was why they used tonifying methods. Jin was also like them and this point of view is universal in internal medicine, gynecology, pediatric medicine and surgery and so on. 3. Jin, who saw the negative form of pulse diagnosis[診脈] emphasized following symptoms over pulse diagnosis using the spirit of ‘finding truth based on truth[實事求是]' in "Maekgyeolcheonmi(脈訣闡微)", but emphasized 'the combination of pulse and symptoms[脈證合參]'. He understood pulse diagnosis as a defining tool for symptoms, and in "Seoksilbirok(石室秘錄)" simplified pulse diagnosis into 10 methods : floating/sunken(浮沉), slow/fast(遲數), large/fine(大小), vacuous/replete(虛實) and slippery/rough(滑澀). 4. Jin used 'large formulas(大方)' a lot that usually featured a large dose, and in " Bonchosinpyeon(本草新編)" he thought of the seven formulas(七方) and ten preparations(十劑) as the standard when using medicine. He did away with old customs and presented a 'new(新)' and 'extra(奇)' point of view. He especially used a lot of Insam(人蔘) when tonifying Gi and Geumeunhwa(金銀花) when treating sores and ulcers. 5. In the area of surgery Jin gave priority to the early finding and treatment of disease with internal treatment[內治] and was against the overuse of acupuncture. However records of surgical measures in a special situation like lung abscesses(肺癰) and liver abscesses(肝癰), and anesthetic measures using 'Manghyeongju(忘形酒)' and 'Singoiyak(神膏異藥)' and opening the abdomen or skull, and organ transplants using a dog's tongue are important data. 6. Jin stated the diseases of Gi and blood broadly. Especially in the principles of treating blood, blood diseases had to be forwarded[順] and Gi regulation[理氣] was the number one priority and stated the following two treatments. First, in "Jeonggiinhyeolpyeon(精氣引血篇)" of volume 6 of "Oegyeongmieon(外經微言)", for the rules for treating blood he stated the pattern identification of finding Gi in blood and blood in Gi. Second, he emphasized Gi regulation(理氣) in blood diseases and stated that the Gi must be tonifyed after finding the source of the loss of blood.

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A TransGate System for Convenient Wireless Internet Contents Generation (편리한 무선인터넷 컨텐츠 생성을 위한 TransGate 시스템)

  • Ryu Dong-Yeop;Han Seung-Hyun;Lim Young-Whan
    • Journal of Internet Computing and Services
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    • v.7 no.2
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    • pp.37-52
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    • 2006
  • A mobile device like ceil phone is the necessity of modern people, of ich con be easily connected to a wireless internet through such a mobile device. The demand for a wireless data communication is growing rapidly. However, agencies have not yet completed standardization of a markup language. Due to the development of the Mobile Device, agencies in this field have provided different data formats with each Mobile Device Platform. Traditionally, contents is hand-tailored to suit the target device. A key problem is that the characteristics and capabilities of the mobile device are too diverse to service the most suitable mobile contents. Because of these problems, the need for a re-usable document description language increases. In this paper, we defined Template file that is common data to service mobile devices. We proposed a method that could be an effective wireless web service though design and the implementation of the Call manager & the XSL Generator. In the methodology, when requesting a wireless internet service, a mobile device finds out markup language and a hardware specification of the mobile device through the Call Manager component supports. The XSL Generator component creates the XSL file dynamically that is the most suitable to a device. Finally, contents is serviced to each device by XSLT. It can generate a wireless page more easily by reusing the existing web contents through such course. Therefore, it can save the time and expense for generating a wireless page.

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A $G_{4}$ Sequence within PHR1 Promoter Acts as a Gate for Cross-Talks between Damage-Signaling Pathway and Multi-Stress Response

  • Jang, Yeun-Kyu;Kim, Eun-Mi;Park, Sang-Dai
    • Animal cells and systems
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    • v.6 no.3
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    • pp.271-275
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    • 2002
  • Rph1 and Gisl are damage-responsive repressors involved in PHR1 expression. They have two $C_{2}$H/ sub 2/ zinc finger motifs as putative DNA binding domains and N-terminal conserved domain with unknown function. They are also found in the human retinoblastoma binding protein 2 and the mouse jumonji- encoded protein. The repressors are able to bind to A $G_{4}$ sequence within a 39-bp sequence called upstream repressing sequence of PHR1 promoter (UR $S_{PHR1}$) responsible for the damage-response of PHR1. We report here that Rph1 is predominantly localized in the nucleus as examined by fluorescence microscopic analysis with GFP-Rph1 fusion protein. On the basis of the fact that the A $G_{4}$ sequence that is recognized by Rph1 and Gisl is also recognized by Msn2 and Msn4 in a process of stress response, we a1so tried to examine the in vivo function of A $G_{4}$ and the role of Msn2 and Msn4 in PHR1 expression. Our results demonstrate that Msn2 and Msn4 are actually required for the basal transcription of PHR1 expression but not for its damage induction. When A $G_{4}$ sequence was inserted into the minimal promoter of the cyc1-LacZ reporter, the increased LacZ expression was observed indicating its involvement in transcriptional activation. The data suggest that the A $G_{4}$ is primarily required for basal transcriptional activation of PHR1 or CYC1 promoter through the possible involvement of Msn2 and Msn4. However, since the A $G_{4}$ is also involved in the repression of PHR1 via Rphl and Gisl, it is proposed that A $G_{4}$ functions as either URS or upstream activating sequence (UAS) depending on the promoter context.t.

Design of a Low Power Capacitor Cross-Coupled Common-Gate Low Noise Amplifier (캐패시터 크로스 커플링 방법을 이용한 5.2 GHz 대역에서의 저전력 저잡음 증폭기 설계)

  • Shim, Jae-Min;Jeong, Ji-Chai
    • The Journal of Korean Institute of Electromagnetic Engineering and Science
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    • v.23 no.3
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    • pp.361-366
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    • 2012
  • This paper proposes a low power capacitor cross-coupled 5.2 GHz band low noise amplifier(LNA) using the current-reused topology with the TSMC 0.18 ${\mu}m$ CMOS process. The proposed 5.2 GHz band LNA uses a capacitor cross-coupled $g_m$-boosting method for reducing current flow of circuit and a current-reused topology to decrease total power dissipation. The parallel LC networks are used to reduce size of spiral inductors. The simulation results show high gain of 17.4 dB and noise figure(NF) of 2.7 dB for 5.2 GHz.

A Neural Metwork's FPGA Realization using Gate Level Structure (게이트레벨 연산구조를 사용한 신경합의 FPGA구현)

  • Lee, Yun-Koo;Jeong, Hong
    • Journal of Korea Multimedia Society
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    • v.4 no.3
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    • pp.257-269
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    • 2001
  • Because of increasing number of integrated circuit, there is many tries of making chip of neural network and some chip is exit. but this is not prefer because YLSI technology can't support so large hardware. So imitation of whole system of neural network is more prefer. There is common procedure in signal processing as in the neural network and pattern recognition. That is multiplication of large amount of signal and reading LUT. This is identical with some operation of MLP, and need iterative and large amount of calculation, so if we make this part with hardware, overall system's velocity will be improved. So in this paper, we design neutral network, not neuron which can be used to many other fields. We realize this part by following separated bits addition method, and it can be appled in the real time parallel process processing.

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Implementation of Remote Control and Monitoring System using Embedded Web Server (임베디드 웹서버를 이용한 원격 감시 및 제어 시스템 구현)

  • 최재우;노방현;이창근;차동현;황희융
    • Journal of the Korea Academia-Industrial cooperation Society
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    • v.4 no.3
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    • pp.301-306
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    • 2003
  • We have designed embedded web server system and ported Linux operating system version 2.4.1 at our system. And then We implemented to control and monitor widely separated hardware. Web server is the Boa web server with General Public License. We designed for this system using of Cirrus logic's EP7312 ARM core base processor and connecting input and output device at GPIO port of EP7312. Device driver of General purpose I/O for Linux OS is designed. And then the application program controlling driver is implemented to use of common gate interface C language. User is available to control and monitor at client PC. This method have benefit to reduce the Expenditure of hardware design and development time against PC base system and have various and capacious application against firmware base system.

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