• Journal of Life Science
• /
• v.30 no.8
• /
• pp.672-679
• /
• 2020

Distal myopathy is a clinically and genetically heterogeneous group of degenerative diseases of the distal muscle. Glycogen storage disease type IXD (GSD9D) is a metabolic distal myopathy characterized by muscle deficiency of phosphorylase kinase, a key regulatory enzyme in glycogen metabolism. Affected individuals may develop muscle weakness, degeneration, and cramps, as well as abnormal muscle pain and stiffness after exercise. It has been reported that mutations in the PHKA1 gene which encodes the alpha subunit of muscle phosphorylase kinase cause GSD9D. In this study, we examined a Korean GSD9D family with a c.3314T>C (p.I1105T) mutation in the PHKA1 gene. This mutation has not been previously reported in any mutation database nor was it found in 500 healthy controls. The mutation region is well conserved in various other species, and in silico analysis predicts that it is likely to be pathogenic. To date, only seven mutations in the PHKA1 gene have been documented, and this is the first report of Korean GSD9D patients. This study also describes and compares the clinical symptoms and pathological conditions of previously reported cases and these Korean patients. We believe that our findings will be useful for the molecular diagnosis of GSD9D.

• Proceedings of the Korean Society for Noise and Vibration Engineering Conference
• /
• 1995.04a
• /
• pp.153-158
• /
• 1995

보 및 평판의 진동에 대해서 에너지의 손실은 재료감쇠를 이용한 표면감쇠처리나 지지부에서의 감쇠처리에 의하여 증가될 수 있다. 감쇠재료를 이용한 표면감쇠처리의 예로서 보/평판 표면에 접착되는 감쇠 테이프나 코팅등은 아주 효과적임이 입증되었다. 마찬가지로 지지부 감쇠처리는 점탄성재료의 삽입에 의해서 설명될수 있다[1]. 보/평판의 감쇠에 대한 최근의 많은 연구들은 진동하고 있는 보/평판의 표면에 적용하는 감쇠 테이프 혹은 적층형태의 감쇠 처리방법에 대해서 주로 수행되었다. 일반적으로 표면감쇠처리방법은 진동감소에 아주 효과적이지만 실제 적용상에서 항상 가능한 것은 아니며, 이와 같은 경웨는 지지부 감쇠 처리방법에 의존하게 된다[2]. 감쇠특성을 갖는 점탄성재료를 실제로 적용할 때 보/평판의 유한한 길이가 점탄성재료로 지지되며 점탄성재료의 물성치와 치수에 따라서 계의 모우드 매개변수(고유진동수, 계의 손실계수 그리고 모우드형상)가 크게 달라진다. 그리고 이와 같은 계에 대해서는 지지부의 거동보다는 전체 시스템의 고유진동수와 손실계수 그리고 보 영역에서의 모우드 형상에 더 관심이 있으므로 지지부 영역을 집중매개변수(lumped parameter)로 나타내어 계를 해석할 수 있다. 일반적으로 보와 평판의 경계에서는 병진 및 회전방향의 강성 뿐만 아니라 두 강성사이에 연성효과도 동시에 발생하게 되므로 이 항을 고려하여 계를 모델링해야 한다. 본 연구에서는 우선 점탄성 재료에 의한 지지부영역의 등가 강성계수들을 구하고 경계에 강성행렬을 갖는 보의 등가시스템을 얻는다. 그리고 등가시스템의 주파수방정식으로부터 모우드 매개변수에 대한 지지부의 영향을 살펴본다. 또한 시스템이 비교적 복잡한 사각 평판의 진동에 대해서도 동강성행렬법(dynamic stiffness matrix method)을 이용하여 해석하고자 한다. 수준임이 입증되었다. 본 연구의 결과를 토대로우리나라 젊고 건강한 남성에게 적합한 무게상수는 작업자군에 대하여 25.05kg, 학생군에 대하여 20.24kg 으로 나타나 이는 미국 NIOSH 안전기준과 대체로 일치함을 발견하였다.ive structures utilized in Client/server architecture for distribution and cooperative processing of application between server and client this study presents two different data management methods under the Client/server environment; one is "Remote Data Management Method" which uses file server or database server and. the other is "Distributed Data Management Method" using distributed database management system. The result of this study leads to the conclusion that in the client/server environment although distributed application is assumed, the data could become centralized (in the case of file server or database server) or decentralized (in the case of distributed database system) and the data management method through a distributed database system where complete responsi

• Journal of the Korea Concrete Institute
• /
• v.22 no.5
• /
• pp.667-673
• /
• 2010

The near fault ground motion (NFGM) is characterized by a single long period velocity pulse with large magnitude. NFGMs have been observed in recent strong earthquakes, Northridge (1994), Japan Kobe (1995), Turkey Izmit (1999), China Sichuan (2008), Haiti (2010) etc. These strong earthquakes have caused considerable damage to infrastructures because the epicenter was close to the urban area, called as NFGM. Extensive research for the far field ground motion (FFGM) have been carried out in strong seismic region, but limited research have been done for NFGM in low or moderate seismic regions because of very few records. The purpose of this research is to investigate and analyze the seismic response of reinforced concrete bridge piers subjected to near-fault ground motions. The seismic performance of six RC bridge piers depending on three confinement steel ratios and three superstructure mass was investigated on the shaking table. From these experimental results, it was confirmed that the reduction of seismic performance was observed for test specimens with lower confinement steel ratio or more deck weight. The displacement ductility of RC bridge piers in terms of the stiffness degradation is proposed based on test results the shaking table.

• Journal of the Korean Geotechnical Society
• /
• v.21 no.6
• /
• pp.101-115
• /
• 2005

For the site characterization at two inland areas, Gyeongju and Hongsung, which represent geomorphic and geologic characteristics of inland region in Korea, in-situ seismic tests containing borehole drilling investigations and resonant column tests were peformed and site-specific seismic response analyses were conducted using equivalent linear as well as nonlinear scheme. The soil deposits in Korea were shallower and stiffer than those in western US, from which the site coefficients and site classification system in Korea were derived. Most sites were categorized as site classes C and D based on the mean shear wave velocity $(V_s)$ of the upper 30 m $(V_s30)$, ranging between 250 and 650 m/s. According to the acceleration response spectra determined from the site response analyses, the site coefficients specified in the current Korean seismic design guide underestimate the ground motion in the short-period band and overestimate the ground motion in mid-period band. These differences can be explained by the differences in the bedrock depth and the soil stiffness profile between Korea and western US. The site coefficients, $F_a$ for short-period and $F_v$ for mid-period, were re-evaluated and the site classification system, in which sites C and D were subdivided according to $V_s20,\;V_s15,\;and\;V_s10$ together with the existing $V_s30$ was introduced accounting for the local geologic conditions at inland region of the Korean peninsula. The proposed site classification system in this paper is still rudimentary and requires modification.

• Geotechnical Engineering
• /
• v.13 no.5
• /
• pp.89-100
• /
• 1997

It is very improtant to evaluate the reliable nonlinear deformational characteristics of soils not only in the analysis of geotechnical structures under working stress conditions but also for the soil dynamic problems. Field testings such as crosshole and pressuremeter tests can be used to determine the modulus of soils under in-situ conditions, but it is not possible to determine the modulus over the entire strain amplitude range. Laboratory methods such as resonant column 1 torsional shear test can be used to determine the modulus over the whole strain amplitude range, but it is very difficult to obtain the representative undisturbed samples on the sixte. For the reliable evaluation of nonlinear deformation characteristics of soils on a typical site, small strain modulus obtained from field testy and nomalized modulus reduction curve determined by laboratory bests need to be combined. In this paper, laboratory and Held testy were performed at a sixte which consisted of granite wearthered residual boils to evaluate the nonlinear deformational characteristics of coils such as the effects of strain amplitude, loading frequency, confining pressure and sample disturbance. It has been shorn that when the effects of these factors are properly taken into account, the stiffness values evaluated by various field and labrotary tests are comparable to each other fairly well. Finally, the procedure to evaluate the nonlinear deformstional characteristics of the sixte was proposed.

• Journal of the Korean GEO-environmental Society
• /
• v.19 no.9
• /
• pp.5-12
• /
• 2018

The foundation of the multi-span greenhouse structures is designed with small shallow concrete foundation considering mainly the vertical load. However, recently, due to an abnormal climate such as strong wind, horizontal load and up-lift load over design strength are applied to the foundation, causing safety problems of the greenhouse foundation. In order to reasonably evaluate the safety of greenhouse foundations, rotational and pullout stiffness expressed by the ground-foundation interaction should be evaluated, which also affects the safety of the upper structural members. In this study, three representative basic foundation types were selected by classifying greenhouse standards in Korea according to the shape, and the horizontal loading tests and theoretical calculation were performed for each foundation type. As a result of the comparison and analysis of the test and calculation, it was found that rotational resistance of the foundation is different according to the ratio of the contact area between the foundation and ground when the conditions of the foundation - ground contact surface and the mechanical properties of the ground are the same.

• Asian Pacific Journal of Cancer Prevention
• /
• v.14 no.8
• /
• pp.4567-4570
• /
• 2013

Background: Nasopharyngeal carcinoma (NPC) is the commonest radiocurable cancer in Malaysia. This study aimed to determine the treatment outcomes and late effects of radiotherapy for NPC patients treated in University Malaya Medical Centre (UMMC). Materials and Methods: All newly diagnosed patients with NPC referred for treatment to the Oncology unit at UMMC from 2004-2008 were retrospectively analyzed. Treatment outcomes were 5 years overall survival (OS), disease free survival (DFS), cause-specific survival (CSS), locoregional control (LRC) and radiotherapy-related late effects. The Kaplan-Meier method was used for survival analysis and differences in survival according to AJCC stage was compared using the log-rank test. Results: A total of 176 patients with newly diagnosed NPC were treated in UMMC during this period. Late presentation was common, with 33.5% presenting with T3-4 disease, 84.7% with N1-3 disease and 75.6% with AJCC stage 3-4 disease. Radical RT was given to 162 patients with 22.7% having RT alone and 69.3% having CCRT. The stipulated OTT was 7 weeks and 72.2% managed to complete their RT within this time period. Neoadjuvant chemotherapy was given to 14.8% while adjuvant chemotherapy was administered to 16.5%. The 5 years OS was 51.6% with a median follow up of 58 months. The 5 years OS according to stage were 81.8% for stage I, 77.9% for stage II, 47.4% for stage III and 25.9% for stage IV. The 5 years overall CSS, DFS and LRC were 54.4%, 48.4% and 70.6%, respectively. RT related late effects were documented in 80.2%. The commonest was xerostomia (66.7%). Other documented late effects were hearing deficit (17.3%), visual deficit (3.1%), neck stiffness (3.1%), dysphagia (3.4%), cranial nerve palsy (2.5%), pneumonitis (0.6%) and hypothyroidism (1.2%). Conclusions: The 5 years OS and LRC in this study are low compared to the latest studies especially those utilizing IMRT. Implementation of IMRT for NPC treatment should be strongly encouraged.

• Transactions of the Korean Society of Mechanical Engineers A
• /
• v.35 no.8
• /
• pp.869-876
• /
• 2011

It is important to analyze the dynamic behavior of counter-rotating rigid/deformable rolls in the roll-coating process, because the stability of the process is affected by the dynamic characteristics. In the present study, the effects of material property, angular velocity, and gap size on the contact pressure and contact shape of the deformable roll are numerically investigated. The behavior of two rolls with a negative gap was analyzed using the finite element method, and the material property of the deformable roll was applied with the Mooney-Rivlin coefficients of the hyper-elastic model. The contact shape is affected by the gap size, and the contact pressure mainly depends on the stiffness of the deformable roll and the gap size. To maintain a negative gap between two rolls, controls such as load and displacement controls must be used. The results indicate that displacement control can reduce the instability.

• Journal of the Korea Academia-Industrial cooperation Society
• /
• v.21 no.10
• /
• pp.283-290
• /
• 2020

In this study, a floater was developed for a frame-type solar power plant. The floater supports the frame and the solar panels. A finite element analysis was performed to design its shape and thickness, and the floater was manufactured by a rotational molding method using linear low-density polyethylene. It was found that the floater did not cause collapse and it maintained its stiffness even at 4 times the maximum load of 322.7 kgf. To perform a long-term compression test, a weight-type load application device that uses gravity was designed and manufactured. The amount of compressive deformation was measured for 7 days, and a long-term deformation equation was obtained. Even under small loads, continuous deformation was observed. However, the 10-year deformation amount for a constant load of 100 kgf was predicted to be small at about 4.64 mm. As a result, it was found that the developed floater could be used in a solar power plant on a body of water.

• Journal of the Korea institute for structural maintenance and inspection
• /
• v.20 no.3
• /
• pp.10-17
• /
• 2016

This paper presents a laboratory validation of a new approach for Finite Element Model Updating(FEMU) on short-span bridges by combining ambient vibration measurements with static load input-deflection output measurements. The conventional FEMU approach based on modal parameters requires the assumption on the system mass matrix for the eigen-value analysis. The proposed approach doesn't require the assumption and even provides a way to update the mass matrix. The proposed approach consists of two steps: 1) updating the stiffness matrix using the static input-deflection output measurements, and 2) updating the mass matrix using a few lower natural frequencies. For a validation of the proposed approach, Young's modulus of the laboratory model was updated by the proposed approach and compared with the value obtained from strain-stress tests in a Universal Testing Machine. Result of the conventional FEMU was also compared with the result of the proposed approach. It was found that proposed approach successfully estimated the Young's modulus and the mass density reasonably while the conventional FEMU showed a large error when used with higher-modes. In addition, the FE modeling error was discussed.