• Molecules and Cells
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• 제28권3호
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• pp.139-148
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• 2009

Cell death has been traditionally classified in apoptosis and necrosis. Apoptosis, known as programmed cell death, is an active form of cell death mechanism that is tightly regulated by multiple cellular signaling pathways and requires ATP for its appropriate process. Apoptotic death plays essential roles for successful development and maintenance of normal cellular homeostasis in mammalian. In contrast to apoptosis, necrosis is classically considered as a passive cell death process that occurs rather by accident in disastrous conditions, is not required for energy and eventually induces inflammation. Regardless of different characteristics between apoptosis and necrosis, it has been well defined that both are responsible for a wide range of human diseases. Glycogen storage disease type I (GSD-I) is a kind of human genetic disorders and is caused by the deficiency of a microsomal protein, glucose-6-phosphatase-${\alpha}$ ($G6Pase-{\alpha}$) or glucose-6-phosphate transporter (G6PT) responsible for glucose homeostasis, leading to GSD-Ia or GSD-Ib, respectively. This review summarizes cell deaths in GSD-I and mostly focuses on current knowledge of the neutrophil apoptosis in GSD-Ib based upon ER stress and redox signaling.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권4호
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• pp.239-247
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• 2014

Purpose: There are no studies of hepatic glycogen storage diseases (GSDs) other than type I and III in Korea. We aimed on investigating the characteristics of hepatic GSDs in Korea diagnosed and followed at a single center. Methods: We retrospectively analyzed patients who were diagnosed as GSD and followed at Samsung Medical Center from January, 1997 to December, 2013. Clinical manifestations, laboratory results, treatment, and prognosis were investigated. Results: Twenty-one patients were included in the study. The types of 17 patients were confirmed by enzyme activity tests and/or gene analysis. GSD Ia was diagnosed in 7 patients (33.3%), Ib in 1 patient (4.8%), III in 2 patients (9.5%), IV in 1 patient (4.8%), and IX in 6 patients (28.6%). Types other than GSD I constituted 52.9% (9/17) of the patients diagnosed with a specific type of hepatic GSD. The median age at presentation was 2 years. Hepatomegaly was observed in 95.2%, elevated liver transaminases in 90.5%, and hyperlactacidemia in 81.0% of the patients. The duration for follow-up was $77{\pm}62.0$ months. Uncooked corn starch was initiated in all the patients. No mortality was observed during the follow-up period, and liver transplantation was performed in 14.3%. Conclusion: Types other than GSD I comprised more than half of the patients diagnosed with a specific type of hepatic GSD. Clinical suspicion and thorough evaluation of hepatic GSDs in Korea should be focused not only on GSD I, but also on other types.

• 생명과학회지
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• 제30권8호
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• pp.672-679
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• 2020

원위 근병증은 원위 근육의 퇴행성 질환이며 임상적, 유전적으로 이질적인 그룹이다. 당원 축적병 9D (GSD9D)는 원위 근병증 중 하나이며, 근육의 포스포릴라아제키나아제(phosphorylase kinase) 결핍을 특징으로 하는 대사 근병증이다. GSD9D 환자는 운동 후 근육 약화, 근육 변성, 경련과 비정상적인 근육통 및 근육 경직이 발생될 수 있다. GSD9D는 글리코겐 대사의 주요 조절 효소 인 근육 포스포릴라아제키나아제의 알파 소단위를 암호화하는 PHKA1 유전자의 돌연변이로 유발된다. 이 연구에서 우리는 한국인 GSD9D 가족에 대해 PHKA1 유전자에서 c.3314T> C (p.I1105T) 돌연변이를 동정하였다. 이 돌연변이는 이전에 어떠한 돌연변이 데이터베이스에서도 보고되지 않았으며 500명의 건강한 대조군에서도 발견되지 않았다. 이 돌연변이 영역은 다양한 다른 종 내에서 잘 보존되었으며 in silico 분석에서 돌연변이가 병원성일 가능성이 있다고 예측했다. 현재까지 PHKA1 유전자에는 보고된 병원성 돌연변이가 7개뿐이며 한국에서는 보고된 사례가 없다. 따라서 이 연구는 한국 GSD9D 환자의 첫번째 사례이다. 또한 이 연구는 이전에 보고된 환자와 한국 환자의 임상 증상과 병리 상태를 비교하고 설명하고자 하였다. 아울러 우리는 본 연구가 GSD9D의 분자 진단에 유용하게 활용될 것으로 기대한다.

• 대한원격탐사학회:학술대회논문집
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• 대한원격탐사학회 2005년도 Proceedings of ISRS 2005
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• pp.649-652
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• 2005

In this paper we present a detail approach of the determination of IFOV (Instantaneous Field of View) of high-resolution (l m) panchromatic satellite image over test site. IFOV is the representative measurements as the determination of the spatial resolution in remote sensed imaging system. It can be defined as some area on the ground with the particular altitude when the satellite acquires the image at any given time. Especially, spatial resolution of passive sensors primarily depends on their IFOV. The determination of IFOV goes through simple steps of procedure as followings: Firstly, the GSD (Ground Sample Distance) should be computed at each point on the geometrically corrected image. Then, The GSD is converted into the IFOV. So we are going to explain our test procedures and results.

• Tuberculosis and Respiratory Diseases
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• 제48권6호
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• pp.973-979
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• 2000

제 I 형 당원병에서 폐고혈압은 드문 합병증으로 외국 문헌에서 6예만이 보고되었으나 국내에는 아직 보고된 바가 없다. 저자들은 최근 제 Ia형 당원병으로 진단 받았던 31세 남자 환자에서 폐고혈압이 동반되었던 예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 한국해안해양공학회지
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• 제19권2호
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• pp.105-112
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• 2007

Lagrangian 개념에 근거하여 입도분포를 고려한 토사 확산범위 예측모형을 구성하여 입도분포를 고려한 경우와 고려하지 않은 경우의 확산범위 변화를 분실하였다. 토사의 입도분포를 Lagransian 관점에서 고려하기 위하여 입도분포곡선을 대수정규분포 함수로 가정하고, 토사입자에 해당하는 난수를 생성하였다. 토사입자는 구형으로 가정하였으며, 입도분포곡선에서 난수의 중량을 기준으로 발생된 난수를 토사입경에 해당하는 인자로 환산하였다. 부유토사 이동양상은 입자의 침강양상 모의를 통하여 검토하였으며, 침강속도는 van Rijn 공식을 이용하였고 침강에 영향을 미치는 인자로는 수평 확산계수만을 상수로 가정하여 포함하였다. 입도분포를 고려한 경우의 확산범위는 표사입도에 의한 영향을 고려하지 않은 경우의 확산범위와 유사하게 산정되었다. 그러나, 확산범위를 공급된 총입자중량의 90%, 99% 토사중량이 차지하는 영역을 기준으로 정의하는 경우, 90% 기준조건에서는 입도분포를 고려하지 않은 경우의 확산범위가 크게 산정되었으나, 99% 기준조건에서는 입도분포를 고려한 경우의 확산범위가 크게 산정되었다. 또한, 토사의 침강양상도 입도분포를 고려한 경우가 보다 현실적인 모습을 반영하고 있는 것으로 판단된다.

• 한국실내디자인학회논문집
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• 제18권6호
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• pp.124-132
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• 2009

Recently, 'Emotion' has attracted public attention socially and continuously in the fields such as construction, environment, design and products. Also, phenomenological concept of space through the physique in the viewpoint of the human-oriented aspect, namely, body, has been managed as a planned measure suitable for a new era and space. In this study, the author examined and analyzed the verbs and emotional vocabulary about the human body's behavior. The phenomenological space conception through body, i.e, flesh in this human centered point of view is being treated as the suitable planning measure on the new era and space. Though the epochal issue for Emotion is being attempted in many ways in various field such as product and marketing, the approach through the relationship between human and space. Therefore, we would like to see how the Emotion related to the movement that occurs in the space by analyzing the human body movement which is the subject of experiencing the space experimentally and research the characteristic of Emotion and space movement. SPSS 12.0 was used for the analysis to perform the analysis of basic statistics and Factor Analysis, and then to analyze the characteristic and emotional attribute for the experimental analysis. Comparing the activity for the 2 groups' behavior through the analyzed result with the test, active behavior was found to be higher than inactive behavior in the emotional value, and their bodies' motion showed averagely positive value in the emotional aspect; their bodies' motion was presented differently according to the attributes and amount of the people who would experience a specific space. However, the author found that affirmative and active behavior showed emotional aspect more than inactive behavior.

• 대한원격탐사학회:학술대회논문집
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• 대한원격탐사학회 1998년도 Proceedings of International Symposium on Remote Sensing
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• pp.301-306
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• 1998

Korea Aerospace Research Institute (KARI) is developing a Korea Multi-Purpose Satellite I (KOMPSAT-I) which accommodates Electro-Optical Camera (EOC), Ocean Scanning Multi-spectral Imager (OSMI), and Space Physics Sensor (SPS). The satellite has the weight of about 500kg and will be operated on the 10:50 AM sun-synchronized orbit with the altitude of 685 km. The satellite will be launched in 1999 and its lifetime is expected to be over 3 years. The main mission of EOC is the cartography to provide the images from a remote earth view for the production of 1/25000-scale maps of KOREA. EOC collects 510 ~ 730 nm panchromatic imagery with the ground sample distance(GSD) of 6.6 m and the swath width of 17 km by push broom scanning. EOC also can scan $\pm$45 degree across the ground track using body pointing method. The primary mission of OSMI is worldwide ocean color monitoring for the study of biological oceanography. It will generate 6 band ocean color images with 800 km swath width and 1km GSD by whiskbroom scanning. OSMI is designed to provide on-orbit spectral band selectability in the spectral range from 400 nm to 900 nm through ground command. This flexibility in band selection can be used for various applications and will provide research opportunities to support the next generation sensor design. SPS consists of High Energy Particle Detector (HEPD) and ionosphere Measurement Sensor (IMS). HEPD has missions to characterize the low altitude high-energy Particle environment and to study the effects of radiation environment on microelectronics. IMS measures densities and temperature of electrons in the ionosphere and monitors the ionospheric irregularities at the KOMPSAT orbit.

• Clinical and Experimental Pediatrics
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• 제51권6호
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• pp.650-654
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• 2008

당원병(GSD)과 점액다당류증(MPS)은 각각 독립된 유전 질환으로, 각기 다른 대사에 관여하는 효소의 결핍으로 인해 전구물질이 축적되어 임상 증상을 나타낸다. 간비대는 두 질환 모두에서 나타나는 중요한 임상 양상이다. 저자들은 이 드문 두 질환이 한 환자에서 동시에 발견되어 이에 보고하고자 한다. 16개월 남아가 2개월 동안 반복적인 중이염을 앓았고, 인근 병원을 다니던 중 우연히 간비대가 발견되었다. 환아는 이마가 튀어나온 조악한 얼굴 모양을 가지고 있었다. 환아의 간은 4횡지 정도로 촉지되었고, 그 성상은 부드럽고 무른 느낌이었으나 경계가 예리하게 만져졌다. 혈액 검사상, AST와 ALT가 85 IU/L, 77 IU/L로 약간 상승되어 있었다. 공복 저혈당은 보이지 않았고, 혈청 젖산, 요산, 콜레스테롤 모두 정상 소견을 보였다. 복부 초음파에서는 간의 메아리 발생 정도(echogenecity)가 증가되어 있었다. 간 생검을 실시하였고, 현미경 검사상 둥근 간세포들이 관찰되었는데, 그 간세포 내에는 Periodic Acid-Schiff 염색에서 진하게 염색되는 거대한 당원 입자들 다수와 이와 동반된 지방 공포(lipid vacuole)가 포함되어 있었다. 전자현미경에서 간세포의 핵과 세포질 모두에 당원 입자들이 포함되어 있었고 거대한 미토콘드리아도 관찰할 수 있었다. 생검을 통해 채취한 간 조직을 분석한 결과, 간의 당원 함량은 9.3%로 정상수치에 비해 증가되어 있었으나, 간의 glucose-6-phosphatase 활성도는 정상이어서 Ia형이 아닌 당원병으로 진단하게 되었다. 한편, X 선상 척추 앞면의 부리모양(beak-shape)와 J 모양의 안장(sella)과 같은 여러 골격의 이상 소견을 보였으며, 소변의 점액다당류 농도 또한 증가되어 있었다. Iduronate sulfatase 활성도가 0.1 nmol/hr/mg protein로 낮은 소견을 보여 헌터 증후군(제2형 점액다당류증, MPS type II)으로 진단할 수 있었다. 이에 저자들은 상기 환자가 당원병과 헌터증후군이 동시에 발견된 첫 환자로 생각되어 보고하는 바이다.

• 동의생리병리학회지
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• 제17권3호
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• pp.700-710
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• 2003

For the experiment of the effects between cadmium aerosol inhalation toxicity and ethyl acetate extracts of Folium Mori, 4 inhalation exposure groups of rat were exposed to cadmium aerosol in air by whole-body inhalation exposure for 6 hours/day, 5 days/week, and 4 weeks. Cadmium concentration in the air was 0.96㎎/㎥ and mass median diameter (MMD) was 2.48㎛ with 1.85 of geometric standard deviation(GSD). Intraperitoneal injections of ethyl acetate extracts of Folium Mori to inhalation exposure groups were performed for 4 weeks and the results were as follows: The highest body weight gain for 4 weeks and food intake per day were 159.29/4 weeks in treated group III and 18.45g/day in treated group I, respectively. The highest lung and liver weights were 1.31 g in treated group I and 9.42g in treated group III, respectively. The highest kidney weight was 2.21g from treated group I. The lowest cadmium content in lung was 86.39㎍/g from treated group III and the lowest cadmium concentration in blood was 2.72㎍/㎗ from treated group II. Cadmium concentrations of 22.09㎍/g in liver and 24.82㎍/g in kidney were the lowest from inhalation exposure group I and III, respectively. For weekly cadmium concentration in urine, the value of the fourth week from treated group III was the highest, 1.35㎍/㎖. For weekly cadmium concentration in feces, the values of the second and fourth week from treated group I were the highest, 1.11㎍/g. The highest metallothionein concentration in lung was 31.85㎍/g from treated group III and the highest metallothionein concentration in liver was 205.77㎍/g from treated group III. The highest metallothionein concentration in kidney was 206.55㎍/g from treated group III. The highest Hct and Hb values were 38.26% and 11.63g/㎗ from treated group III, respectively. The highest RBC and WBC values were 7.68×106/㎣ and 9.85×10³/㎣ from treated group I, respectively.