• Molecules and Cells
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• v.28 no.3
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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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• v.17 no.4
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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.

• Journal of Life Science
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• v.30 no.8
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• pp.672-679
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• 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 KSRS Conference
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• 2005.10a
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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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• v.48 no.6
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• pp.973-979
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• 2000

A glycogen storage disease(GSD) type I is a metabolic disease caused by a deficiency in one of the components of the glucose-6-phosphatase(G-6-Pase) system. This disorder results in hypoglycemia, hepatomegaly, lactic acidemia, hyperlipidemia, and hyperuricemia. Comon long(-)term complications include growth retaradation, gout, hepatic adenomas, osteoporosis and renal disease. However the cardiovascular system is rarely involved, and only six cases of pulmonary hypertension associated with GSD I have been reported in the literature. We experienced a case of pulmonary hypertension with type I GSD. A 31-year-old rnan, who had discovered type I GSD and received portocaval shunt operation 22 years ago, was admitted to the hospital with the chief complaint of dyspnea. Echocardiographic examination and cardiac catheterization revealed severe pulmonary hypertension. Nitric oxide and oral prostacycline derivative(beraprost) were tried without acute favorable response. After one year with beraprost, dyspnea, exercise capacity and hemodynamic parameters were improved. We report this case with a review of the literature.

• Journal of Korean Society of Coastal and Ocean Engineers
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• v.19 no.2
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• pp.105-112
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• 2007

Sediment transport model based on the Lagrangian concept considering the grain size distribution(GSD) was setup and the change of the sediment diffusion range was analysed in the condition of considering and not considering the GSD. The GSD curve is assumed as the Log-normal distribution function in order to consider the GSD with respect to the Lagrangian concept and the random numbers, i.e. sediment particles, are generated based on the distribution function. The sediment particles is assumed as the spherical type and the random numbers based on the sediment weight is converted to the sediment diameters. Sediment transport patterns are analysed by the settling simulation, in which the settling velocity is computed by the van Rijn formulae and the horizontal diffusion coefficient is used as the constant parameter. The diffusion patterns are very similar to the patterns with GSD condition. The diffusion range defined as the range including 90%, 99% sediment weight of the total sediment weight, however, is larger than without considering GSD condition in 90%-option and shorter than with considering GSD condition in 99-option, respectively. The diffusion range is defined as tile p-percentage of the cumulative sediment weight region with reference to the 50% region, 90%- option, 99%-option, respectively.

• Korean Institute of Interior Design Journal
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• v.18 no.6
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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.

• Proceedings of the KSRS Conference
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• 1998.09a
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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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• v.51 no.6
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• pp.650-654
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• 2008

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

• Journal of Physiology & Pathology in Korean Medicine
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• v.17 no.3
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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.