• Journal of Genetic Medicine
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• v.11 no.1
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• pp.22-26
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• 2014

Maple syrup urine disease (MSUD) is a disorder that involves the metabolism of branched chain amino acids, arising from a defect in branched-chain ${\alpha}$-keto acid dehydrogenase complex. Mutations have been identified in the BCKDHA, BCKDHB, or DBT genes, which encode different subunits of the BCKDH complex. Although encephalopathy and progressive neurodegeneration are its major manifestations, the severity of the disease may range from the severe classic type to milder intermediate variants. We report two Korean siblings with the milder intermediate MSUD who were diagnosed with MSUD by a combination of newborn screening tests using tandem mass spectrometry and family genetic screening for MSUD. At diagnosis, the patients' plasma levels were elevated for leucine, isoleucine, valine, and alloisoleucine, and branched-chain ${\alpha}$-keto acids and branched-chain ${\alpha}$-hydroxy acids were detected in their urine. BCKDHA, BCKDHB, and DBT analysis was performed, and two novel mutations were identified in BCKDHB. Our patients were thought to have the milder intermediate variant of MSUD, rather than the classic form. Although MSUD is a typical metabolic disease with poor prognosis, better outcomes can be expected if early diagnosis and prompt management are provided, particularly for milder forms of the disease.

• Journal of the Korean Society of Hazard Mitigation
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• v.11 no.3
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• pp.17-28
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• 2011

This study applied the Kalman Filter for real-time forecasting the G/R (ground rain gauge rainfall/radar rainfall) ratio to correct the mean field bias of the very-short-range-forecast (VSRF) rainfall. The MAPLE-forecasted rainfall was used as the VSRF rainfall, also the methodology for deciding the G/R ratio was improved by evaluating the change of G/R ratio characteristics depending on the threshold and accumulation time. This analysis was done for the inland, mountain, and coastal regions, separately, for their comparison. As the results, more stable G/R ratio could be estimated by applying the threshold and accumulation time, whose forecasting accuracy could also be secured. The accuracy of the corrected rainfall forecasting by the forecasted G/R ratio was the best in the inland region but the worst in the coastal region.

• Proceedings of the Plant Resources Society of Korea Conference
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• 2019.04a
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• pp.55-55
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• 2019

Strawberry which is the genus Fragaria under family Rosaceae is one of the most important fruit plants for both fresh consumption and food processing in the temperate and subtropical countries. Propagation of strawberry is achieved either through runners or by in vitro micropropagation. Meristem tips, generally obtained from runners of virus-free plants, are commonly used to establish in vitro cultures, which are employed for mass propagation or as a source of plant material for regeneration and transformation experiments. This study was conducted to determine the optimal natural additives strength to improve sprouting shoot rate of apical meristem of strawberry 'Seolhyang'. Strawberry apical meristem at size (0.2 mm to 0.3 mm) with leaf primordials were cultured on the 1/3MS(Murashige & Skoog) medium supplemented with five natural additives such as coconut milk, maple sap, banana powder and peptone. The sprouting ratio and growth characteristics were evaluated after eight weeks after in vitro culture. Shoot ratio of 'Seolhyang' apical meristem was 72.9% in 1/3MS medium supplemented with maple sap. On the other hand, the low shoot ratio was observed 47.7% in 1/3MS medium supplemented with banana powder. Shoot length was different as natural additives but numbers of leaf was not significantiy different among the natural additives. As a result, the sprouting ratio and plant growth were enhanced effectively in 1/3MS medium with maple sap compared to the others.

• Proceedings of the Korea Water Resources Association Conference
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• 2021.06a
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• pp.371-371
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• 2021

돌발홍수 예경보시스템의 입력자료로 예측강우가 활용된다. 기상청과 환경부에서는 초단기 예보의 목적으로 MAPLE(McGill Algorithm for Precipitation nowcasting and Lagrangian Extrapolation)을 생산하고 있다. MAPLE은 선행 30분까지의 예측품질은 어느 정도 정확하다고 볼 수 있으나 그 이후 특히 3시간 이상이 되면 예측품질이 크게 떨어지는 문제가 있다. 예측강우의 편의보정을 위한 여러 시도들이 있었으나 호우의 규모 및 이동특성을 고려한 사례는 제한적이다. 호우의 이동특성을 고려해야하는 이유로는 첫째, 예측의 특성상 예측강우가 생성되고 편의보정이 이루어지는 시간 동안 호우는 이동을 하기 때문이다. 둘째, 호우가 이동을 하면서 편의보정의 대상이 되는 지역에 적합한 보정계수의 결정이 어렵기 때문이다. 마지막으로 돌발홍수는 장마와 같은 전선형 강수가 아닌 국지성 호우와 같이 빠르게 움직이며 강한 호우를 내리는 강수에 의해 발생하기 때문이다. 본 연구에서는 이러한 문제점을 극복하기 위해 호우의 이동특성을 고려하여 예측강우 보정계수를 결정하고 이를 예측강우에 실시간으로 적용할 수 있는 방법을 제시하였다. 이 과정에서 Backward tracking은 미래에 호우가 도달할 지역(대상지역)으로부터 현재 호우가 위치하는 지역을 추적하는데 이용된다. 추적된 지역에서 보정계수가 결정된다. Forward tracking은 현재 호우가 위치하는 지역으로부터 대상지역을 다시 추적하는데 이용된다. 앞서 결정된 보정계수는 대상지역의 예측강우에 적용된다. 해당 방법론을 2019년에 발생한 주요 호우사상에 실시간 적용하고 평가하였다. 그 결과, Backward-Forward tracking 기반 예측강우 보정방법을 적용한 경우에는 실제 관측된 강우와 매우 유사한 보정결과가 도출됨을 확인되었다.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.31-38
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• 2023

Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder caused by a deficiency in branched chain α-keto acid dehydrogenase (BCKAD). Between 1997, when Korea's MSUD case was first reported, and 2023, 14 cases were reported in the literature. 29% of the cases experienced developmental delay, and 29% expired. The prevalence of MSUD in Korea was estimated to be 1 in 230,000. Of 21 MSUD patients currently being treated at the Korea Genetics Research Center, 19 were detected through newborn screening program, and 2 were diagnosed by the symptoms. 14 MSUD patients had confirmed genetic mutations; 6 (43%) were BCKDHA and 8 (57%) were BCKDHB. In one case, a large deletion was observed. 4 patients had leucine levels above 2,000 (umo/L), and post-dialysis diet therapy was initiated in the newborn period. No patient required further dialysis as diet therapy and regular monitoring proved highly effective. Most MSUD patients were growing normally; weight and height growth were above the 50th percentile in 76% of the cases while BMI values were higher than normal in 71% of cases. Developmental delays were observed only in 2 cases (10%) and anticonvulsant use in 3 cases (14%). With newborn screening available to all Korean infants, early diagnosis and intervention should allow most patients to remain asymptomatic. However, ongoing surveillance, dietary management and continued patient compliance as well as rapid correction of acute metabolic decompensations remain critical to a favorable long-term prognosis.

• Journal of Korean Society of Forest Science
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• v.96 no.5
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• pp.551-560
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• 2007

We investigated physiological damages and biochemical alleviation of five species of genus Acer under ozone fumigation in order to assess their tolerant ability against ozone toxicity. At the end of 150 ppb $O_3$ fumigation, photosynthetic characteristics were measured, and chlorophyll contents, malondialdehyde (MDA) and antioxidative enzyme activities were analyzed in the leaves of five maple trees (Acer buergerianum, A. ginnala, A. mono, A. palmatum, and A. palmatum var. sanguineum). The reduction of chlorophyll (chl) a in ozone-exposed plants was 16.8% (A. buergerianum) to 26.7% (A. ginnala) of control plants. For the content of chi b, A. ginnala and A. palmatum var. sanguineum represented the high reduction of 26.3% and 23.6%, respectively. The highest reduction on the chi a:b ratio was observed in the leaves of A. palmatum. The reduction of net photosynthesis in five species varied from 2.4% to 37.6%. Among five species, A. ginnala showed remarkable reduction (37.6%) for net photosynthesis in comparison with control. Carboxylation efficiency differed significantly (P < 0.05) among species and between control and ozone treatment. The reduction of carboxylation efficiency was the highest in the leaves of A. ginnala (44.7%). A. palmatum var. sanguineum showed the highest increase (41.7%) for MDA content. The highest increase of superoxide dismutase (SOD) activity represented in A. palmatum (26.1%) and the increase of ascorbate peroxidase (APX) activity ranged from 16.5% (A. ginnala) to 49.1% (A. palmatum var. sanguineum). A. mono showed the highest increase (376.6%) of glutathione reductase (GR) activity under ozone fumigation and A. buergerianum also represented high increase (42.3%) of GR activity. Catalse (CAT) activity increased in the leaves of A. ginnala, A. palmatun and A. palmatum var. sanguineum under ozone exposure, whereas A. buergerianum and A. mono decreased in comparison with control plants. In conclusion, physiological markers such as chlorophyll content and photosynthesis that responded sensitively to $O_3$ in maple trees were considered as the very important indicators in order to evaluate the tolerance against $O_3$ stress, and parameters were closely related with each other. Among anti oxidative enzymes, SOD and APX might be contributed to alleviate to $O_3$ toxicity through the increase of activity in all maple trees. Therefore, these compounds can be used as a biochemical maker to assess the stress tolerance to $O_3$.

• Korean Journal of Heritage: History & Science
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• v.47 no.3
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• pp.4-23
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• 2014

Seokjojeon Hall is the Neoclassic style building situated in Deoksugung Palace, which was proposed by John McLeavy Brown who was a chief commissioner of the Daehan Empire and designed by John Reginald Hardings in 1897. Construction of the Seokjojeon Hall began in 1900 and completed in 1910 at the total cost of one million won. Decorating and furnishing of the interior was designed by Lovell and all the furniture of Seokjojeon Hall had been purchased from Maple&Co. The Maple&Co was the supplier of luxury furnitures and decorating items for luxurious residentials, hotels, embassies and the palaces and its headquarter was located in London. Ready-made furnitures were purchased as shown in the Maple's catalog. The designs and styles of the west were applied to Seokjojeon Hall. That is one of the aspects showing Daehan Imperial underwent a period of Westernization.

• Journal of Korean Society of Forest Science
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• v.112 no.1
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• pp.32-39
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• 2023

Using the logarithmic methods and the generalized method of moments (GMM), this study developed carbon storage equations for maple trees (Acer palmatum Thunb.) planted in an urban settlement area. A total of 20 maple trees of various ages and diameters were destructively harvested to determine their dry weight and carbon concentration by component. The allometric equations with DBH and DBH²×H as independent variables were developed to estimate the carbon storage for each tree component. The carbon concentration of tree components was the highest in stem wood (49.8%) and lowest in stem bark (46.5%). Allometric equations to estimate the carbon storage of tree components (stem, root, aboveground, and total) showed a similar coefficient of determinations (R²) between the allometric equations of the logarithmic method (0.7494-0.9036) and the GMM (0.7085-0.8847). However, the R² values of the leaves and branches were in the range of 0.3027 to 0.6380, lower than those of the R² of the other tree components. These results indicate that the carbon storage of maple trees growing in urban settlement areas can be efficiently predicted from the equations of GMM methods in the case of a small sample size or the heteroscedasticity of logarithmic equations.

• Honam Mathematical Journal
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• v.28 no.4
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• pp.521-531
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• 2006

In this paper, we construct a procedure of Maple programming for (0, 1)-matrix with a prescribed permanent, $1,2,...,2^{n-1}$. An application of such construction is given, and we obtain the some results of (0, 1)-matrices with the permanent less than or equal to n! by replacing elements 0's by 1's.

• Proceedings of the Korean Statistical Society Conference
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• 2002.11a
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• pp.235-237
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• 2002

통계학 과목 교수 시 보조 수단으로 자주 사용하는 것이 통계패키지이다 이러한 통계패키지 외에 수학패키지를 통계학 과목 수업에 활용하면 학습효과를 높이는 데 효과적일 것이다.