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A study on the development of a system for collecting and displaying disaster site information for disaster situation management : focusing on earthquakes (재난상황관리를 위한 재난현장정보 수집 및 표출시스템 개발 연구 : 지진을 중심으로)

  • Koo, Jee Hee;Song, Juil;Cho, Jung Yun
    • Journal of the Korean Society of Surveying, Geodesy, Photogrammetry and Cartography
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    • v.40 no.1
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    • pp.31-40
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    • 2022
  • The importance of disaster management and response is emerging as various disasters such as COVID-19, torrential rains, and fires occur one after another. In addition, in order to respond efficiently throughout disaster response activities, it is necessary to quickly collect disaster site information and quickly check the site situation through photo and video information so that rapid disaster response can be achieved. In this study, essential information required for decision-making was derived by analyzing the essential activities of each disaster response stage, analyzing the crisis management standard manual and related laws for each disaster type, and daily comprehensive report. In addition, a list of information necessary to grasp the situation of the disaster site and grasp the status of real-time damage was derived to establish guidelines for collecting volatile disaster site information, and disaster situation information can be efficiently displayed through a spatial information-based display system. By presenting essential disaster management information to be collected first, the person in charge of collecting information can efficiently collect information, and the situation room in charge of disaster response decision-making is expected to enable more efficient disaster situation management by receiving only the necessary information.

Protective Effect of Betula Platyphylla on Ultraviolet B-irradiated HaCaT Keratinocytes (화피(樺皮) 에탄올 추출물의 Ultraviolet B로 자극한 피부 각질 세포 보호 작용)

  • Hag Soon Choi;Hyun Joo Kim;Hark Song Lee;Seung Won Paik;Ji Eun Kim;Yung Sun Song
    • The Journal of Korean Medicine
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    • v.44 no.2
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    • pp.119-131
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    • 2023
  • Objectives: Betula Platyphylla(BP) has been used as a analgesic, anti-microbial, anti-oxidant drug in Eastern Asia. However, it is still unknown whether BP ethanol extract could exhibit the inhibitory activities against ultraviolet B(UVB)-induced skin injury on human keratinocytes, HaCaT cells. This study was aimed to investigate the protective activity of BP ethanol extract on UVB-irradiated skin injury in HaCaT cells. Methods: The skin injury model of HaCaT cells was established under UVB stimulation. HaCaT keratinocyte cells were pre-treated with BP ethanol extract for 1 h, and then stimulated with UVB. Then, the cells were harvested to measure the cell viability, production of reactive oxygen species(ROS), pro-inflammatory cytokines such as interleukin(IL) 1-beta, IL-6, and tumor necrosis factor(TNF)-𝛼, hyaluronidase, type 1 collagen, matrix metalloproteinase(MMP)s. In addition, we examined the mitogen activated protein kinases(MAPKs) and inhibitory kappa B alpha(I𝜅;-B𝛼) as inhibitory mechanisms of BP ethanol extract. Results: The treatment of BP ethanol extract inhibited the UVBinduced cell death and ROS production in HaCaT cells. BP ethanol extract treatment inhibited the UVB-induced increase of IL-1beta, IL-6, and TNF-𝛼. BP ethanol extract treatment inhibited the increase of hyaluronidase, MMP and decrease of collagen. BP ethanol extract treatment inhibited the activation of MAPKs and the degradation of I𝜅-B𝛼. Conclusions: Our result suggest that treatment of BP ethanol extract could inhibit the UVB-induced skin injury via deactivation of MAPKs and nuclear factor kappa B(NF-𝜅B) in HaCaT cells. This study could suggest that BP ethanol extract could be a beneficial agent to prevent skin damage or inflammation.

Nucleomodulin BspJ as an effector promotes the colonization of Brucella abortus in the host

  • Ma, Zhongchen;Yu, Shuifa;Cheng, Kejian;Miao, Yuhe;Xu, Yimei;Hu, Ruirui;Zheng, Wei;Yi, Jihai;Zhang, Huan;Li, Ruirui;Li, Zhiqiang;Wang, Yong;Chen, Chuangfu
    • Journal of Veterinary Science
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    • v.23 no.1
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    • pp.8.1-8.15
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    • 2022
  • Background: Brucella infection induces brucellosis, a zoonotic disease. The intracellular circulation process and virulence of Brucella mainly depend on its type IV secretion system (T4SS) expressing secretory effectors. Secreted protein BspJ is a nucleomodulin of Brucella that invades the host cell nucleus. BspJ mediates host energy synthesis and apoptosis through interaction with proteins. However, the mechanism of BspJ as it affects the intracellular survival of Brucella remains to be clarified. Objectives: To verify the functions of nucleomodulin BspJ in Brucella's intracellular infection cycles. Methods: Constructed Brucella abortus BspJ gene deletion strain (B. abortus ∆BspJ) and complement strain (B. abortus pBspJ) and studied their roles in the proliferation of Brucella both in vivo and in vitro. Results: BspJ gene deletion reduced the survival and intracellular proliferation of Brucella at the replicating Brucella-containing vacuoles (rBCV) stage. Compared with the parent strain, the colonization ability of the bacteria in mice was significantly reduced, causing less inflammatory infiltration and pathological damage. We also found that the knockout of BspJ altered the secretion of cytokines (interleukin [IL]-6, IL-1β, IL-10, tumor necrosis factor-α, interferon-γ) in host cells and in mice to affect the intracellular survival of Brucella. Conclusions: BspJ is extremely important for the circulatory proliferation of Brucella in the host, and it may be involved in a previously unknown mechanism of Brucella's intracellular survival.

Seismic Capacity Evaluation of Existing R/C Buildings Retrofitted by Internal Composite Seismic Strengthening Method Based on Pseudo-dynamic Testing (유사동적실험기반 내부접합형 합성내진보강공법을 적용한 기존 R/C 건물의 내진성능평가 )

  • Eun-Kyung Lee;Jin-Young Kim;Ho-Jin Baek;Kang-Seok Lee
    • Journal of the Korea institute for structural maintenance and inspection
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    • v.27 no.2
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    • pp.67-76
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    • 2023
  • In this study, in order to enhance the joint capacity between the existing reinforced concrete (R/C) frame and the reinforcement member, we proposed a novel concept of Internal Composite Seismic Strengthening Method (CSSM) for seismic retrofit of existing domestic medium-to-low-rise R/C buildings. The Internal CSSM rehabilitation system is a type of strength-enhancing reinforcement systems, to easily increase the ultimate horizontal shear capacity of R/C structures without seismic details in Korea, which show shear collapse mechanism. Two test specimens of full-size two-story R/C frame were fabricated based on an existing domestic R/C building without seismic details, and then retrofitted by using the proposed CSSM seismic system; therefore, one control test specimen and one test specimen reinforced with the CSSM system were used. Pseudo-dynamic testing was carried out to evaluate seismic strengthening effects, and the seismic response characteristics of the proposed system, in terms of the maximum shear force, response story drift, and seismic damage degree compared with the control specimen (R/C bare frame). Experiment results indicated that the proposed CSSM reinforcement system, internally installed to the existing R/C frame, effectively enhanced the horizontal shear force, resulting in reduced story drift of R/C buildings even under a massive earthquake.

Development of Truck Axle Load Distribution Model using WIM Data (WIM 자료를 활용한 화물차 축하중 분포 모형 개발)

  • Lee, Dong Seok;Oh, Ju Sam
    • KSCE Journal of Civil and Environmental Engineering Research
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    • v.26 no.5D
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    • pp.821-829
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    • 2006
  • Traffic load comprise primary input to pavement design causing pavement damage. therefore it should be proceeded suitable traffic load distribution modeling for pavement design and analysis. Traffic load have been represented by equivalent single axle loads (ESALs) which convert mixed traffic stream into one value for design purposes. But there are some limit to apply ESALs to other roads because it is empirical value developed as part of the original AASHO(American Association of State Highway Officials) road test. There have been many efforts to solve these problems. Several leading country have implemented M-E(Mechanistic-Empirical) design procedures based on mechanical concept. As a result, they established traffic load quantification method using load distribution model known as Axle Load Spectra. This paper details Axle Load Spectra and presents axle load distribution model based on normal mixture distribution function using truck load data collected by WIM system installed in national highway. Axle load spectra and axle load distribution model presented in this paper could be useful for basic data when making traffic load quantification plan for pavement design, overweight vehicle permit plan and pavement maintenance cost plan.

An Experimental Study for Characteristics Evaluation of Cement Mortar Using Infrared Thermography Technique (적외선 화상기법을 이용한 시멘트 모르타르 특성의 실험적 평가)

  • Kwon, Seung-Jun;Maria, Q. Feng
    • KSCE Journal of Civil and Environmental Engineering Research
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    • v.30 no.1A
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    • pp.53-59
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    • 2010
  • Recently, NDTs (Non-Destructive Techniques) using infrared camera are widely studied for detection of damage and void in RC (reinforced concrete) structures and they are also considered as an effective techniques for maintenance of infrastructures. The temperature on concrete surface depends on material and thermal properties such as specific heat, thermal conductivity, and thermal diffusion coefficient. Different porosity on cement mortar due to different mixture proportions can show different heat behavior in cooling stage. The porosity can affect physical and durability properties like strength and chloride diffusion coefficient as well. In this paper, active thermography which uses flash for heat induction is utilized and thermal characteristics on surface are evaluated. Samples of cement mortar with W/C (water to cement ratio) of 0.55 and 0.65 are prepared and physical properties like porosity, compressive strength, and chloride diffusion coefficient are evaluated. Then infrared thermography technique is carried out in a constant room condition (temperature $20{\sim}22^{\circ}C$ and relative humidity 55-60%). The mortar samples with higher porosity shows higher residual temperature at the cooling stage and also shows reduced critical time which shows constant temperature due to back wall effect. Furthermore, simple equation for critical time of back wall effect is suggested with porosity and experimental constants. These characteristics indicate the applicability of infrared thermography as an NDT for quality assessment of cement based composite like concrete. Physical properties and thermal behavior in cement mortar with different porosity are analyzed in discussed in this paper.

Ecological Changes of Insect-damaged Pinus densiflora Stands in the Southern Temperate Forest Zone of Korea (I) (솔잎혹파리 피해적송림(被害赤松林)의 생태학적(生態学的) 연구(研究) (I))

  • Yim, Kyong Bin;Lee, Kyong Jae;Kim, Yong Shik
    • Journal of Korean Society of Forest Science
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    • v.52 no.1
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    • pp.58-71
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    • 1981
  • Thecodiplosis japonesis is sweeping the Pinus densiflora forests from south-west to north-east direction, destroying almost all the aged large trees as well as even the young ones. The front line of infestation is moving slowly but ceaselessly norhwards as a long bottle front. Estimation is that more than 40 percent of the area of P. densiflora forest has been damaged already, however some individuals could escapes from the damage and contribute to restore the site to the previous vegetation composition. When the stands were attacked by this insect, the drastic openings of the upper story of tree canopy formed by exclusively P. densiflora are usually resulted and some environmental factors such as light, temperature, litter accumulation, soil moisture and offers were naturally modified. With these changes after insect invasion, as the time passes, phytosociologic changes of the vegetation are gradually proceeding. If we select the forest according to four categories concerning the history of the insect outbreak, namely, non-attacked (healthy forest), recently damaged (the outbreak occured about 1-2 years ago), severely damaged (occured 5-6 years ago), damage prolonged (occured 10 years ago) and restored (occured about 20 years ago), any directional changes of vegetation composition could be traced these in line with four progressive stages. To elucidate these changes, three survey districts; (1) "Gongju" where the damage was severe and it was outbroken in 1977, (2) "Buyeo" where damage prolonged and (3) "Gochang" as restored, were set, (See Tab. 1). All these were located in the south temperate forest zone which was delimited mainly due to the temporature factor and generally accepted without any opposition at present. In view of temperature, the amount and distribution of precipitation and various soil factor, the overall homogeneity of environmental conditions between survey districts might be accepted. However this did not mean that small changes of edaphic and topographic conditions and microclimates can induce any alteration of vegetation patterns. Again four survey plots were set in each district and inter plot distance was 3 to 4 km. And again four subplots were set within a survey plot. The size of a subplot was $10m{\times}10m$ for woody vegetation and $5m{\times}5m$ for ground cover vegetation which was less than 2 m high. The nested quadrat method was adopted. In sampling survey plots, the followings were taken into account: (1) Natural growth having more than 80 percent of crown density of upper canopy and more than 5 hectares of area. (2) Was not affected by both natural and artificial disturbances such as fire and thinning operation for the past three decades. (3) Lower than 500 m of altitude (4) Less than 20 degrees of slope, and (5) Northerly sited aspect. An intensive vegetation survey was undertaken during the summer of 1980. The vegetation was devided into 3 categories for sampling; the upper layer (dominated mainly by the pine trees), the middle layer composed by oak species and other broad-leaved trees as well as the pine, and the ground layer or the lower layer (shrubby form of woody plants). In this study our survey was concentrated on woody species only. For the vegetation analysis, calculated were values of intensity, frequency, covers, relative importance, species diversity, dominance and similarity and dissimilasity index when importance values were calculated, different relative weights as score were arbitrarily given to each layer, i.e., 3 points for the upper layer, 2 for the middle layer and 1 for the ground layer. Then the formula becomes as follows; $$R.I.V.=\frac{3(IV\;upper\;L.)+2(IV.\;middle\;L.)+1(IV.\;ground\;L.)}{6}$$ The values of Similarity Index were calculated on the basis of the Relative Importance Value of trees (sum of relative density, frequency and cover). The formula used is; $$S.I.=\frac{2C}{S_1+S_2}{\times}100=\frac{2C}{100+100}{\times}100=C(%)$$ Where: C = The sum of the lower of the two quantitative values for species shared by the two communities. $S_1$ = The sum of all values for the first community. $S_2$ = The sum of all values for the second community. In Tab. 3, the species composition of each plot by layer and by district is presented. Without exception, the species formed the upper layer of stands was Pinus densiflora. As seen from the table, the relative cover (%), density (number of tree per $500m^2$), the range of height and diameter at brest height and cone bearing tendency were given. For the middle layer, Quercus spp. (Q. aliena, serrata, mongolica, accutissina and variabilis) and Pinus densiflora were dominating ones. Genus Rhodedendron and Lespedeza were abundant in ground vegetation, but some oaks were involved also. (1) Gongju district The total of woody species appeared in this district was 26 and relative importance value of Pinus densiflora for the upper layer was 79.1%, but in the middle layer, the R.I.V. for Quercus acctissima, Pinus densiflora, and Quercus aliena, were 22.8%, 18.7% and 10.0%, respectively, and in ground vegetation Q. mongolica 17.0%, Q. serrata 16.8% Corylus heterophylla 11.8%, and Q. dentata 11.3% in order. (2) Buyeo district. The number of species enumerated in this district was 36 and the R.I.V. of Pinus densiflora for the uppper layer was 100%. In the middle layer, the R.I.V. of Q. variabilis and Q. serrata were 8.6% and 8.5% respectively. In the ground vegetative 24 species were counted which had no more than 5% of R.I.V. The mean R.I.V. of P.densiflora ( totaling three layers ) and averaging four plots was 57.7% in contrast to 46.9% for Gongju district. (3) Gochang-district The total number of woody species was 23 and the mean R.I.V. of Pinus densiflora was 66.0% showing greater value than those for two former districts. The next high value was 6.5% for Q. serrata. As the time passes since insect outbreak, the mean R.I.V. of P. densiflora increased as the following order, 46.9%, 57.7% and 66%. This implies that P. densiflora was getting back to its original dominat state again. The pooled importance of Genus Quercus was decreasing with the increase of that for Pinus densiflora. This trend was contradict to the facts which were surveyed at Kyonggi-do area (the central temperate forest zone) reported previously (Yim et al, 1980). Among Genus Quercus, Quercus acutissina, warm-loving species, was more abundant in the southern temperature zone to which the present research is concerned than the central temperate zone. But vice-versa was true with Q. mongolica, a cold-loving one. The species which are not common between the present survey and the previous report are Corpinus cordata, Beltala davurica, Wisturia floribunda, Weigela subsessilis, Gleditsia japonica var. koraiensis, Acer pseudosieboldianum, Euonymus japonica var. macrophylla, Ribes mandshuricum, Pyrus calleryana var. faruiei, Tilia amurensis and Pyrus pyrifolia. In Figure 4 and Table 5, Maximum species diversity (maximum H'), Species diversity (H') and Eveness (J') were presented. The Similarity indices between districts were shown in Tab. 5. Seeing Fig. 6, showing two-dimensional ordination of polts on the basis of X and Y coordinates, Ai plots aggregate at the left site, Bi plots at lower site, and Ci plots at upper-right site. The increasing and decreasing patterns as to Relative Density and Relative Importance Value by genus or species were given in Fig. 7. Some of the patterns presented here are not consistent with the previously reported ones (Yim, et al, 1980). The present authors would like to attribute this fact that two distinct types of the insect attack, one is the short war type occuring in the south temperate forest zone, which means that insect attack went for a few years only, the other one is a long-drawn was type observed at the temperate forest zone in which the insect damage went on continuously for several years. These different behaviours of infestation might have resulted the different ways of vegetational change. Analysing the similarity indices between districts, the very convincing results come out that the value of dissimilarity index between A and B was 30%, 27% between B and C and 35% between A and C (Table 6). The range of similarity index was obtained from the calculation of every possible combinations of plots between two districts. Longer time isolation between communities has brought the higher value of dissimilarity index. The main components of ground vegetation, 10 to 20 years after insect outbreak, become to be consisted of mainly Genus Lespedeza and Rhododendron. Genus Quercus which relate to the top dorminant state for a while after insect attack was giving its place to Pinus densiflora. It was implied that, provided that the soil fertility, soil moisture and soil depth were good enough, Genus Quercuss had never been so easily taken ever by the resistant speeies like Pinus densiflora which forms the edaphic climax at vast areas of forest land. Usually they refer Quercus to the representative component of the undisturbed natural forest in the central part of this country.

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Effect of Seed Mixture on Forage Yields and Botanical Composition at an Altitude of 400 m in Jeju island (표고 400m 제주 중산간 지역에서 혼합조합별 초지 생산성 및 식생구성)

  • Chae, Hyun Seok;Kim, Nam Young;Woo, Jae Hoon;Shin, Moon Cheol;Son, Jun Kyu;Seong, Pil Nam;Lee, Wang Shik;Kim, Si Hyun;Hwang, Kyung Jun;Kim, Young Jin;Park, Nam Gun
    • Journal of The Korean Society of Grassland and Forage Science
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    • v.37 no.1
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    • pp.19-27
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    • 2017
  • The objective of this study was to determine the growth characteristics of cool (C1) and warm season grasses (C2) in pastures mixed with C1 and C2 at an altitude of 400 m in Jeju island to establishing pasture suitable for grazing horses and to evaluate the effect of pastures mixed with tall and short type grasses on the intake characteristics of horses. C1 used in this study was Kentucky bluegrass, redtop (short type grass) and tall type grasses were orchardgrass and tall fescue, respectively. Treatments of this study were consisted of four groups and the short type grass used in pastures mixed with C1 and C2 was mainly bermudagrass. Four treatment groups were follow as; Treatment 1 (bermudagrass + Kentucky bluegrass + redtop) 2) Treatment 2 (bermudagrass + tall fescue + orchardgrass) 3) Treatment 3 (Kentucky bluegrass + redtop) 4) Treatment 4 (tall fescue + orchardgrass). Bermudagrass was a little winter killing and inhibition of plant growth at an altitude of 400 m. Plant heights in pastures mixed with C1 and C2 were grown better than that in pastures mixed with C1. Especially, plant height in Treatment 4 was higher than other treatments. Dry matter yield was in the following order: Treatment 4> Treatment 3> Treatment 2> Treatment 1. Dry matter yield in pastures mixed with C1 increased as compared with pastures mixed with C1 and C2. Dry matter yield in Treatment 3 was higher than other treatments. In the first investigation regarding vegetation distribution, bermudagrass ratios among grasses in Treatment 1 and Treatment 2 were 11.7 and 13.3%, respectively. The growth of bermudagrass in winter was low due to the cold damage. However the growth of Kentucky bluegrass, redtop, tall fescue and orchardgrass was good. In the second investigation, bermudagrass ratios among grasses in Treatment 1 and Treatment 2 were 5.0 and 11.7%, respectively. Growth of forage in the second investigation was poor as compared to the first investigation. nutritive values(crude protein content, neutral detergent fiber content, acid detergent fiber content, digestibility) were good in pastures mixed with C1 Especially, nutritive values in pastures mixed with tall was higher than those of pastures mixed short grasses. P content among minerals in Treatment 1 was higher than other groups. However, the content of Ca, Mg and Mn were lower. The contents of Ca, K, Mg, Na, Cu, Zn and Fe in Treatment 2 were higher. However, the contents of K, Mg, Na, Cu, Zn and Fe in Treatment 3 were lower. Therefore, we suggest that cool season grasses with short grasses were sowed to establishing pasture suitable for grazing horses at an altitude of 400 m in Jeju island.

Studies on the Low Temperature Injury of the Rice Varieties (통일계(統一系) 수도품종(水稻品種)의 저온장해(低溫障害)에 관(關)한 연구(硏究))

  • Choi, Chang-Yoel;Kim, Moon-Kyu;Jo, Jai-Seong;Kim, Choong-Soo
    • Korean Journal of Agricultural Science
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    • v.4 no.1
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    • pp.10-20
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    • 1977
  • An expriment was conducted to determine the rate of germination under low temperature and effects of low temperature on seedling of varieties derived from the cross between Indica and Japonica type of rice. Total of 30 varieties including leading variety, Tongil, were germinated at the temperature of $10^{\circ}C$. and $15^{\circ}C$. to determine the rate of germination, and six varieties also including Tongil were planted in pot, and seedlings were exposed to the temperature of $5^{\circ}C$. and $1^{\circ}C$. for 15 days and damage of plant growth due to low temperature was observed. 1. Under the condition of $10^{\circ}C$., varieties such as Iri #323, Suwon #253, Iri #325, Milyang #22, Suwon #251, and Suwon #267 were showing over 70 percent of germination ratio, while varieties, Dodolokiwase, Milyang #23, Yusin, Iri #328, and Iri #329 were below 10 percent in germination ratio. 2. Under the condition of $15^{\circ}C$., variety Suwon #262 was shown 100 percent of germination and varieties such as Milyg an#22, Dodlokiwase, Tongil, Milyang #29, Suwon #258, Milyang #23, Milyang #24, Milyang #28 and Milyang #21 were over 90 percent in germination ratio. However, varieties such as Iri #328, Iri #329, Jinheung, and Minehikari were below 50 percent in germination rate. 3. Considering the germination rate and average days required to germination, Milyang #22 and Iri #323 were highly resistant varieties to low temperature. The rate of germination of varieties from the cross between Indica and Japonica under low temperature was not always lower than that of varieties from Japonica type of rice. 4. Most of the seedlings of varieties used were quite resistant to $1^{\circ}C$. at least for six hours. However, leaves of rice were exposed to the the temperature of $1^{\circ}C$. for 12 hours, and the withered leaves were recovered soon when put in under normal temperature. The degree of leaf withering under low temperature was lower in Milyang #15, Japonica type than in varietieties belonging to $Indica{\times}Japonica$ type of rice. 5. When the seedlings of varieties such as Josaengtongil, Tongil and Yusin were exposed to the temperature of $1^{\circ}C$. for 36 hours, 70 percent of leaves were withered and when the time of expose were doubled, the leaves were completely died. When the temperature was fluctuated, over 75 percent of leaves were died, while 65 percent of leaves of Milyang #15 were died when exposed to $1^{\circ}C$, for 72 hours. 6. Significant growth retardation was observed for all entries when exposed to $1^{\circ}C$. for 24 to 36 hours. The growth retardation was apparently increased as the time of expose was extended.

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A Case of Carbamoyl Phosphate Synthetase 1 Deficiency with Novel Mutations in CPS1 Treated by Liver Cell Transplantation (신생아기에 새로운 CPS1 유전자 돌연변이가 확인되고, 간세포이식 시행 받은 Carbamoyl phosphatase synthetase 1 deficiency 1례)

  • Lee, Jisun;Yang, Aram;Kim, Jinsup;Park, Hyung-Doo;Lee, Sanghoon;Lee, Suk-Koo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.17 no.1
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    • pp.31-37
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    • 2017
  • Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

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