• Title/Summary/Keyword: X9R

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Clinical Features, Response to Treatment, Prognosis, and Molecular Characterization in Korean Patients with Inherited Urea Cycle Defects

  • Yoo, Han-Wook;Kim, Gu-Hwan;Seo, Eul-Ju
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.2 no.1
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    • pp.77-79
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    • 2002
  • The urea cycle, consisting of a series of six enzymatic reactions, plays key roles to prevent the accumulation of toxic nitrogenous compound and synthesize arginine de novo. Five well characterized diseases have been described, resulting from an enzymatic defect in the biosynthesis of one of the normally expressed enzyme. This presentation will focus on two representative diseases; ornithine transcarbamylase(OTC) deficiency and citrullinemia(argininosuccinate synthetase deficiency). OTC deficiency is one of the most common inborn error of urea cycle, which is inherited in X-linked manner. We identified 17 different mutations in 20 unrelated Korean patients with OTC deficiency; L9X, R26P, R26X, T44I, R92X, G100R, R141Q, G195R, M205T, H214Y, D249G, R277W, F281S, 853 del C, R320X, V323M and 10 bp del at nt. 796-805. These mutations occur at well conserved nucleotide sequences across species or CpG hot spot. The L9X and R26X lead to the disruption of leader sequences, required for directing mitochondrial localization of the OTC precursor. Their phenotypes are severe, and neonatal onset. The G100R, R277W and V323M mutations were uniquely identified in patients with late onset OTC deficiency. The other genotypes are associated with neonatal onset. Out of 20 patients with OTC deficiency, only 6 patients are alive; two were liver transplanted, and normal in growth and development at 2, 4 years after transplantation respectively. Citrullinemia is an autosomal recessive disease, caused by the mutations in the argininosuccinate synthetase(ASS) gene. We identified in 3 major mutations in 11 unrelated Korean patients with citrullinemia; G324S, $IVS6^{-2}$ A to G, and 67 bp ins at nt 1125-1126. Among these, the 67 base pair insertion mutation is novel. The allele frequency of each mutation is; G324S(45%), IVS6-2 A to G(32%), and 67 base pair insertion(14%). All patients are diagnosed at neonatal or infantile age. Interestingly, two patients presented with stroke like episode. Out of 11 patients, 5 patients died. Among 6 patients alive, one patient was successfully liver transplanted.

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Analysis of Growth Characteristics as Affected by Air Temperature Variation in Dasanbyeo, a Indica/Japonica High Yielding Rice Cultivar (기온변리에 따른 다수성 품종 '다산벼'의 생육특성 분석)

  • Kim Deog-Su;Shin Jin-Chul;Ryu Jeom-Ho
    • Korean Journal of Agricultural and Forest Meteorology
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    • v.2 no.2
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    • pp.41-46
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    • 2000
  • This experiment was carried out to find out the responses of the growth characteristics to various air temperature in a high yielding indica/japonica rice cultivar, 'Dasanbyeo'. Five different transplanting dates with 30 day-old seedlings were tried to induce various environmental conditions, starting from 10th May to 19th June in the interval of 10 days at National Crop Experiment Station, in 1998. Planting densities with different plants per hill but same hill spacing were tested. The daily mean air temperature during 30 days after transplanting was 19.0, 20,8, 21.9, 24.4 and 25.2$^{\circ}C$ when transplanted on the 10th May, the 20th May, the 30th May, the 9th June and 19th June, respectively. The number of tillers per hill at 30 days after transplanting was affected by the daily mean air temperature during 30 days after transplanting. The higher the daily mean air temperature, the more tillers per hill at 30 days after transplanting were. The growth duration from transplanting to heading was greatly changed according to the transplanting dates. It was shortened as the transplanting date was delayed until transplanted on the 9th June, but it was prolonged when transplanted on the 19th June compared to that of the 9th June. The number of panicles per hill increased with the increase of the transplanted plants per hill up to 7 plants per hill. The number of panicle per unit area was closely related to the number of tillers per unit area at maximum tillering stage. It was found that the average effective temperature during first 10 tiller formation related to the days to the tenth tiller formation with the relationship of y = 1.071x$^2$ - 37.307x + 342.38($R^2$ = 0.9823).

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Studies on the G-banding Patterns of Normal and of Delayed Spiralized Chromosomes by BUdR in Dwarf Hamsters (Dwarf Hamster의 正常染色體와 BUdR에 의해 凝縮遲延된 染色體의 G-banding Pattern에 대한 硏究)

  • Hahn, Sahsook
    • The Korean Journal of Zoology
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    • v.18 no.2
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    • pp.71-86
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    • 1975
  • The G-banding patterns of normal and of delayed spiralized chromosomes by BUdR were investigated in three established cell lines of dwarf hamsters. The results obtained were as follows: 1. The number of G-bands appeared in Chinese hamster T-233 cell line was 65. The centromeric dark band was found in No.1 chromosome and weakly stained bands were also observed in part of the centromeric regions of Nos. 2, 3, 8 and $X_2$ chromosomes. Two homologous X chromosomes were found in different banding patterns. Terminal dark bands were shown in No. 1 chromosome. No conspicuous bands appeared in No. 10 chromosome. 2. Eighty four bands appeared in Armenian hamster Y-1249 cell line. Centromeric dark bands were observed in Nos. 5 and 10 chromosomes and moderatly stained bands were also found in near the centromeric region of the long arms of Nos. 7 and 9 chromosomes. Two isomorphic X chromosomes were also distinguished by their banding patterns. 3. In Y-1313 Armenian hamster cell line, the bands were 69. No centromeric dark bands were observed in this cell line, but moderatly stained bands appeared in the centromeric area in the long arm of No. 9 chromosome. The banding patterns of these two cell lines of Armenian hamster were quite different and readily distinguished. Only No. 8 chromosome showed similar G-banding patterns. Although Nos. 5, 7 abd 8 chromosomes revealed the same number of bands in these two cell lines, the location and staining intensity were quite different. 4. Chromosomes of Nos. 1, 2, 6, $X_1$ and $X_2$ in T-233 cell line and of 1, 4, 7, 8, 9, $X_1$ and $X_2$ in both cell lines of Armenian hamster were found to be elongated due to the inhibition of mitotic spiralization by BUdR. G-banding patterns of these chromosomes were found to be identical to those of normal chromosomes in these cell lines.

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3-Port Circulator for X-Band Radar (X-Band 레이더를 위한 3-포트 서큘레이터)

  • Yoon, Sung-Hyun
    • The Journal of Korean Institute of Communications and Information Sciences
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    • v.40 no.2
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    • pp.355-362
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    • 2015
  • In this study, we have fabricated 9.385[GHz] circulator that is composed of WR112 waveguide and Ferrite for X-band radar. For designing Ferrite, B/R mode(Below Resonance mode) was used and calculated the condition of 120 degree rotation of the electric field in Ferrite and calculated internal DC magnetic field and external DC magnetic field. Also, dielectric materials of the same shape with Ferrite was filled between two Ferrite for improving the performance of the circulator, including impedance matching, bandwidth, quality factor, insertion loss. To obtain optimum shape of the Ferrite and dielectric material, we used CST MWS. Simulation result of the circulator is that 1.02 : 1 VSWR, -40dB isolation, 0.2dB insertion loss and measurement result is that 1.03 : 1, -38dB, 1.2dB at 9.385[GHz]. We can get good agreement at isolation and VSWR, but insertion loss was 1 dB great than simulation result.

The Effect of Bismuth Composition Controlled by Heat Treating Heterostructure on the Ferroelectric Properties of SrBi$_2$Ta$_{2-x}$Nb$_x$O$_9$ Thin Films (Heterostructure 열처리에 의한 Bi 조성 제어가 SrBi$_2$Ta$_{2-x}$Nb$_x$O$_9$ 박막의 강유전 특성에 미치는 영향)

  • 박윤백;이전국;정형진;박종완
    • Journal of the Korean Ceramic Society
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    • v.35 no.10
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    • pp.1040-1048
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    • 1998
  • Ferroelectric properties of{{{{ { { { {SrBi }_{2 }Ta }_{2-x }Nb }_{x }O }_{9 } }} (SBIN) thin films were affected by the amount of Bi content in SBTN. The addition of Bi into the SBTN films could be accomplished by heat treating SBTN/Bi2O3/SBTN het-erostructures fabricated by r.f. magnetron sputtering method. The variation of Bi content was controlled by changing the thickness of the sandwiched Bi2O3 in SBTN/Bi2O3/SBTN heterostructure from 50 to 400$\AA$. As changing the thickness of the sandwiched Bi2O3 in Bi2O3 films was increased from 0 to 100$\AA$ the grain grew faster and the ferroelectric pro-perties were improved. On the other hand when the thickness of Bi2O3 films was thicker than 150$\AA$ the fer-roelectric properties were deteriorated Especially for SBTN thin films inserted by 400$\AA$ Bi2O3 layer a Bi2Phase appeared as a second phase resulting in poor ferroelectric properties. The maximum remanent po-larization (2Pr) and coercive field(Ec) were obtained for the SBTN/Bi2O3/(100$\AA$)/SBTN thin films. In this case 2Pr and Ec were 14.75 $\mu$C/cm2 and 53.4kV/cm at an applied voltage of 3V respectively.

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Statistical Characteristics of Diazinon Degradation using E-beam (전자빔을 이용한 통계적 Diazinon 분해특성 연구)

  • Lee, Sijin
    • Journal of the Korean GEO-environmental Society
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    • v.14 no.5
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    • pp.57-63
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    • 2013
  • In this study, the characteristics of degradation and mineralization of diazinon using a statistical approach based on Box-Behnken design (BBD, one of response surface method) was investigated in an E-beam process, and also the main factors with diazinon concentration ($X_1$), irradiatin intensity ($X_2$) and pH ($X_3$) which consisted of 3 levels in each factor was set up to determine the effects of factors and optimization. At first, effects of pH and diazinon concentration were investigated to determine the proper range of application on response surface method(RSM). In statistical approach, the regression analysis and analysis of variance (ANOVA) were applied to evaluate the quantitative comparison of each factors in order to obtain the effects were irradiation intensity>diazinon concentration>pH. The regression model predicted the optimization point using the response optimizer to consider the effects of operation conditions were $Y_1=81.73-5.58X_1+23.69X_2-14.23X{_2}^2+4.22X{_3}^2(R^2=99.7%)$, $Y_2=35.23-3.01X_1+10.79X_2-7.58X_2{^2}(R^2=97.9%)$ and 95.7% of diazinon degradation, 41.8% of TOC reduction at 12.75mg/L and 4.26kGy, respectively. The pH condition was not significantly affects on E-beam process than other advanced oxidation processes (AOPs).

Investigation of Optimal ionic Concentration of Nutrient Solution for the Water Culture of Young Welsh Onion (실파의 수경재배에 적합한 양액농도 구명)

  • Won Jae Hee;Kim Sang Soo;Jeong Byung Chan;Park Kuen Woo
    • Journal of Bio-Environment Control
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    • v.14 no.4
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    • pp.269-274
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    • 2005
  • The purpose of this experiment was to investigate optimal ionic concentration of nutrient solution for water culture of young welsh onion (Allium fistulosum). For the purpose of clarification of optimal nutrient concentration to maximize growth of young welsh onion, different nutrient concentrations of Yamazaki's solution for welsh onion seedling $(NO_3^--N\;9.0,\;NH_4^+-N\;3.0,\;PO_4^{3-}-P\;6.0,\;K^+7.0,\;Ca^{2+}\;2.0,\;Mg^{2+}\;2.0,\;and\;SO_4^{2-}-S\;4.4me{\cdot}L^{-1})$ which selected by prior experiment were treated as 0.6, 1.2, 1.8, and $2.4dS{\cdot}m^{-1}$. Increments of fresh weight, dry weight and top length were the highest in 1.2 and, in the next, were placed by the order of 1.8, 2.4, and $0.6dS{\cdot}m^{-1}$ The regression coefficients for the maximal growth of fresh weight of cv. 'Geurnjanguedaepa' and 'Tokyokuro' were $y=-42.091x^2+171.79x+11.047 (R^2=0.8946,\; R=0.9458^*)\;and\;y=-50.069x2+157.58x+15.414(R^2=0.9343,\;R=0.9692^{**})$, respectively, and optimal EC levels according to regression coefficients were 1.68 and $1.57dS{\cdot}m^{-1}$. As the conclusions, optimal nutrient levels far young welsh onion were $1.2dS{\cdot}m^{-1}$ EC in the early growth stage and $1.6\~l .7dS{\cdot}m^{-1}$ in the later growth stage.

Load Bearing Capacity of Welded Joints between Dissimilar Pipelines with Unequal Wall Thickness (두께가 다른 이종배관 용접부 면삭 각도 변화에 따른 하중지지능력 평가)

  • Baek, Jong-Hyun;Kim, Young-Pyo;Kim, Woo-Sik
    • Transactions of the Korean Society of Mechanical Engineers A
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    • v.36 no.9
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    • pp.961-970
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    • 2012
  • The behavior of the load bearing capacity of a pipeline with unequal wall thickness was evaluated using finite element analyses. Pipelines with a wall thickness ratio of 1.22-1.89 were adopted to investigate plastic collapse under tensile, internal pressure, or bending stress. A parametric study showed that the tensile strength and moment of a pipeline with a wall thickness ratio less than 1.5 were not influenced by the wall thickness ratio and taper angle; however, those of a pipeline with a wall thickness ratio more than 1.5 decreased considerably at a low taper angle. The failure pressure of a pipeline with unequal wall thickness was not influenced by the wall thickness ratio and taper angle.