• Clinical and Experimental Pediatrics
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• 제57권2호
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• pp.91-95
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• 2014

Griscelli syndrome type 2 (GS2) is a rare autosomal recessive disease caused by mutations in the RAB27A gene. It is characterized by cutaneous hypopigmentation, immunodeficiency, and hemophagocytic lymphohistiocytosis. We describe 2 brothers who had GS2 with clinically diverse manifestations. The elder brother presented with a purely neurological picture, whereas the younger one presented with fever, pancytopenia, hepatosplenomegaly, and erythema nodosum. Considering that cutaneous hypopigmentation was a common feature between the brothers, genetic analysis for Griscelli syndrome was performed. As the elder sibling had died, mutation analysis was only performed on the younger sibling, which revealed a novel homozygous mutation in the RAB27A gene on chromosome 15 showing a single-base substitution (c.136T>A p.F46I). Both parents were heterozygous for the same mutation. This confirmed the diagnosis of GS2 in the accelerated phase in both siblings. The atypical features of GS2 in these cases are a novel mutation, isolated neurological involvement in one sibling, association with erythema nodosum, and 2 distinct clinical presentations in siblings with the same genetic mutation.

• 한국수학교육학회지시리즈B:순수및응용수학
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• 제11권2호
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• pp.167-173
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• 2004

Given vectors x and y in a separable Hilbert space $\cal H$, an interpolating operator is a bounded operator A such that Ax = y. In this article, we investigate Hilbert-Schmidt interpolation problems for vectors in a tridiagonal algebra. We show the following: Let $\cal L$ be a subspace lattice acting on a separable complex Hilbert space $\cal H$ and let x = ($x_{i}$) and y = ($y_{i}$) be vectors in $\cal H$. Then the following are equivalent; (1) There exists a Hilbert-Schmidt operator A = ($a_{ij}$ in Alg$\cal L$ such that Ax = y. (2) There is a bounded sequence {$a_n$ in C such that ${\sum^{\infty}}_{n=1}\mid\alpha_n\mid^2 < \infty$ and $y_1 = \alpha_1x_1 + \alpha_2x_2$ ... $y_{2k} =\alpha_{4k-1}x_{2k}$ $y_{2k=1} = \alpha_{4kx2k} + \alpha_{4k+1}x_{2k+1} + \alpha_{4k+1}x_{2k+2}$ for K $\epsilon$ N.

• 한국진공학회:학술대회논문집
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• 한국진공학회 2011년도 제41회 하계 정기 학술대회 초록집
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• pp.196-196
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• 2011

Molecular $N_2O$ has bee known to react over oxygen vacancy on a reduced rutile $TiO_2$(110)-1${\times}$1 surface to desorb as molecular $N_2$ leaving oxygen atom behind. In the present study, we investigated the reaction of $N_2O$ on rutile $TiO_2$(110) using temperature-programmed desorption (TPD). Our results indicate that $N_2O$ does not react over the oxygen vacancy under a typical UHV experimental condition. On a rutile $TiO_2$(110)-1${\times}$1 with a well-defined oxygen vacancy concentration of 5% ($2.6{\times}10^{13}/cm^2$), $N_2O$ desorption features show a monolayer peak maximum at 135 K followed by a small peak maximum at 170 K. When the oxygen vacancy is blocked with $H_2O$, the $N_2O$ peak at 170 K disappears completely, indicating that the peak is due to molecular $N_2O$ interacting with oxygen vacancy. The integrated amount of desorbed $N_2O$ plotted against the amount of adsorbed $N_2O$ however shows a straight line with no offset indicating no loss of $N_2O$ during our cycles of TPD measurements. In addition, our $N_2O$ uptake measurements at 70~100 K showed no $N_2$ (as a reaction product) desorption except contaminant $N_2$. Also, $H_2O$ TPD taken after $N_2O$ scattering up to 350 K indicates no change in the vacancy-related $H_2O$ desorption peak at 500 K showing no change in the oxygen vacancy concentration after the interaction with $N_2O$.

• Archives of Pharmacal Research
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• 제29권1호
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• pp.16-25
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• 2006

A novel series of 1-(1-benzofuran-2-yl-ethylidene)-4-substituted thiosemicarbazides (2a-d) along with some derived ring systems: substituted-2,3-dihydro-thiazoles(3a-c, 4a-f) and thiazolidin-4-ones(5a-d and 6a-d), were synthesized. In addition, cyanoacetic acid-(1-benzofuran-2-yl-ethylidene) hydrazide(7) was used to prepare another new series of compounds consisting of substituted pyridin-2(1H)-ones(8a-c); 2-thioxo-2,3-dihydro-thiazoles(9a-d) and 2-thioxo-2,3-dihydro-6H-thiazolo[4,5-d]pyrimidin-7-ones (10a-c, 11a-c). The absolute configuration of compound 5c was determined by X-ray crystallography. The compounds prepared were evaluated for their in vitro anti-HIV, anticancer, antibacterial, and antifungal activities. Among the tested compounds, compounds 5c and 9a produced a significant reduction ㅐ ㄹ the viral cytopathic effect (93.19% and 59.55%) at concentrations $>2.0{\times}10^{-4}\;M\;and\;2.5{\times}10^{-5}\;M$respectively. Compound 9a was confirmed to have moderate anti-HIV activity. Compounds 2a, 2d, and 5c showed mild antifungal activity. However, none of the tested compounds showed any significant anticancer activity.

• 대한화학회지
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• 제44권4호
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• pp.311-315
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• 2000

The Fe(II)-isothiocyanato complex $trans-[FeH(NCS)(dppe)_2]$ (1) eactedwith iodomethane(Mel) to give methyl isothiocyanide-Fe(n) complex, $trans-FeH(NCS(Me)-S)(dppe)_2]I(2)$. Compound 2 was oxidized to $trans-[Fe(NCS)_2(Ph_2P(O)CH_2CH_2P(O)Ph_2)_2][I_3]$ (3), which was structurally characterized by X-ray diffraction. The molecular structure of 3 showed a bent Fe-NCS group, Crystallographic data for 3: triclinic space group P1,a=11.071(2) A,b=12.054(2)A,c=12.121(1)A, $\alpha=101.02(1){\circ}C{\beta}=95.887(9){\circ}Cr=110.34(1){\circ}C$, $Z=1R(wR_2)=0.0567(0.1294)$.

• 한국결정학회지
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• 제2권1호
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• pp.17-22
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• 1991

Cd2+ 이온으로 이온 교환된 제올라이트 A를 탈 수한 후 C2H2 기체를 흡착한 결정구조를 단결정 X-선 회절법으로 입방공간군 Pm3m을 사용하여 해석하였다. (a=12.202(3)남 이고 Z:1 임). 이 결정은 723 K에서 2.67×10-4 Pa의 진공하에 서 2일간 탈수시킨 뒤 1.60×104 Pa의 C2H2 기체를 298 K에서 흡착시켜 만들었다. 단위 세포당 6개의 Cd2+ 이온은 모두 골조의 결정학적으로 서로 다른 2개의 3회 회전축상 6 산 소고리 위치에 위치한다. 즉 이들 Cd2+ 이온중 2개 는 3개의 0(3) 산소로 이루어진 (111) 평면에서 0. 694A 만큼 sodalite 동공내로 들어간 위치에 위치하 였고 나머지 4개의 Cd2+ 이온은 0(3)의 (111) 평면에서 큰 동공으로 0.586A.들어가 있었다. 이 4개의 Cd2+ 이온은 거의 정사면체형으로서, 3개의 골조 산소로부터 2.220(9)남 그리고 윤Ha 분자(여기서 는 한자리 배위자로 간주)의 탄소로부터는 2.74(7) A 떨어진 위치에 있다. I>3c (I)인 292개의 회절점으로 Rl=0.093, R2 =0.105까지 정밀화시켰다.

• 한국세라믹학회지
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• 제26권4호
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• pp.479-486
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• 1989

According to the phase diagram, 6Bi2O3.GeO2 composition melts congruently at 93$0^{\circ}C$ and forms a stable ${\gamma}$-6Bi2O3.GeO2 crystal phase below the melting point. But when the melt of this composition was cooled at a rate 1-15$0^{\circ}C$/min without tapping by a glass rod or impurity addition, a metastable $\delta$-6Bi2O3.GeO2 crystal phase was formed. It is due to that as the nucleation energy barrier of $\delta$-6Bi2O3.GeO2 crystals, which have more open and defective structure, is lower than that of ${\gamma}$-6Bi2O3.GeO2 crystals. When impurities or ${\gamma}$-6Bi2O3.GeO2 crystals existed in the melt, stable ${\gamma}$-6Bi2O3.GeO2 crystal phase was formed at various cooling rate. It is because of that the impurities or the ${\gamma}$-6Bi2O3.GeO2 crystals role as a seed crystal and as a result the nucleation energy barrier of ${\gamma}$-6Bi2O3.GeO2 crystals is lowered.

• 한국육종학회지
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• 제41권4호
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• pp.463-467
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• 2009

Allium 속 근연종인 파와 양파간 종간교잡을 이용하여 양파로부터 새로운 형질을 도입하는 과정에서 유래되는 종간교잡 F1과 여교잡 세대들의 게놈크기의 변화를 flow cytometry를 이용하여 측정한 결과 다음과 같은 결과를 얻었다. 1. 양파와 파의 종간교잡 F1의 식물학적 특성은 양친의 중간형을 보였으나 여교잡이 진전됨에 따라 반복친인 파의 표현형이 우세하였다. flow cytometry를 이용하여 2C nuclear DNA content을 측정한 결과 $F_1$은 양친에 비해 오히려 증가하였으나 여교잡이 진행됨에 따라 급속히 감소하여 $BC_2F_1$에서는 반복친과 비슷한 수준으로 감소하였다. 여교잡세대의 자식인 $BC_1F_2$은 반복친과 비슷한 $23.1{\pm}1.0pg$으로 측정되었으나 $BC_2F_2$는 $22.9{\pm}0.4pg$으로 반복친의 2C nuclear DNA content인 $23.2{\pm}0.2pg$ 보다 오히려 감소하였다.

• 구강회복응용과학지
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• 제31권4호
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• pp.294-300
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• 2015

목적: 저점도 벌크필레진과 복합레진을 적층한 수복물의 색조를 평가하기 위함이다. 연구 재료 및 방법: 저점도 벌크필레진(SDR)과 A2, A3 색조의 미세혼합형 복합레진(A2, A3)을 4 mm 두께로 제작하여 광중합한 후 색차계를 이용하여 레진 시편의 CIE $L^*a^*b^*$ 값을 측정하였다. 이후 저점도 벌크필레진 상방에 2 mm 두께로 A2, A3 색조의 미세혼합형 복합레진을 적층하여(SA2, SA3) 광중합한 후 색측정을 하고(n = 10), 저점도 벌크필레진과 미세혼합형 복합레진, 그리고 이들을 적층한 시편의 색차(${\Delta}E$)를 계산하였다. 결과: $L^*$ 값은 SDR이 가장 컸고 SA2, SA3 그리고 A2, A3 순으로 감소했다. $a^*$ 값은 SDR이 가장 작았고 SA2, SA3 그리고 A2, A3 순으로 증가했다. $b^*$ 값은 SDR이 가장 작았으며 A2, SA2 그리고 A3, SA3 순으로 증가했다. 적층한 시편과 미세복합형 복합레진 시편의 ${\Delta}E$ 값은 A2와 SA2 사이에 ${\Delta}E=3.4$, A3와 SA3 사이에 ${\Delta}E=3.1$로 계산되었다. 결론: 저점도 벌크필레진과 미세혼합형 복합레진을 적층한 시편과 미세혼합형 복합레진만으로 제작한 시편의 색조의 차이는 일반적으로 인지하지 못할 정도로 작았다.

• 한국결정학회지
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• 제9권2호
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• pp.149-152
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• 1998

The accurate expression of the anisotropic thermal parameters is either exp (-2$π^{2}$ < $h^{2}$ $\frac{$u_x^2$}{$a^{2}$}$ + $k^{2}$ $\frac{$u_y^2$}{$b^{2}$}$ + $l^{2}$ $\frac{$u_z^2$}{$c^{2}$}$ + 2hk $\frac{$u_{x}}{a}$ $\frac{$u_{y}}{b}$ 2hl $\frac{$u_{x}}{a}$ $\frac{$u_{z}}{c}$ 2kl $\frac{$u_{y}}{b}$ $\frac{$u_{z}}{c}$ > ) with the small displacements Ux, Uy, uz, in absolute measure or exp (-2$π^{2}$ < $h^{2}$ $u_x^2$ + $k^{2}$ $u_y^{2}$ + $l^{2}$ $u_z^{2}$ + 2hk$u_{x}$ $u_{y}$ + 2hl$u_{x}$$u_{z}$ + 2kl$u_{y}$ $u_{z}$ > ) with the small displacements Ux, Uy, Uz in fractional measure.