• 대한유전성대사질환학회지
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• 제6권1호
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• pp.15-23
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• 2006

Purpose: Glycogen storage disease type III (GSD-III), is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme is amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), which is responsible for the debranching of the glycogen molecule during catabolism. The disease has been demonstrated to show clinical and biochemical heterogeneity, reflecting the genotype-phenotype heterogeneity among different patients. In this study, we analyzed mutations of the AGL gene in three unrelated Korean GSD-III patients and discussed their clinical and laboratory implications. Methods: We studied three GSD-III patients and the clinical features were characterized. Sequence analysis of 35exons and part exon-intron boundaries of the AGLgene in patients were carried out by direct DNA sequencing method using genomic DNA isolated from patients' peripheral leukocytes. Results: The clinical features included hepatomegaly (in all patients), seizures (in patient 2), growth failure (in patients 1), hyperlipidemia (in patients 1 and 3), raised transaminases and creatinine kinase concentrations (in all patients) and mild EKG abnormalities (in patients 2). Liver transplantation was performed in patient 2due to progressive hepatic fibrosis. Administration of raw-corn-starch could maintain normoglycemia and improve the condition. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 with c.1510_1511insT (p.Y504LfsX10), and patient 3 with c.3416 T>C (p.L1139P) and c.l735+1 G>T (Y538_R578delfsX4) mutations. Except R428K mutation, 4 other mutations identified in3 patients were novel. Conclusion: GSD-III patients have variable phenotypic characteristics resembling GSD-Ia. The molecular defects in the AGL gene of Korean GSD-III patients were genetically heterogeneous.

• 대한화장품학회:학술대회논문집
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• 대한화장품학회 2003년도 IFSCC Conference Proceeding Book II
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• pp.73-86
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• 2003

Endothelins secreted from keratinocytes are intrinsic madiators for human melanocytes in UVB-induced pigmentation. Antimelanogenic ingredient, 1,2-Ο-diferulylglycerol(SM709) isolated from bamboo extract inhibited the melanin synthesis of Bl6F10 melanoma cells by 62%. To understand the cellular mechanism of antimelanogenic activity of SM709 in human melanocytes, the effects of SM709 on the ET-l-induced $Ca^{2+}$ mobilization were investigated. ET-l receptors in human melanocytes were characterized by using specific antagonist and found that ET-l increased intracellular $Ca^{2+}$ by activating ET-B receptor. SM709 completely blocked the ET-l-induced intracellular $Ca^{2+}$ increase and its inhibitory effect showed dose- and time- dependent manners. To investigate the role of SM709 on intracellular $Ca^{2+}$ store, when the $Ca^{2+}$ store was partially depleted by thapsigargin; a specific inhibitor of ER-type $Ca^{2+}$-ATPase, caffeine-induced $Ca^{2+}$ mobilization did not changed in the presence or absence of SM709, suggesting that SM709 has no effect on the $Ca^{2+}$ store. It is known that LPA receptor and P$_2$ receptor are linked to InsP$_3$ second messenger system. When these receptors in melanocytes were activated by LPA and ATP, the intracellular $Ca^{2+}$ signaling was observed even in the presence of SM709. From the above results, it can be suggested that SM709 has an antimelanogenic activity by antagonizing the ET-B receptor, resulting in subsequent intracellular $Ca^{2+}$ signaling, in UV induced pigmentation.nduced pigmentation.

• 대한치과교정학회지
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• 제28권1호
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• pp.123-133
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• 1998

TiN ion-plating된 Co-Cr 선재(.016", .016"x.022")와 ion-plating되지 않은 선재(.016", .016"x.022")를 이용하고, 3 가지 종류의 브라켓(TiN ion-plating된 금속 브라켓, 세라믹 브라켓 및 플라스틱 브라켓)을 대상으로 마찰실험을 행하고, 거기에서 얻어진 마찰 특성곡선과 곡선으로부터 구한 최대 정지마찰력, 그리고 선재와 브라켓의 표면양상을 주사전자현미경으로 관찰하여 TiN ion-plating의 효과를 검토한 결과 다음과 같은 결론을 얻었다. $\cdot$3가지 종류의 브라켓에 TiN ion-plating된 선재를 사용한 경우의 마찰력은 TiN ion-plating되지 않은 선재를 사용한 경우의 마찰력보다 각각 통계학적으로 유의성있게 낮았다(p<0.05). $\cdot$3가지 종류의 브라켓에 원형 선재를 사용한 경우의 마찰력은 각형 선재를 사용한 경우의 마찰력보다 각각 통계학적으로 유의성있게 낮았다(p<0.05). $\cdot$원형 선재를 사용한 경우 TiN ion-plating되지 않은 선재를 사용한 경우가 TiN ion-plating된 선재를 사용한 경우보다 선재 및 브라켓 슬롯의 표면이 더욱 거친 양상을 나타내었다. $\cdot$각형 선재를 사용한 경우 전반적으로 원형 선재를 사용한 경우보다 선재 및 브라켓 슬롯의 표면이 더욱 거친 양상을 나타내었다. $\cdot$TiN ion-plating된 원형 선재를 사용한 경우 정지마찰력과 운동마찰력의 차는 별로 없었으나 TiN ion-plating된 각형 선재를 사용한 경우 정지마찰력은 운동마찰력보다 다소 높았다. $\cdot$TiN ion-plating되지 않은 선재를 사용한 경우가 TiN ion-plating된 선재를 사용한 경우에 비해 정지마찰력이 운동마찰력보다 휠씬 높았다.

• 한국식품영양과학회지
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• 제35권2호
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• pp.199-204
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• 2006

본 연구에서는 NF 막 분리에 의하여 제조된 농축 분말을 사용하여 국수를 제조하고 특성을 살펴봄으로써 새로운 기능성 식품소재로의 활용가능성을 검토하였다. NF 분말의 첨가량에 따른 밀가루 혼합분의 점도 변화는 NF분말 첨가량이 증가함에 따라 최고점도(peak), 최저점도(trough), 최종점도(final viscosity)가 감소하는 경 향을 보였다. 국수의 조리 후 무게 및 부피증가율은 NF분말 첨가량이 증가할수록 높았고 국물의 탁도 역시 NF분말 첨가 국수가 대조군보다 높아 고형분의 손실이 큰 것으로 나타났다. 색도 측정결과 생국수와 조리 국수의 L값은 시험군 간에 큰 차이가 없었고 a값은 (-)값으로 녹색을 나타냈고 b값은 (+)값으로 황색을 나타냈으며 NF분말 첨가량이 증가할수록 모두 증가하였다. 생국수의 경도는 NF 분말 첨가량 증가에 따라 감소하였고 응집성, 탄성, 껌성, 깨짐성은 시료간에 뚜렷한 경향을 보이지 않았다. 조리 후에는 응집성을 제외한 경도, 탄성, 껌성, 깨짐성 등의 조직감 특성에서 NF분말 첨가량이 증가 할수록 대조군에 비해 크게 낮아지는 경향을 나타내었다. 관능검사결과 색과 냄새는 NF 분말 첨가군이 대조군보다 높은 점수로 평가되었고 부드러운 정도는 NF 분말 첨가군과 대조군 간에 유의적인 차이가 없었다. Stickiness는 NF 분말 첨가군이 대조군보다 강한 것으로 평가되었고 전체적인 바람직성은 대조군과 NF분말 $3\%$ 첨가까지 모두 차이가 없는 것으로 평가되었고 $5\%$ 첨가군만이 유의적으로 낮은 점수를 받았다. 이상의 결과로 볼 때 NF분말을 이용하여 첨가수준을 적절히 조절하고 제조방법을 다양화한다면 기능성이 강화된 국수로서 그 이용가치가 있을 것으로 기대된다. 제품에서는 표시기준에 의거 표시된 소르빈산 $0.02\%$보다 15배$(0.30\%)$ 높은 검출결과가 나왔다. 결론적으로 경인지역 초등학교주변에서 판매되고 있는 식품은 식품의 제조단계에서부터 유통, 판매에 이르기까지 안전을 위한 관리방안이 조속히 수립되어야 한다.hrombin 부하 후 증강되지는 않았다. $Ins(1,4,5)P_3$ 증가에 이어서, $[Ca^{2+}]_i$은 thrombin 부하 후 20초에 최고점에 이르며, 이러한$[Ca^{2+}]_i$, 증가는 세 약물에 의하여 현저하게 억제되었다. 혈소판 단백인산화에 대해서, thrombin은 $41{\sim}43\;kDa$ 및 20kDa 단백인산화를 현저하게 증가시켰으며, 이는 AMT, SRT 및 CPZ에 의하여 억제되었다. CPZ, AMT 및 SRT 등의 세 약물은 유의한 항응집효과와 thromboxane생성억제 효과를 나타냈으며, 이들 약물에 의한 protein kinase C 활성억제 및 $Ins(1,4,5)P_3$의 함량증가는 각각 이들약물의 항응집효과 및 항우울성 작용기전과 연관될 수 있음을 시사한다.in banding pattern을 분석한 결과 C. militaris, C. roseostromata, C. kyushuensis는 근연종으로 clustering 되었으며 C. scarabaeicola, Phytocordyceps ninchukiospora는 비교적 유연관계가 먼 것으로 나타났다. 경우 $logk=11.1140-4.1226{\times}10^3(1/T)$, waxy corn starch의 경우 $logk=10.

• 한국자기공명학회논문지
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• 제4권1호
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• pp.19-28
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• 2000

Authors evaluated alterations of observable metabolite ratios between the cerebral lesion and the contralateral region related to the clinical symptomatic side in levodopa-treated Parkinson's disease (PD) and investigated correlation between age in patients with PD and metabolite ratios of the lesion. Patients with levodopa-treated PD (n = 54) and age-matched normal controls (n = 15) underwent magnetic resonance spectroscopy (MRS) examinations using a stimulated echo acquisition mode (STEAM) pulse sequence that provided 2$\times$2$\times$2 cm3 volume of interest in the selected regions of substantia nigra (SN) and putamed-globus pallidus (PG). To evaluate dependence of metabolite ratios on age, we divided into two groups (i.e., younger and older age). We quantitatively measured N-acetylaspartate (NAA), creatine (Cr), choline-containing compounds (Cho), inositols (Ins), and the sum of glutamate (Glx) and GABA levels and obtained proton metabolite ratios relative to Cr using a Marquart algorithm. Compared with the contralateral region, a significant neuronal laterality of the NAA/Cr ratio in the lesion of SN related to the clinical symptomatic side was established (P = 0.01), but was not established in the lesion of PG (P = 0.24). Also, Cho/Cr ratio tended toward significance in the lesion of SN (P = 0.07) and was statistically significant in the lesion of PG(P = 0.01). Compared with that in the younger age group, NAA/Cr ratio in the older age was decreased in the lesion of SN (P = 0.02), while NAA/Cr ratio was not statistically significant in the lesion of PG ( P = 0.21). Significant metabolic alterations of NAA/Cr and Cho/Cr ratios might be closely related with functional changes of neuropathological process in SN and PG of levodopa treated PD and could be a valuable finding for evaluation of the PD. A trend of NAA/Cr reduction, being statistically significant in older patients, could be indicative of more pronounced neuronal damage in the SN of the progressive PD.

• 전력전자학회논문지
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• 제5권2호
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• pp.154-162
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• 2000

본 논문에서는 3상 강압형 다이오드 정류기에서 출력전압의 저주파 리플 전압을 감소시키기 위한 새로운 제어기법을 제안한다. 제안한 펄스 주파수 변조 기법은 강압형 다이오드 정류기의 출력전압과 넓은 부하 범위에 대한 주 스위치의 영전류 스위칭을 보장하기 위해 적용된다. 본 논문에서 적용된 펄스 주파수 변조 기법은 일반적으로 입력전류의 낮은 고조파의 단위 역률의 장점을 지니고 있다. 또한 출력전압에서 보여진 저주파 리플전압을 감소시키기 위해 효과적으로 사용되어진다. 제안된 제어기법을 자세하게 설명하며 그 타당성을 검증하기 위해 시뮬레인션 및 실험을 통하여 검증한다.

• The Korean Journal of Physiology and Pharmacology
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• 제3권2호
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• pp.119-125
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• 1999

Mammalian gastric smooth muscles generate spontaneous rhythmic contractions which are associated with slow oscillatory potentials (slow waves) and spike potentials. Spike potentials are blocked by organic $Ca^{2+}-antagonists,$ indicating that these result from the activation of L-type $Ca^{2+}-channel.$ However, the cellular mechanisms underlying the generation of slow wave remain unclear. Slow waves are insensitive to $Ca^{2+}-antagonists$ but are blocked by metabolic inhibitors or low temperature. Recently it has been suggested that Interstitial Cells of Cajal (ICC) serve as pacemaker cells and a slow wave reflects the coordinated behavior of both ICC and smooth muscle cells. Small segments of circular smooth muscle isolated from antrum of the guinea-pig stomach generated two types of electrical events; irregular small amplitude (1 to 7 mV) of transient depolarization and larger amplitude (20 to 30 mV) of slow depolarization (regenerative potential). Transient depolarization occurred irregularly and membrane depolarization increased their frequency. Regenerative potentials were generated rhythmically and appeared to result from summed transient depolarizations. Spike potentials, sensitive to nifedipine, were generated on the peaks of regenerative potentials. Depolarization of the membrane evoked regenerative potentials with long latencies (1 to 2 s). These potentials had long partial refractory periods (15 to 20 s). They were inhibited by low concentrations of caffeine, perhaps reflecting either depletion of $Ca^{2+}$ from SR or inhibition of InsP3 receptors, by buffering $Ca^{2+}$ to low levels with BAPTA or by depleting $Ca^{2+}$ from SR with CPA. They persisted in the presence of $Ca^{2+}-sensitive$ $Cl^--channel$ blockers, niflumic acid and DIDS or $Co^{2+},$ a non selective $Ca^{2+}-channel$ blocker. These results suggest that spontaneous activity of gastric smooth muscle results from $Ca^{2+}$ release from SR, followed by activation of $Ca^{2+}-dependent$ ion channels other than $Cl^-$ channels, with the release of $Ca^{2+}$ from SR being triggered by membrane depolarization.

• 생명과학회지
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• 제20권3호
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• pp.453-456
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• 2010

백혈병은 조혈모세포의 비정상적인 증식에 의해 일어나서 질환이고, adenosine deaminase (ADA) 유전자는 백혈병의 약물 작용점으로 중요하다. 이러한 연구의 일환으로 한국인 백혈병 환자 20명의 ADA 유전자의 변이를 조사하기 위해 혈액 genomice DNA를 추출하여 염기서열을 결정하였다. 그 결과 nonsense 변이인 F101F 하나, missense 변이 E260K, D8Y 각각 하나, 그리고 외국에서는 보고되지 않은 것으로 정상인에서 IVS6-52 에 GC가 도입된 것을 확인하였다. 백혈병 환자와 유전자 변이간에 통계학적인 차이점은 없지만 이러한 연구는 앞으로 백혈병의 진단 마크 개발에 도움이 될 것으로 사료된다.

• The Korean Journal of Physiology and Pharmacology
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• 제2권3호
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• pp.353-359
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• 1998

In gastrointestinal smooth muscle, muscarinic stimulation by carbachol (CCh) activates nonselective cation channel current ($I_{CCh}$) which is facilitated by intracellular [$Ca^{2+}$] increase. Caffeine is widely used in experiments to mobilize $Ca^{2+}$ from intracellular stores. This study shows a strong inhibitory effect of caffeine on $I_{CCh}$ in guinea-pig gastric myocyte. In this study, the underlying mechanism of the inhibitory effect of caffeine was investigated. $I_{CCh}$ was completely suppressed by the addition of caffeine (10 mM) to the superfusing solution. Inhibition of $I_{CCh}$ by caffeine was not related to the intracellular cAMP accumulation which was expected from the phosphodiesterase-inhibiting effect of caffeine. The blockade of $InsP_3-induced$ $Ca^{2+}$ release by heparin had no significant effects on the activation of $I_{CCh}$. When the same cationic current had been induced by intracellular dialysis of $GTP[{\gamma}S]$ in order to bypass the muscarinic receptor, the inhibitory effect of caffeine was significantly attenuated. The results of this study indicate that both intracellular signalling pathways for $I_{CCh}$, proximal and distal to G-protein activation, are suppressed by caffeine. A major inhibition was observed at the proximal level.

• Molecules and Cells
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• 제22권2호
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• pp.210-219
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• 2006

Dynamin-related protein 2A (AtDRP2A, formally ADL6), a member of the dynamin family, is critical for protein trafficking from the TGN to the central vacuole. However, the mechanism controlling its activity is not well understood in plant cells. We isolated Arabidopsis sec13 homolog1 (AtSeh1) that interacts with AtDRP2A by a yeast two-hybrid screening. AtSeh1 has four WD40 motifs and amino acid sequence homology to Sec13, a component of COPII vesicles. Coimmunoprecipitation and protein pull-down experiments demonstrated specific interaction between AtSeh1 and AtDRP2A. AtSeh1 bound to the pleckstrin homology domain of AtDRP2A in competition with the C-terminal domain of the latter, and this resulted in inhibition of the interaction between AtDRP2A and PtdIns3P in vitro. AtSeh1 localized to multiple locations: the nucleus, the prevacuolar compartment and the Golgi complex. Based on these results we propose that AtSeh1 plays a role in regulating cycling of AtDRP2A between membrane-bound and soluble forms.