• BMB Reports
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• 제28권1호
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• pp.40-45
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• 1995

Acetolactate synthase purified from Serratia marcescens ATCC 25419 was rapidly inactivated by the thiol specific reagent p-chloromercuribenzoate (PCMB), the tryptophan specific reagent N-bromosuccinimide (NBS), and the arginine modifying reagent phenylglyoxal (PGO). Inactivation by PCMB was prevented by both ${\alpha}$-ketobutyrate and pyruvate, and the second order rate constant for the inactivation was $2480\;M^{-1}{\cdot}min^{-1}$. The reaction order with respect to PCMB was 0.94. The inactivation of the enzyme by NBS was also substantially reduced by both ${\alpha}$-ketobutyrate and pyruvate. The second order rate constant for inactivation by NBS was $15,000\;M^{-1}{\cdot}min^{-1}$, and the reaction order was 2.0. On the other hand, inactivation by PGO was partially prevented by ${\alpha}$-ketobutyrate, but not by pyruvate. The second order rate constant for the inactivation was $1480\;M^{-1}{\cdot}min^{-1}$ and the order of reaction with respect to PGO was 0.75. These results suggest that essential cysteine, tryptophan and arginine are located at or near the substrate binding site.

• 대한유전성대사질환학회지
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• 제22권1호
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• pp.1-8
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• 2022

Long-chain fatty acid oxidation disorders (LC-FAOD) are an autosomal recessive inherited rare disease group that result in an acute metabolic crisis and chronic energy deficiency owing to the deficiency in an enzyme that converts long-chain fatty acids into energy. LC-FAOD includes carnitine palmitoyltransferase type 1 (CPT1), carnitine-acylcarnitine translocase (CACT), carnitine palmitoyltransferase type 2 (CPT2), very long-chain acyl-CoA dehydrogenase (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), and trifunctional protein (TFP) deficiencies. Common symptoms of LC-FAOD are hypoketotic hypoglycemia, cardiomyopathy, and myopathy. Depending on symptom onset, the disease can be divided as neonatal period, late infancy and early childhood, adolescence, or adult onset, but symptoms can appear at any time. The neonatal screening test (NBS) can be used to identify the characteristic plasma acylcarnitine profiles for each disease and confirmed by deficient enzyme analysis or molecular testing. Before introduction of NBS, the mortality rate of LC-FAOD was very high. With NBS implementation as routine neonatal care, the mortality rate was dramatically decreased, but severe symptoms such as rhabdomyolysis recur frequently and affect the quality of life. Triheptanoin (Dojolvi^®), the first drug for pediatric and adult patients with molecularly confirmed LC-FAOD, has recently been approved by the US Food and Drug Administration in 2020. In this review, the diagnosis of LC-FAOD and treatment including triheptanoin are summarized.

• 한국방사선학회논문지
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• 제17권1호
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• pp.149-156
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• 2023

보리의 생산년도에 따른 생장과 광합성 차이를 알아보고자 질병의 진단 및 치료에 가장 많이 이용하는 고에너지 X-선을 묵은보리씨앗과 햇보리씨앗에 조사하여 연구를 수행하였다. 묵은보리와 햇보리 종자가 싹이 튼 것을 확인 후, 대조군을 제외한 실험군에 대해 방사선종양학과의 선형가속기(Clinac IS, VERIAN, USA, 2011)를 이용하여 30 Gy X-선을 조사하였다. 조사 조건은 6 MV X-ray, 조사거리(Source-surface distance) 100 cm, 조사면적(field size) 18 × 10 cm², 선량률(dose rate) 600 MU/min 에서 실시하였다. 보리의 생장은 종자별로 X-선 조사 후 4일차 부터 1일 간격으로 9일차 까지 줄기의 길이를 측정하였다. X-선이 보리 싹의 엽록소 형성에 미치는 영향을 알아보기 위하여 분광광도법을 이용하여 클로로필 a 농도를 측정하였다. 통계분석은 SPSS ver 26.0(Chicago, IL, USA)을 사용하여 독립표본 T-검정(Independent T-test)을 실시하였다. 대조군에서는 햇보리가 묵은보리 보다 성장이 빨랐지만, 실험군에서는 묵은보리가 햇보리 보다 빠른 성장을 보였다. 묵은보리에서 대조군의 길이가 실험군 보다 모든 일차에서 유의하게 큰 것으로 나타났다. 대조군에서는 묵은보리 보다 햇보리의 무게가 무거웠지만, 실험군에서는 묵은보리의 무게가 햇보리 무게 보다 높았다. 클로로필 농도를 측정하여 비교한 결과, 대조군 실험군 모두 묵은보리 보다 햇보리가 높은 결과를 보였고, 대조군 보다 실험군이 높게 나타났다. X-선은 햇보리 보다 묵은보리의 성장과 무게를 촉진시켰고, 클로로필 농도에는 햇보리에게 더 큰 영향을 미치는 것으로 나타났다. 이와 같은 결과는 향후 X-선이 새싹보리에 미치는 영향에 대한 연구의 기초 자료로 사용될 수 있을 것이다.

• Bulletin of the Korean Chemical Society
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• 제22권1호
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• pp.68-76
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• 2001

We have employed chemical modification to identify amino acids essential for the catalytic activity of alliinase (EC 4.4.1.4) from garlic (Allium sativum). Alliinase degrades S-alkyl-L cysteine sulfoxides, causing the characteristic odor of garlic. The activity of alliinase was rapidly and completely inactivated by N-bromosuccinimide(NBS) and slightly decreased by succinic anhydride and N-acetylimidazole. These results indicate that tryptophanyl, lysyl, and tyrosyl residues play an important role in enzyme catalysis. The reaction of alliinase with NBA yielded a characteristic decrease in both the absorbance at 280 nm and the intrinsic fluorescence at 332 nm with increasing reagent concentration of NBS, consistent with the oxidation of tryptophan residues. Kinetic analysis, fluorometric titration of tryptophans and correlation to residual alliinase activity showed that modification of only one residue present on alliinase led to complete inhibition of alliinase activity. To identify this essential tryptophan residue, we employed chemical modification by NBS in the presence and absence of the protecting substrate analogue, S-ethyl-L-cysteine (SEC) and N-terminal sequence analysis of peptide fragment isolated by reverse phase-HPLC. A fragment containing residues 179-188 was isolated. We conclude that Trp182 is essential for alliinase activity.

• Asian Pacific Journal of Cancer Prevention
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• 제13권3호
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• pp.851-856
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• 2012

Objective: NBS1 plays a key role in the repair of DNA double-strand break (DSB). We conducted this study to investigate the effect of two critical polymorphisms (rs1805794 and rs13312840) in NBS1 on treatment response and prognosis of advanced non-small cell lung cancer (NSCLC) patients with platinum-based chemotherapy. Methods: Using TaqMan methods, we genotyped the two polymorphisms in 147 NSCLC patients. Odds ratios (ORs) and their 95% confidential intervals (CIs) were calculated as a measure of difference in the response rate of platinum-based chemotherapy using logistic regression analysis. The Kaplan-Meier and log-rank tests were used to assess the differences in progression-free survival (PFS) and overall survival (OS). Cox proportional hazards model was applied to assess the hazard ratios (HRs) for PFS and OS. Results: Neither of the two polymorphisms was significantly associated with treatment response of platinum-based chemotherapy. However, patients carrying the rs1805794 CC variant genotype had a significantly improved PFS compared to those with GG genotype (16.0 vs. 8.0 months, P = 0.040). Multivariable cox regression analysis further showed that rs1805974 was a significantly favorable prognostic factor for PFS [CC/CG vs. GG: Adjusted HR = 0.62, 95% CI: 0.39-0.99; CC vs. CG/GG: Adjusted HR = 0.56, 95% CI: 0.32-0.97). Similarly, rs13312840 with a small sample size also showed a significant association with PFS (CC vs. CT/TT: Adjusted HR = 25.62, 95% CI: 1.53-428.39). Conclusions: Our findings suggest that NBS1 polymorphisms may be genetic biomarkers for NSCLC prognosis especially PFS with platinum-based chemotherapy in the Chinese population.

• 자원환경지질
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• 제42권5호
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• pp.501-507
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• 2009

본 연구에서는 단일 벽(Single-wall) 구조를 갖는 나노크기의 속이 빈(hollow) 볼(Ball)형(NBs; Nano-balls), 짧은 길이 튜브(S-NTs; Short-fiber Nano-tubes)형 및 긴 길이 튜브(L-NTs; Long-fiber Nano-tubes) 물질을 동일한 조성 Al/Ge=1.33을 갖는 Aluminogermanate(ALGE) Precursor 최초 혼합용액의 단순한 pH 조절 과정에서 자기조합(Self-assembly)을 통해 성공적으로 합성하였다. 먼저 pH=13으로의 염기성화(Na/Al=28~30) 및 최종 pH=9 조건에서 5 nm의 균질한 크기를 갖으며, 화학조성인 Al/Ge=1.5~1.6인 속이 빈 구조의 NBs가 합성되었다. pH=13으로의 염기성화 및 최종 pH=4 조건에서는 3.3 nm 지름, 길이 15~20 nm, Al/Ge=2.6~2.8의 S-NTs가 합성되었다. 그러나 pH=9로 염기성화(Na/Al=3.8) 시킨 후 최종 pH=4조건에서는 나노-튜브의 길이가 100 nm 이상인 L-NTs가 처음으로 합성되었다. 이러한 결과는 ALGE Precursor로부터 -Ge-OH 표면에서의 $H^+$-dissociation 정도 차이, 즉, $Na^+$ 양 및 최종 pH 조건을 달리함으로써 의도하는 형태의 ALGE 나노물질 유도가 가능함을 시사한다.

• Structural Engineering and Mechanics
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• 제49권1호
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• pp.81-93
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• 2014

Corrosion is one of the primary reasons why structures have limited durability. The present investigation is carried out to study the behavior of RC (Reinforced Concrete) structural members subjected to corrosion. Experimental investigations were carried out on National Bureau of Standard (NBS), RC beam specimens made of Ordinary Portland Cement (OPC) concrete. Load versus deflection behaviour was studied for different levels of corrosion i.e., 2.5%, 5%, 7.5% and 10%. It is observed that for every percentage increase in corrosion level, there is about 1.6% decrease in load carrying capacity. Also as the amount of corrosion increases there is a reduction in bond stress.

• 대한유전성대사질환학회지
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• 제18권2호
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• pp.35-42
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• 2018

목적: 한국 유전학 연구소에서 실시한 광범위 신생아 스크리닝 검사(Newborn screening, NBS)로 진단된 선천성 대사질환 및 내분비질환을 가진 한국인 환아의 추적 관찰 및 장기적인 예후를 평가하기 위하여 본 연구를 시작하였다. 방법: 2000년 1월부터 2017년 12월까지 태어난 283,626명의 신생아를 대상으로 하였으며 출생 48시간 이후에 발뒤꿈치, 혹은 정맥혈액을 채취하여 특수여과지에 묻혀 건조시켰다. 건조 혈액여지를(Dried blood spot, DBS) 이용하여 탠덤 질량 분석법과 형광 면역 측정법을 사용하여 광범위 신생아 스크리닝 검사(NBS)를 실시하였다. 신생아 스크리닝 선별검사 프로그램은 갈락토오스 혈증, 선천성 갑상선 기능 저하(Congenital hypothyroidism, CH), 선천성 부신 과형성증(Congenital adrenal hyperplasia, CAH), 아미노산, 지방산 및 유기산 대사질환등 예방 가능한 질환 50여종을 선별하여 검사를 시행하였다. 결과: 광범위 신생아 스크리닝 검사(Extended NBS)를 통해 아미노산 대사질환 28예, 유기산 대사질환 75예, 지방산 대사질환 27예, 요소회로 대사질환 51예, CH 127예, CAH 14예, 갈락토스혈증 15예가 선별하여 확진검사로 진단되었다. 아미노산 대사 장애, 갈락토스혈증, CH, CAH 환자는 조기에 발견 치료 할 경우 예후가 더 좋았다. 단풍당뇨(MSUD) 환아에서는 조기 진단 치료로 90% 이상이 정상 성장 발달을 보였다. 그러나 유기산 혈증 환아에서는 32%에서 발달 지연 및 신경학적 휴유증이 관찰되었다. 지방산 대사 질환에서는 다양한 결과가 나타났다. 단쇄지방산(SCAD, EMA)와 중쇄지방산(MCA, MCAD) 환자는 예후가 좋았으나 초장쇄지방산(VLCAD) 환자는 대부분 심각한 신경학적 장애를 보이거나 사망하였다. 요소회로 대사질환(UCD) 환아는 조기진단과 치료에도 불구하고 75%가 심각한 신경학적 합병증과 높은 사망률을 경험했다. 결론: 전국적인 신생아 스크리닝(NBS) 프로그램은 국가적인 차원에서 전국민을 대상으로 포괄적인 검사, 관리, 치료가 필요하다. 이를 위하여 숙련된 의료진과 환아의 부모 혹은 관련된 가족에 대한 특수교육이 필요하다.

• IEIE Transactions on Smart Processing and Computing
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• 제1권1호
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• pp.50-64
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• 2012

Quality of Service (QoS) and fairness considerations are undoubtedly essential parameters that need to be considered in the design of next generation scheduling algorithms. This work presents a novel game theoretic cross-layer design that offers optimal allocation of wireless resources to heterogeneous services in Orthogonal Frequency Division Multiple Access (OFDMA) networks. The method is based on the Axioms of the Symmetric Nash Bargaining Solution (S-NBS) concept used in cooperative game theory that provides Pareto optimality and symmetrically fair resource distribution. The proposed strategies are determined via convex optimization based on a new solution methodology and by the transformation of the subcarrier indexes by means of time-sharing. Simulation comparisons to relevant schemes in the literature show that the proposed design can be successfully employed to typify ideal resource allocation for next-generation broadband wireless systems by providing enhanced performance in terms of queuing delay, fairness provisions, QoS support, and power consumption, as well as a comparable total throughput.

• BMB Reports
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• 제31권3호
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• pp.258-263
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• 1998

Acetolactate synthase (ALS) is the common enzyme in the biosynthetic pathways leading to leucine, valine, and isoleucine. Tobacco ALS was expressed in E. coli and purified to homogeneity. The recombinant tobacco ALS was inactivated by thiol-specific reagents, N-ethylmaleimide (NEM) and 5,5'-dithio-bis-(2-nitrobenzoic acid) (DTNB). Inactivation of the ALS by NEM followed pseudo-first order kinetics and was first order with respect to the modifier. The substrate pyruvate protected the enzyme against the inactivation by NEM and DTNB. Extrapolation to complete inactivation of the enzyme by DTNB showed modification of approximately 2 out of 4 total cysteinyl residues (or 2 cysteinyl and 1 cysteinyl residues), with approximately 1 residue protected by pyruvate. The tobacco ALS was also inactivated by the tryptophanspecific reagent, N-bromosuccinimide (NBS), and was similarly protected by pyruvate. The kinetics of the inactivation was first-order with respect to NBS. The present data suggest that cysteinyl and tryptophanyl residues play a key role in the catalytic function of the enzyme.