• International Journal of Oncology
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• v.56 no.1
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• pp.368-378
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• 2020

Meridianin C is a marine natural product with anticancer activity. Several meridianin C derivatives (compounds 7a-j) were recently synthesized, and their inhibitory effects on pro-viral integration site for Moloney murine leukemia virus (PIM) kinases, as well as their antiproliferative effects on human leukemia cells, were reported. However, the anti-leukemic effects and mechanisms of action of meridianin C and its derivatives remain largely unknown. The aim of the present study was to investigate the effects of meridianin C and its derivatives on MV4-11 human acute myeloid leukemia cell growth. The parent compound meridianin C did not markedly affect the viability and survival of MV4-11 cells. By contrast, MV4-11 cell viability and survival were reduced by meridianin C derivatives, with compound 7a achieving the most prominent reduction. Compound 7a notably inhibited the expression and activity of PIM kinases, as evidenced by reduced B-cell lymphoma-2 (Bcl-2)-associated death promoter phosphorylation at Ser112. However, meridianin C also suppressed PIM kinase expression and activity, and the pan-PIM kinase inhibitor AZD1208 only slightly suppressed the survival of MV4-11 cells. Thus, the anti-survival effect of compound 7a on MV4-11 cells was unrelated to PIM kinase inhibition. Moreover, compound 7a induced apoptosis, caspase-9 and -3 activation and poly(ADP-ribose) polymerase (PARP) cleavage, but did not affect death receptor (DR)-4 or DR-5 expression in MV4-11 cells. Compound 7a also induced the generation of cleaved Bcl-2, and the downregulation of myeloid cell leukemia (Mcl)-1 and X-linked inhibitor of apoptosis (XIAP) in MV4-11 cells. Furthermore, compound 7a increased eukaryotic initiation factor (eIF)-2α phosphorylation and decreased S6 phosphorylation, whereas GRP-78 expression was unaffected. Importantly, treatment with a pan-caspase inhibitor (z-VAD-fmk) significantly attenuated compound 7a-induced apoptosis, caspase-9 and -3 activation, PARP cleavage, generation of cleaved Bcl-2 and downregulation of Mcl-1 and XIAP in MV4-11 cells. Collectively, these findings demonstrated the strong anti-survival and pro-apoptotic effects of compound 7a on MV4-11 cells through regulation of caspase-9 and -3, Bcl-2, Mcl-1, XIAP, eIF-2α and S6 molecules.

• Neonatal Medicine
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• v.28 no.2
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• pp.89-93
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• 2021

Nemaline myopathy is a genetically heterogeneous neuromuscular disorder and one of the most common congenital myopathies. The clinical manifestations usually vary depending on the age of onset. Neonatal nemaline myopathy has the worst prognosis, primarily due to respiratory failure. Several genes associated with nemaline myopathy have been identified, including NEB, ACTA1, TPM3, TPM2, TNNT1, CFL2, KBTBD13, KLHL40, KLHL41, LMOD3, and KBTBD13. Here, we report a neonatal Korean female patient with nemaline myopathy carrying compound heterozygous mutations in the gene KLHL40 as revealed using next generation sequencing (NGS). The patient presented with postnatal cyanosis, respiratory failure, dysphagia, and hypotonia just after birth. To identify the genetic cause underlying the neonatal myopathy, NGS-based gene panel sequencing was performed. Compound heterozygous pathogenic variants were detected in KLHL40: c.[1405G>T];[1582G>A] (p. [Gly469cys];[Glu528Lys]). NGS allows quick and accurate diagnosis at a lower cost compared to traditional serial single gene sequencing, which is greatly advantageous in genetically heterogeneous disorders such as myopathies. Rapid diagnosis will facilitate efficient and timely genetic counseling, prediction of disease prognosis, and establishment of treatments.

• Journal of Advanced Marine Engineering and Technology
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• v.28 no.2
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• pp.292-299
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• 2004

This study is intended to check a temperature of the flame to raise by burning A heavy oil in a boiler. to measure the concentration of DS and SOF after collecting the PM(Particulate Matters). and to analyze the components ingredients of SOF by G.C Mass for presupposing the generation of particulate matters(soot). It is thought that the methyl(CH3) of the cyclic compound is changed to the materials of 2 cycles and 3 cycles after becoming CH by dehydrogenation and also mixing with the CH of a chain compound. form H-$\cdot$C=C$\cdot$-H that is mentioned before. in order to become Polycyclic Aromatic Hydrocarbon.

• Journal of Genetic Medicine
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• v.17 no.2
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• pp.97-101
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• 2020

The combination of central nervous system abnormalities and renal impairment is a notable characteristic of Galloway-Mowat syndrome (GAMOS), a disease which often accompanies microcephaly, developmental delay, and nephrotic syndrome. Many subtypes exist having various phenotypes and genotypes, and many genetic causes are still being identified. An 18-month-old boy first visited our clinic for seizure, delayed development, and microcephaly. During follow-up visits he developed proteinuria and nephrotic syndrome at the age of 6. Nephrotic syndrome became refractory to treatment. These phenotypes were suggestive of GAMOS. Next generation sequencing was performed for genetic analysis and revealed novel compound heterozygous variants in the WDR4 gene: c.494G>A (p.Arg165Gln) and c.540C>G (p.Ile180Met). This is the first case in Korea of GAMOS involving the WDR4 gene.

• Journal of Genetic Medicine
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• v.15 no.2
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• pp.110-114
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• 2018

Ataxia-telangiectasia (AT; OMIM 208900) is a rare autosomal recessive inherited progressive neurodegenerative disorder, with onset in early childhood. AT is caused by homozygous or compound heterozygous mutations in ATM (OMIM 607585) on chromosome 11q22. The average prevalence of the disease is estimated at 1 of 100,000 children worldwide. The prevalence of AT in the Republic of Korea is suggested to be extremely low, with only a few cases genetically confirmed thus far. Herein, we report a 5-year-old Korean boy with clinical features such as progressive gait and truncal ataxia, both ankle spasticity, dysarthria, and mild intellectual disability. The patient was identified as a compound heterozygote with two novel genetic variants: a paternally derived c.5288_5289insGA p.(Tyr1763*) nonsense variant and a maternally derived c.8363A>C p.(His2788Pro) missense variant, as revealed by next-generation sequencing and confirmed by Sanger sequencing. Based on claims data from the Health Insurance Review and Assessment Service Republic of Korea, we calculated the prevalence of AT in the Republic of Korea to be about 0.9 per million individuals, which is similar to the worldwide average. Therefore, we suggest that multi-gene panel sequencing including ATM should be considered early diagnosis.

• Development and Reproduction
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• v.27 no.2
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• pp.77-89
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• 2023

Metformin is the most widely used anti-diabetic drug that helps maintain normal blood glucose levels primarily by suppressing hepatic gluconeogenesis in type II diabetic patients. We previously found that metformin induces apoptotic death in H4IIE rat hepatocellular carcinoma cells. Despite its anti-diabetic roles, the effect of metformin on hepatic de novo lipogenesis (DNL) remains unclear. We investigated the effect of metformin on hepatic DNL and apoptotic cell death in H4IIE cells. Metformin treatment stimulated glucose consumption, lactate production, intracellular fat accumulation, and the expressions of lipogenic proteins. It also stimulated apoptosis but reduced autophagic responses. These metformin-induced changes were clearly reversed by compound C, an inhibitor of AMP-activated protein kinase (AMPK). Interestingly, metformin massively increased the production of reactive oxygen species (ROS), which was completely blocked by compound C. Metformin also stimulated the phosphorylation of p38 mitogen-activated protein kinase (p38MAPK). Finally, inhibition of p38MAPK mimicked the effects of compound C, and suppressed the metformin-induced fat accumulation and apoptosis. Taken together, metformin stimulates dysregulated glucose metabolism, intracellular fat accumulation, and apoptosis. Our findings suggest that metformin induces excessive glucose-induced DNL, oxidative stress by ROS generation, activation of AMPK and p38MAPK, suppression of autophagy, and ultimately apoptosis.

• Proceedings of the Korean Society of Soil and Groundwater Environment Conference
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• 2004.04a
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• pp.147-152
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• 2004

Aerobic cometabolism of cis-1,2-dichloroethylene (c-DCE) and c-DCE epoxide by a butane-grown mixed culture was evaluated. Transformation of c-DCE resulted in the concomitant generation of c-DCE epoxide. Chloride release studies showed nearly complete oxidative dechlorination of c-DCE (approximately 75%). Mass spectrometry confirmed tile presence of a compound with mass-to-charge-fragment ratios of 112, 83, 48, and 35. The values are in agreement with the spectra of a chemically synthesized c-DCE epoxide. Some evidences indicating the involvement of the monooxygenase in the transformation of c-DCE epoxide are: 1) $O_2$ requirement for c-DCE transformation and butane degradation; 2) butane inhibition on c-DCE transformation and vice versa; 3) the inactivation of c-DCE and c-DCE epoxide transformations by acetylene (a known monooxygenase inactivator); and 4) tire inhibition of c-DCE epoxide transformation by c-DCE.

• Electrical & Electronic Materials
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• v.7 no.4
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• pp.281-288
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• 1994

According to spread volume of B(BiPbCuO) layer, composition ratio and each stage of sintering process, we studied stability of high Tc superconductor phase and generation and growth movement of superconducting phase. The dual layer composed of SrCaCuO and BiPbCuO compound were prepared to develop the Bi-2223 superconductor[108K] through interaction and diffusion during sintering process. The dual layer samples were sintered at 830.deg. C for 0-210 hours. From the result, the optimum conditions were : spread volume(A:B=1:0.6), sintering time(210h) and composition ratio(A:S $r_{2}$C $a_{2}$C $u_{2}$- $O_{x}$, B:B $i_{1.9}$P $b_{0.5}$C $u_{3}$ $O_{y}$) at 830.deg. C.. C.C.C.

• KOREAN JOURNAL OF CROP SCIENCE
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• v.51 no.2
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• pp.173-178
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• 2006

Anthocyanin in blackish purple rice is composed of cyanidin, peonidin, malvidin, pelargonidin and delphinidin flavylium ion and their glucosides, which shows antioxidant activity similar to a-tocoperol. It has been demonstrated that cyanidin-3-glucoside(C3G) of anthocyanin has a high-potency antioxidant activity, and is a prominent in anticancer and antithrombotic activity. In analysis of content of anthocyanin from the genetic source of blackish purple rice, the level of C3G was approximately 80% of total contents of anthocyanins, and showed the highest content in Heugjinjubyeo, of which contains an amount of 500 mg by weight of 100g brown rice in comparison that most of varieties has less than 50mg by weight of 100g brown rice. We found that C3G pigment in blackish purple rice was considerably affected by cultural season, cultivation area, ripening temperature, and weather condition, etc. as well as genetic properties. In cross combination between Heugjinjubyeo and Suwon425, intermediate parent $F_9$ generation which was three times higher or more in C3G content than that of Heugjinjubyeo was bred, of which it contained an amount of 1678 mg by weight of 100g brown rice, and we called it 'C3GHi' Rice. And, we found that the C3GHi rice was more excellent than that of the existing Heugjinjubyeo in anticancer and antithrombotic activity. Methanol extract from Heugjinjubyeo was fractionated by organic solvents in order of n-hexane, $CH_{2}Cl_2$ and n-BuOH, and then oryzafuran, quercetin, vanillic acid and protocatechuic acid and their structures from the n-BuOH fraction were ascertained. Oryzafuran of these compounds was a natural compound found firstly in nature. Quercetin and protocatechuic acid besides new compound oryzafuran showed excellent antioxidant ability to vitamin C. These results suggest that blackish purple rice has very high value as a source of various functional food as well as staple food.

• Journal of the Microelectronics and Packaging Society
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• v.30 no.4
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• pp.17-31
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• 2023

Amidst escalating global warming fueled by indiscriminate fossil fuel consumption, concerted efforts are underway worldwide to mitigate atmospheric carbon dioxide (CO₂) levels. Electrochemical CO₂ reduction technology is recognized as a promising and environmentally friendly approach to convert CO₂ into valuable hydrocarbon compounds, deemed essential for achieving carbon neutrality. Copper, among the various materials used as CO₂ reduction electrodes, is known as the sole metal capable of generating C₂₊ compounds. However, low conversion efficiency and selectivity have hindered its widespread commercialization. This review highlights diverse research endeavors to address these challenges. It explores various studies focused on utilizing copper-based electrodes for CO₂ reduction, offering insights into potential solutions for advancing this crucial technology.