• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.2
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• pp.219-222
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• 2008

Crigler-Najjar syndrome is a rare inherited disease associated with unconjugated hyperbilirubinemia. It is inherited via an autosomal recessive pattern and is caused by mutation in one of the five exons of the bilirubin uridine-diphosphoglucuronate glucuronosyltransferase (UGT1A1) gene. The synthesis of inactive isoforms of bilirubin uridine-diphosphoglucuronate glucuronosyltransferase (B-UGT) results in unconjugated hyperbilirubinemia. A 13-year-old boy with jaundice for 4 months was admitted to our hospital. He had unconjugated hyperbilirubinemia with no evidence of infection, hemolysis, or structural abnormalities on abdominal ultrasonography or 99mTc-DISIDA scan. The authors identified a missense mutation of Tyr486Asp in the fifth exon of the UGT1A1 gene and diagnosed the patient with Crigler-Najjar syndrome type II. This is the first reported case of Crigler-Najjar syndrome in a Korean child, and it is also the first reported case of a genetic mutation leading to Crigler-Najjar syndrome in Korea.

• Korean Chemical Engineering Research
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• v.44 no.1
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• pp.92-96
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• 2006

Enzymes in organic media afford many advantages such as chiral synthesis and resolution, modification of fats and oils and production of biodegradable polymers. However, the nature of solvents influences the activity and stability of enzymes, and the presence of organic solvents always constitute a risk of enzyme inactivation. Heat-shock protein Hsp90, one of the molecular chaperone, was applied for understanding of enzyme inactivation and for increasing of enzyme stability in water-miscible organic solvent. Hsp90 showed stabilization effect on HRP in the 30％ of DMSO, in the 30％ and 50％ of dioxane. Hsp90 also showed reactivation effect on the inactivated HRP by water-miscible organic solvent such as dioxane and DMSO. In addition, structural analysis using fluorescence spectrophotometry and circular dichroism showed that exposure of HRP in water-miscible organic solvent caused appreciable conformational changes and enzyme inactivation, and the unfolded HRP by water-miscible organic solvent was refolded by Hsp90.

• Journal of the Korean institute of surface engineering
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• v.46 no.4
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• pp.139-144
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• 2013

c-BN(cubic boron nitride) is known to have extremely high hardness next to diamond, as well as very high thermal and chemical stability. The c-BN in the form of film is useful for wear resistant coatings where the application of diamond film is restricted. However, there is less practical application because of difficult control of processing variables for synthesis of c-BN film as well as unclear mechanism on formation of c-BN. Therefore, in the present study, the structural characterization of c-BN thin film were investigated using $B_4C$ target in r.f. magnetron sputtering system as a function of processing variables. c-BN films were coated on Si(100) substrate using $B_4C$ (99.5% purity). The mixture of nitrogen and argon was used for carrier gas. The deposition processing conditions were changed with substrate bias voltage, substrate temperature and base pressure. Fourier transform infrared microscopy (FT-IR) and X-ray photoelectron spectroscopy (XPS) were used to analyze crystal structures and chemical binding energy of the films. In the case of the BN film deposited at room temperature, c-BN was formed in the substrate bias voltage range of -400 V~ -600 V. Less c-BN fraction was observed as deposition temperature increased and more c-BN fraction was observed as base pressure increased.

• Microbiology and Biotechnology Letters
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• v.26 no.1
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• pp.34-39
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• 1998

We have already prepared a human lysozyme (HLY) structural gene from chemically synthesized 38 oligomers with high codon usage in Saccharomyces cerevisiae. For directing the synthesis and secretion of HLY in S. cerevisiae, two types of expression vectors, a YCp centromere-based vector, pHK101 and a YEp 2-$\mu\textrm{m}$ circle-based vector, pHK501 were constructed. With the resulting plasmids, we have confirmed that yeast transformant harboring pHK501 has more secreted HLY than pHK101-transformant by using a lysoplate and a turbidimetric assay. In flask cultivation, pHK501-transformant produced active HLY about 8 times (55 units/$m\ell$) higher than pHK101-transformant. From batch cultivation, the HLY productivity was obtained with 1.12 units/$m\ell$/h, corresponding to a 1.8-fold increase compared with flask fermentation. These results indicate that yeast transformant with pHK501 vector overexpressed and secreted HLY than that of YCp type vector.

• Proceedings of the Korean Society of Applied Pharmacology
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• 1996.11a
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• pp.65-73
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• 1996

LPS/LOS, the compound found only in gram-negative bacterial outer membrane, plays important roles in bacterial maintenance as well as its pathogenesis. We isolated and characterized several genes required for NTHi 2019 LOS biosynthesis, which encode enzymes required for sugar substrate synthesis or the transfer of substrates to receptor molecules. The htrB gene, however, appears to have more complex role. It has acryltransferase activity as well as various other activity, which may control regulation of LOS biosynthesis as well as its pathogenicity. Evidences supporting the latter come from the observations that the lipid A of the B29 induced significantly less TNF ${\alpha}$ from macrophages than that of the wild type LOS (unpublished data). H. influenzae A2-htrB mutant strain was also significantly less invasive than the wild type strain. The structural similarities of the enterobacterial LPS and the Haemophilus LOS enabled us to isolate the NTHi 2019 genes involved in LOS biosynthesis genes by using the S. typhimurium LPS deep core mutants. While a similar approach has been used for E. coli, this technique for selection of an LPS phenotype has not been applied to nonenterobacterial species. The difficulties inherent in the molecular manipulation of organism such as Neisseria and Haemophilus species make this approach particularly attractive in the identification and cloning LOS genes. Studies on genetic features of LPS/LOS biosynthesis would be useful for understanding bacterial pathogenesis as well as for developing vaccines for these gram-negative pathogenic bacteria.

• Journal of Genetic Medicine
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• v.13 no.1
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• pp.41-45
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• 2016

Marfan syndrome (MFS) is an inherited connective tissue disorder with a mutation in the fibrillin-1 (FBN1) gene. Fibrillin is a major building block of microfibrils, which constitute the structural component of the connective tissues. A 10-year-old girl visited our hospital with the chief complaint of precocious puberty. According to her medical history, she had a pulmonary wedge resection for a pneumothorax at 9 years of age. There was no family history of MFS. Mid parental height was 161.5 cm. The patient's height was 162 cm (>97th percentile), and her weight was 40 kg (75th-90th percentile). At the time of initial presentation, her bone age was approximately 11 years. From the ophthalmologic examination, there were no abnormal findings except myopia. There was no wrist sign. At the age of 14 years, she revisited the hospital with the chief complaint of scoliosis. Her height and weight were 170 cm and 50 kg, respectively, and she had arachnodactyly and wrist sign. We performed an echocardiograph and a test for the FBN1 gene mutation with direct sequencing of 65 coding exons, suspecting MFS. There were no cardiac abnormalities including mitral valve prolapse. A cytosine residue deletion in exon 7 (c.660delC) was detected. This is a novel mutation causing a frameshift in protein synthesis and predicted to create a premature stop codon. We report the case of a patient with MFS with a novel FBN1 gene missense mutation and a history of pneumothorax at a young age without cardiac abnormalities during her teenage years.

• Applied Chemistry for Engineering
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• v.8 no.2
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• pp.237-245
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• 1997

A DEAE-chitin was prepared with DEAE HCl in an aqueous alkali-chitin solution. The resulting DEAE-chitin exhibited a highly improved affinity to water and organic solvents, It was N-deacetylated by heating in aqueous 10% sodium hydroxide containing sodium borohydride for 9h at $80^{\circ}C$ to produce DEAE-chitosan. These conditions were milder than those for the N-deacetylation of chitin. In order to increase its cationic character, the DEAE-chitin was treated with ethyl halide to give TEAE-chitin. The structural changes in the chitin derivatives were confirmed by using both FT-IR and $^1H$ NMR, and their flocculating behavior, in kaoline suspension showed the optimum property at a weak alkaline pH and 8 ppm concentration of resin conditions.

• Applied Chemistry for Engineering
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• v.21 no.5
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• pp.564-569
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• 2010

Highly anti-reflective optical property has been focussed in the field of thin film and display because of increasing demands to the high transparency and clearness of optical component. In this study, to obtain anti-reflective property, the formation of aluminium oxide with nanoscaled flowerlike frame structure was introduced as oxide material monolayer on the substrate by hydrothermal synthesis through sol-gel method. The properties of coating layer were measured by the means of UV-Vis spectroscopy, FT-IR spectroscopy, XRD, and FE-SEM. The morphology of coating layer in alumina-sol coated samples was controlled by hydrothermal temperature and time with aid of ultrasound. It was found that high transparency and anti-reflective optical properties were obtained the formation of flowerlike nanoframe structure.

• Korean Journal of Weed Science
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• v.18 no.3
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• pp.246-256
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• 1998

The objective of this study was to investigate the cause of anthocyanin accumulation in the corn leaves treated with metsulfuron-methyl. The accumulation of anthocyanin and total sugar was increased with the time after metsulfuron-methyl application and with the greater herbicide concentration as well. The anthocyanin increase was alleviated by the combined treatment of DUMU or the tank-mixture treatment of metsulfuron-methyl, isoleucine and valine. Metsulfuron-methyl could not enhance the anthocyanin formation in the nonchlorophyllous tissue in which photosynthetic carbohydrate production was limited. Upon the exogenous supply of sucrose, however, the contents of anthocyanin was increased in the both chlorophyllous and nonchlorophyllous leaf segment of corn without the herbicide treatment. On the other hand, the herbicide itself did not influence Hill reaction in vitro and photosynthesis electron transport in vivo. The anthocyanin accumulation by metsulfitronmethyl did not occur in the corn mutants deficient in the structural gene of anthocyanin synthesis, but corns deficient in the regulatory gene had the anthocyanin accumulation only in R-r and r-r type. The above results suggest that the purple pigmentation in the corn leaves treated with metsulfuron-methyl is related to the accumulation of photosynthetic carbohydrate which can stimulate the. regulatory gene related to anthocyanin biosynthetic pathway.

• Proceedings of the Korean Vacuum Society Conference
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• 2012.02a
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• pp.354-354
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• 2012

In this study, we report the vertically aligned ZnO nanowires by using different type of Ga-doped ZnO (GZO) thin films as seed layers to investigate how the underlying GZO film micro structure affects the distribution of ZnO nanowires. Arrays of highly ordered ZnO nanowires have been synthesized on GZO thin film seed layer prepared on p-Si substrates ($7-13{\Omega}cm$) with utilize of a pulsed laser deposition (PLD). With the vapor-liquid-solid (VLS) growth process, the ZnO nanowire synthesis carries out no metal catalyst and is cost-effective; furthermore, The GZO seed layer facilitates the uniform growth of well-aligned ZnO nanowires. The influence of the growth temperature and various thickness of GZO seed layer have been analyzed. Crystallinity of grown seed layer was studied by X-Ray diffraction (XRD); diameter and morphology of ZnO nanowires on seed layer were investigated by field emission scanning electron microscopy (FE-SEM). Our results suggest that the GZO seed layer with high c-axis orientation, good crystallinity, and less lattice mismatch is key parameters to optimize the growth of well-aligned ZnO nanowire arrays.