• Korean Journal of Metals and Materials
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• v.50 no.12
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• pp.955-959
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• 2012

To increase the hydrogen storage capacity of Mg-based materials, a sample with a composition of 69.7 wt% $MgH_2$ + 30.3 wt% $LiBH_4$ was prepared by planetary ball milling under hydrogen. The absorption and desorption properties of unmilled $MgH_2$, unmilled $LiBH_4$, and 69.7 wt% $MgH_2$ + 30.3 wt% $LiBH_4$ were examined. At 648 K the unmilled $MgH_2$ desorbed 5.70 wt% for 60 min. The unmilled $LiBH_4$ desorbed 6.40 wt% H for 780 min at 673 K. The 69.7 wt% $MgH_2$ + 30.3 wt% $LiBH_4$ sample desorbed 3.10 wt% H for 50 min, and 3.32 wt% H for 300 min at 623 K at the second cycle.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.2
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• pp.55-58
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• 2015

Tetrahydrobiopterin ($BH_4$) can normalize blood phenylalanine (Phe) levels in $BH_4$ deficiency, but typically not in phenylketonuria (PKU). In 1999, Kure et al. reported that some PKU patients showed decreased blood Phe levels after $BH_4$ loading, and thereafter, those PKU patients were identified by neonatal PKU screening. A natural cofactor for phenylalanine hydroxylase (PAH) is a 6R-isomer of $BH_4$, which is first synthesized in Japan as Sapropterin dihydrochloride (Biopten$^{(R)}$) in 1982. In Japan, Biopten$^{(R)}$ is first approved for the treatment of $BH_4$ deficiency in 1992, and then for $BH_4$-responsive PAH deficiency (BPKU) in 2008. The discovery of BPKU has vast clinical implications. After Biopten$^{(R)}$ (Kuvan$^{(R)}$) is available for the treatment of BPKU, the QOL of both patients and their families were improved very much, since the serum phenylalanine levels were controlled within 4 mg/dL by $BH_4$ mono-therapy with a normal diet or $BH_4$ combined use of mild phenylalanine-restricted diet. Biopten$^{(R)}$ therapy in patients with BPKU is highly efficacious (70%) at maintaining serum Phe levels within recommended control range and provides excellent safety at least average use period of 10 years (range, 1-17 years) with no unwarranted side effects in Japan. In addition it has been confirmed that sapropterin therapy initiated before 4 years of age was very effective to maintain plasma Phe levels within the favorable range and was safe in Japanese patients with BPKU.

• The Korean Journal of Physiology and Pharmacology
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• v.18 no.2
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• pp.129-134
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• 2014

It has been suggested that transition metal ions such as iron can produce an oxidative injuries to nigrostriatal dopaminergic neurons, like Parkinson's disease (PD) and subsequent compensative increase of tetrahydrobiopterin ($BH_4$) during the disease progression induces the aggravation of dopaminergic neurodegeneration in striatum. It had been established that the direct administration of $BH_4$ into neuron would induce the neuronal toxicity in vitro. To elucidate a role of $BH_4$ in pathogenesis in the PD in vivo, we assessed the changes of dopamine (DA) and $BH_4$ at striatum in unilateral intranigral iron infused PD rat model. The ipsistriatal DA and $BH_4$ levels were significantly increased at 0.5 to 1 d and were continually depleting during 2 to 7 d after intranigral iron infusion. The turnover rate of $BH_4$ was higher than that of DA in early phase. However, the expression level of GTP-cyclohydrolase I mRNA in striatum was steadily increased after iron administration. These results suggest that the accumulation of intranigral iron leads to generation of oxidative stress which damage to dopaminergic neurons and causes increased release of $BH_4$ in the dopaminergic neuron. The degenerating dopaminergic neurons decrease the synthesis and release of both $BH_4$ and DA in vivo that are relevance to the progression of PD. Based on these data, we propose that the increase of $BH_4$ can deteriorate the disease progression in early phase of PD, and the inhibition of $BH_4$ increase could be a strategy for PD treatment.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.3
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• pp.118-126
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• 2015

Background and objectives: A deficiency of BH4 (tetrahydrobiopterin) not only causes the classical phenylketonuric phenotype, but also is the source of neurological signs and symptoms due to impaired syntheses of L-Dopa and serotonin. The treatment of BH4 deficiency usually consists of replacement with BH4 and the neurotransmitters. We performed this study to finding out long-term follow-up clinical symptoms and prognosis of BH4 deficiency. Methods: Clinical and biochemical, genetic analysis were done retrospectively from January 1999 to July 2015 in Soonchunhyang University Hospital. Results: In our study, total 207 patients were confirmed to hyperphenylalaninemia. Among them, 10 patients were BH4 deficiency. 9 patients were 6-pyruvoyl-tetrahydropterin (PTPS) deficiency and one patient was dihydropteridine reductase (DHPR) deficiency. The patients who received delayed treatment, most of our patients suffered from severe psychomotor retardation, hypotonia and seizure. c.259C>T mutation was identified most commonly in PTPS gene analysis. A patient with DHPR deficiency had a mental retardation, dystonia, seizure. His seizure semiology was dialeptic feature. His EEG showed generalized spike wave patterns. All patients had treated with tolerate L-Dopa, BH4 and 5-hydroxytryptophan. Most of the early treated patients have a good tolerance for drugs well. But some patients had neurologic symptoms, despite early detection and treatment. Conclusion: BH4 deficiency patients who had delayed treatment tend to have severe psychomotor problem and neurologic deficits.

• KSBB Journal
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• v.18 no.6
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• pp.455-460
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• 2003

This study was designed to find out the rapid fermentation of soy sauce from koji hydrolyzates using air bubble column reactor packed with coimmobilized mixed culture system. Continuous ripid production was performed by coimmobilized Z. rouxiii BH-90 and C. versatilis BH-91. Coimmobilized cells of Zygosaccharomyces rouxii BH-90 and Candida versatilis BH-91 mixture cells in the column reactor produced 2.8％ ethyl alcohol and 18mg/L 4-ethylguaiacol over 96 hours under the optimal conditions. Coimmobilized cells produced 2.30∼2.4% ethyl alcohol during 30 days, and decreased gradually from 40 days to 70 days. Also coimmobilized cells produced 4-ethylguaiacol at the constant rate of 16∼18mg/L and decreased gradually after 40 days. Final product of soy sauce contained 2.4% ethyl alcohol and 18mg/L 4-ethylguaiacol. However, amino acid compositions of soy sauce were consisted of predominantly glutamic acid, leucin, arginine, aspartic acid, Iysine and valine, which were more than 50% of total amino acid.

• The Korean Journal of Food And Nutrition
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• v.17 no.1
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• pp.72-79
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• 2004

This study was conducted to investigate the identification of lactic acid bacteria produced ν-aminobutyric acid(GABA) from traditional salt fermented anchovy. There was no appreciable difference in the number of lactic acid bacteria from fermented anchovy. Among the types of lactic acid bacteria, three strains of lactic acid bacteria produced ν-aminobutyric acid from those sample were identified temporary as name of Lactobacillus brevis BH-21, Lactobacillus rhamnosus BH-32 and Lactobacillus plantarum BH-38 by using gram positive identification(GPI) card and API 50 kit, respectively. 3 strains of Lactobacillus sp. were found to produce GAB A in the culture of filtrate. Lactobacillus brevis BH-21 produced GABA, some of which yielded 43.2 mg/mL GABA in the medium of 0.1% glucose, 0.1% yeast extract, 0.05% polypeptone, 0.002% MgSO$_4$$.$4H$_2$O, 0.001% FeSO$_4$$.$7H$_2$O, 0.01% NaCl, 0.1% monosodium glutamate, pH 6.0. This result suggests that Lactobacillus brevis BH-21 has the potential to be developed as a strain of GABA production.

• Korean Chemical Engineering Research
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• v.54 no.1
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• pp.11-15
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• 2016

Proton Exchange Membrane Fuel Cells (PEMFC) instead of batteries is appropriate for long time flight of unmanned aero vehicles (UAV). In this work, $NaBH_4$ hydrolysis system supplying hydrogen to PEMFC was studied. In order to decrease weight of $NaBH_4$ hydrolysis system, enhancement of hydrogen yield, recovery of condensing water and maintenance of stable hydrogen yield were studied. The hydrogen yield of 3.4% was increased by controlling of hydrogen pressure in hydrolysis reactor. Condensing water formed during air cooling of hydrogen was recovered into storage tank of $NaBH_4$ solution. In this process the condensing water dissolved $NaBH_4$ powder and then addition of $NaBH_4$ solution decreased system weight of 14%. $NaBH_4$ hydrolysis system was stably operated with hydrogen yield of 96% by 2.0g Co-P-B catalyst for 10 hours at 2.0L/min hydrogen evolution rate.

• BMB Reports
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• v.43 no.9
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• pp.593-598
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• 2010

Tetrahydrobiopterin ($BH_4$) is a pivotal cofactor for enzymes responsible for the synthesis and release of monoamine neurotransmitters including dopamine and serotonin as well as the release of glutamate. Deficiencies in $BH_4$ levels and reduced activities of $BH_4$-associated enzymes have been recently reported in patients with schizophrenia. Accordingly, it is possible that abnormalities in the biochemical cascades regulated by $BH_4$ may alter DA, 5-HT and Glu neurotransmission, and consequently contribute to the pathophysiology of different neuropsychiatric diseases including schizophrenia. The development of a novel strain of mutant mice that is deficient in $BH_4$ by knocking out the expression of a functional sepiapterin reductase gene (spr -/-) has added new insights into the potential role of $BH_4$ in the pathophysiology and improved treatment of schizophrenia.

• Proceedings of the Korean Society of Propulsion Engineers Conference
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• 2012.05a
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• pp.149-152
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• 2012

This paper describes the study of integrated hydrogen supply system for environment friendly propulsion systems of fuel-cell UAV. Diluted hydrochloric acid was used for direct-decomposing solid-state $NaBH_4$ and generating hydrogen. Self-hydrogen pressurized reactor and pressure regulator was introduced for stable hydrogen supply. Prototype of integrated hydrogen supply systems using the solid-state $NaBH_4$ direct-decomposition was designed for performance evaluation and concept demonstration.

• KSBB Journal
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• v.15 no.2
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• pp.150-155
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• 2000

Antioxidative activity of c비ture of Streptomyces sp. BH-405 was investigated. After removal of pellets of Streptomyces sp. B BH-405, antioxidative substances were is미ated and suc$\infty$sively purified from culture of Streptomyces sp. BH-405 by by thin | layer chromatography $\pi$LC) or silica gel column chromatography. The fraction 3 obtained from ethylether fractionation of the C culture appeared highest level of anti oxidative activity as determined by thiocyanate method. Band 2 obtained by further P purification of this fraction showed higher anti oxidation level than that of same concentration of dl- $\alpha$ -tocopherol, butylated h hydroxy anisole (BHA). The band 2 showed higher rate of 1, 1.diphenyl 2-picrylhydrazyl (DPPH) decolorization than dl-$\alpha$-tocopherol. In the rat liver microsomes, band 2 rapidly inhibited lipid peroxidation which was initiated enzymatically by r reduced nicotinamide adenine dinucleotide phosphate (NADPH) or non-enzymatically by Fenton’s reagent. Band 2 inhibited on | lipid peroxidation of mitochondria or the linoleic acid hydro peroxide induced peroxidation system. It is concluded that band 2 obtained by fractionation of Streptomyces sp. BH-405 cultivation contained antioxidants with the capacity to inhibit oxidative m modification.