• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.25-28
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• 2016

Phelan-McDermid syndrome is a rare genetic disorder caused by the terminal or interstitial deletion of the chromosome 22q13.3. Patients with this syndrome usually have global developmental delay, hypotonia, and speech delays. Several putative genes such as the SHANK3, RAB, RABL2B, and IB2 are responsible for the neurological features. This study describes the clinical features and outcomes of Korean patients with Phelan-McDermid syndrome. Two patients showing global developmental delay, hypotonia, and speech delay were diagnosed with Phelan-McDermid syndrome via chromosome analysis, fluorescent in situ hybridization, and multiplex ligation-dependent probe amplification analysis. Brain magnetic resonance imaging of Patients 1 and 2 showed delayed myelination and severe communicating hydrocephalus, respectively. Electroencephalography in patient 2 showed high amplitude spike discharges from the left frontotemporoparietal area, but neither patient developed seizures. Kidney ultrasonography of both the patients revealed multicystic kidney disease and pelviectasis, respectively. Patient 2 experienced recurrent respiratory infections, and chest computed tomography findings demonstrated laryngotracheomalacia and bronchial narrowing. He subsequently died because of heart failure after a ventriculoperitoneal shunt operation at 5 months of age. Patient 1, who is currently 20 months old, has been undergoing rehabilitation therapy. However, global developmental delay was noted, as determines using the Korean Infant and Child Development test, the Denver developmental test, and the Bayley developmental test. This report describes the clinical features, outcomes, and molecular genetic characteristics of two Korean patients with Phelan-McDermid syndrome.

• Journal of the Korean Society of Food Science and Nutrition
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• v.14 no.4
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• pp.381-386
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• 1985

This experiment was conducted to investigate changes of soybean curd yield according to the extension of soaking time during manufacturing of soybean curd. To investigate those changes systematically, transmission electron microscopy and disc-gel electrophoresis were used. The soybean curd yield was increased from 45.0% to 50.5% and 55.4% respectively as soaking time is extended from 5 hours to 10 and 24 hours. The solid extraction and soybean milk coagulation were also increased according to the extension of soaking time. From disc-gel electrophoresis patterns of soybean milk protein and soybean curd protein, numbers of band were increased and major band thickened by expending the soaking time. Most of high molecular bands of soybean milk protein were transfered to soybean curd. Crude 7S proteins of soybean milk and soybean curd in dis-gel electrophoresis were appeared to be 4 and 5 bands respectively, and crude 11S proteins of soybean milk and soybean curd were appeared to be 9 and 8 bands respectively. Of soybean milk bands, most of 11S component transfered to soybean curd. Transmission electron photomicrographs revealed that the dimension of each protein body became larger and the numbers of spherosome around the protein bodies in unit area fewer by extending the soaking time of soybean.

• Asian-Australasian Journal of Animal Sciences
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• v.24 no.4
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• pp.463-470
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• 2011

Gonadotropin-releasing hormone receptor (GnRHR) gene is expressed at the anterior pituitary gland and plays a key role in gonad development. This study aimed to investigate molecular genetic characteristics of the GnRHR gene and elucidate the effects of single nucleotide polymorphisms (SNPs) of GnRHR gene on sex steroid level in Japanese flounder (Paralichthys olivaceus). We used polymerase chain reaction single-strand conformation polymorphism (PCR-SSCP) and sequencing of the GnRHR gene in 75 individuals. We identified three SNPs in the GnRHR gene: P1 locus (C759A and C830T) in the coding region of exon2 which were both linked together and P2 locus (G984T) in the coding region of exon3, which added a new transcript factor (ADR1) and a new methylation site (CG). Only C830T of P1 leads to amino acid changes Thr266Ile. Statistical analysis showed that P1 was significantly associated with $17{\beta}$-estradiol ($E_2$) level (p<0.01) and gonadosomatic index (GSI) (p<0.05). Individuals with genotype BB of P1 had significantly higher serum $E_2$ levels (p<0.01) and GSI (p<0.05) than those of genotype AA or AB. Another SNP, P2, synonymous mutation, was significantly associated with GSI (p<0.05). Individuals with genotype AB of P2 had significantly higher GSI (p<0.05) than that of genotype AA. In addition, there was a significant association between one diplotype based on three SNPs and reproductive traits. The genetic effects for both serum $E_2$ level and GSI of diplotype D4 were super diplotypes (p<0.05). These results suggest that the SNPs in Japanese Flounder GnRHR are associated with $E_2$ level and GSI.

• Asian-Australasian Journal of Animal Sciences
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• v.31 no.4
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• pp.607-615
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• 2018

Objective: This study was conducted to isolate the cellulolytic microorganism from the rumen of Holstein steers and characterize endoglucanase gene (Cel5A) from the isolated microorganism. Methods: To isolate anaerobic microbes having endoglucanase, rumen fluid was obtained from Holstein steers fed roughage diet. The isolated anaerobic bacteria had 98% similarity with Eubacterium cellulosolvens (E. cellulosolvens) Ce2 (Accession number: AB163733). The Cel5A from isolated E. cellulolsovens sp. was cloned using the published genome sequence and expressed through the Escherichia coli BL21. Results: The maximum activity of recombinant Cel5A (rCel5A) was observed at $50^{\circ}C$ and pH 4.0. The enzyme was constant at the temperature range of $20^{\circ}C$ to $40^{\circ}C$ but also, at the pH range of 3 to 9. The metal ions including $Ca^{2+}$, $K^+$, $Ni^{2+}$,$Mg^{2+}$, and $Fe^{2+}$ increased the endoglucanase activity but the addition of $Mn^{2+}$, $Cu^{2+}$, and $Zn^{2+}$ decreased. The Km and Vmax value of rCel5A were 14.05 mg/mL and $45.66{\mu}mol/min/mg$. Turnover number, Kcat and catalytic efficiency, Kcat/Km values of rCel5A was $96.69(s^{-1})$ and 6.88 (mL/mg/s), respectively. Conclusion: Our results indicated that rCel5A of E. cellulosolvens isolated from Holstein steers had a broad pH range with high stability under various conditions, which might be one of the beneficial characteristics of this enzyme for possible industrial application.

• Proceedings of the Korean Vacuum Society Conference
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• 2014.02a
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• pp.324-325
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• 2014

Quantum wells infrared photodetectors (QWIPs) have been used to detect infrared radiations through the principle based on the localized stated in quantum wells (QWs) [1]. The mature III-V compound semiconductor technology used to fabricate these devices results in much lower costs, larger array sizes, higher pixel operability, and better uniformity than those achievable with competing technologies such as HgCdTe. Especially, GaAs/AlGaAs QWIPs have been extensively used for large focal plane arrays (FPAs) of infrared imaging system. However, the research efforts for increasing sensitivity and operating temperature of the QWIPs still have pursued. The modification of heterostructures [2] and the various fabrications for preventing polarization selection rule [3] were suggested. In order to enhance optical performances of the QWIPs, double barrier quantum well (DBQW) structures will be introduced as the absorption layers for the suggested QWIPs. The DBWQ structure is an adequate solution for photodetectors working in the mid-wavelength infrared (MWIR) region and broadens the responsivity spectrum [4]. In this study, InGaAs/GaAs/AlGaAs double barrier quantum well infrared photodetectors (DB-QWIPs) are successfully fabricated and characterized. The heterostructures of the InGaAs/GaAs/AlGaAs DB-QWIPs are grown by molecular beam epitaxy (MBE) system. Photoluminescence (PL) spectroscopy is used to examine the heterostructures of the InGaAs/GaAs/AlGaAs DB-QWIP. The mesa-type DB-QWIPs (Area : $2mm{\times}2mm$) are fabricated by conventional optical lithography and wet etching process and Ni/Ge/Au ohmic contacts were evaporated onto the top and bottom layers. The dark current are measured at different temperatures and the temperature and applied bias dependence of the intersubband photocurrents are studied by using Fourier transform infrared spectrometer (FTIR) system equipped with cryostat. The photovoltaic behavior of the DB-QWIPs can be observed up to 120 K due to the generated built-in electric field caused from the asymmetric heterostructures of the DB-QWIPs. The fabricated DB-QWIPs exhibit spectral photoresponses at wavelengths range from 3 to $7{\mu}m$. Grating structure formed on the window surface of the DB-QWIP will induce the enhancement of optical responses.

• Proceedings of the Korean Vacuum Society Conference
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• 2014.02a
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• pp.363-363
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• 2014

Ruthenium (Ru) has attractive material properties due to its promising characteristics such as a low resistivity ($7.1{\mu}{\Omega}{\cdot}cm$ in the bulk), a high work function of 4.7 eV, and feasibility for the dry etch process. These properties make Ru films appropriate for various applications in the state-of-art semiconductor device technologies. Thus, it has been widely investigated as an electrode for capacitor in the dynamic random access memory (DRAM), a metal gate for metal-oxide semiconductor field effect transistor (MOSFET), and a seed layer for Cu metallization. Due to the continuous shrinkage of microelectronic devices, better deposition processes for Ru thin films are critically required with excellent step coverages in high aspect ratio (AR) structures. In these respects, atomic layer deposition (ALD) is a viable solution for preparing Ru thin films because it enables atomic-scale control of the film thickness with excellent conformality. A recent investigation reported that the nucleation of ALD-Ru film was enhanced considerably by using a zero-valent metallorganic precursor, compared to the utilization of precursors with higher metal valences. In this study, we will present our research results on the synthesis and characterization of novel ruthenium complexes. The ruthenium compounds were easy synthesized by the reaction of ruthenium halide with appropriate organic ligands in protic solvent, and characterized by NMR, elemental analysis and thermogravimetric analysis. The molecular structures of the complexes were studied by single crystal diffraction. ALD of Ru film was demonstrated using the new Ru metallorganic precursor and O2 as the Ru source and reactant, respectively, at the deposition temperatures of $300-350^{\circ}C$. Self-limited reaction behavior was observed as increasing Ru precursor and O2 pulse time, suggesting that newly developed Ru precursor is applicable for ALD process. Detailed discussions on the chemical and structural properties of Ru thin films as well as its growth behavior using new Ru precursor will be also presented.

• Mycobiology
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• v.47 no.4
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• pp.449-456
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• 2019

Invasive fungal infections caused by Cyberlindnera fabianii have recently increased. However, biochemical kits such as API 20 C AUX and Vitek-2C have misidentified this species as other Candida spp. such as C. pelliculosa or C. utilis due to no information of Cy. fabianii in yeast database. During our 2016-2017 surveys, eleven isolates of Cy. fabianii were obtained in International St. Mary's Hospital in Korea. Here, we describe its morphological and molecular characteristics and tested its antifungal susceptibility against nine antifungal agents. The sequences of the ITS region and the D1/D2 region of LSU revealed 100% identity with the sequences of Cy. fabianii. In comparison with the results from MALDI-TOF mass spectrometry, we found that Cy. fabianii can be distinguished from other species. In antifungal susceptibility test, voriconazole and echinocandins exhibited good antifungal activities against the majority of Cy. fabianii isolates despite the absence of standard criteria.

• Polymer(Korea)
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• v.25 no.1
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• pp.78-89
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• 2001

Morphological changes of unsaturated polyester/polyvinylacetate semi-IPN were studied while the phase separation and the cure reaction occurred in a competing fashion. The light scattering and thermal analysis techniques were used to investigate the phase separation rates and mechanical properties resultantly induced by molecular diffusion of thermoplastic polymer during the curing process of thermosetting polymer. The reaction activation energy was calculated by using Flynn-Wall method and the semi-IPN structure exhibited various phase-separation morphological characteristics. When PVAc composition was 10 wt%, the phase separation was not observed during the curing reaction, but the phase separation occurred in a similar fashion to nucleation and growth(NG) mechanism at room temperature. On the other hand, when PVAc composition was over 11.65 wt%, the phase separation was generated in the middle of the curing process. Consequently, the phase separation seemed to influence the curing reaction rate, which was also supported by the changing activation energy with conversion and PVAc composition. Finally, the total scattered intensity was measured at various temperature, and subsequently the diffusion rates of phase separation R(${\beta}m$) were evaluated.

• 한국생물공학회:학술대회논문집
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• 2002.04a
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• pp.461-464
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• 2002

A Chemoautotroph identified as an Aeromonas sp. strain JS-1 was isolated from fresh water. Aeromonas sp. strain JS-1 used the $H_2$ and $CO_2$ as energy and carbon sources, respectively. Growth characteristics for improving the $CO_2$ fixation rate were examined in batch cultivation. Its results shown that the optimal growth appeared at culture conditions of $35^{\circ}C$, pH 7 and NaCl 0.1%(w/v). Some hydrogen-oxidizing bacteria were reported that the enzyme activity of ribulose 1,5-bisphosphate carboxylase- oxygenase (RubisCO-EC 4.1.1.39), in the key enzyme of the Calvin-Benson cycle. A RubisCO was purified from a chemoautotrophic bacterium, Aeromonas sp. strain JS-1. the enzyme was purified by ammonium sulfate precipitation, DEAE-sepharose CL-6B and gel filtration chromatography. The RubisCO showed that molecular mass was about 560KDa from gel filtration chromatography and nondenaturing PAGE, and the RubisCO was confirmed to consist of $L_8S_8$ enzyme structure by sodium dodecyl sulfate polyacrylamide gel electrophoresis. A large subunit was about 56KDa and small one was about 15kDa. The Km values of the enzyme for ribulose 1,5-bisphosphate(RUBP), $NaH^{14}CO_3$, and $Mg^{++}$ were estimated to be 0.25mM, 5.2mM, and 0.91mM, respectively. The optimum temperature for RubisCO enzymatic activity were $50^{\circ}C$, and the enzymatic activity was stable up to $45^{\circ}C$.

• Journal of Conservation Science
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• v.27 no.4
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• pp.345-356
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• 2011

To remove metal stains of the ceramics, chemical cleaning is essential case by case. This study investigated the removal characteristics of iron stains by oxalic acid and citric acid including their application methods of soaking and poultice. The soaking method in cleaning agents showed removal process by color difference and released iron contents from iron stains on ceramics. Iron stains were removed successfully from ceramics, which soaked in oxalic acid for 60 hours. However, it is recommendable to soak in 0.25M oxalic acid for one to three hours because most iron stains were disappeared in 3 hours soaking. Citric acid is less effective than oxalic acid in removing iron stains because of heavy molecular weight and low acidity. Poultices (bentonite, sepiolite, activated carbon fiber and celite) with oxalic acid were applied on contaminated ceramics. After ten hours, iron stains on ceramics were removed successfully by poultice. Among them, bentonite and sepiolite have better application. Therefore, sepiolite with 0.25M oxalic acid was applied on the iron stains of whiteware and celadon from Ma Island, and then stains were removed. However, it is judged that the application methods can be varied according to the form and depth of contaminant. In addition, the residues of poultice on the ceramics will be considered for preventing contamination.