• Journal of the Korean Child Neurology Society
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• v.24 no.3
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• pp.71-83
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• 2016

X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ATP binding cassette subfamily D member 1 (ABCD1), a gene that encodes peroxisomal membrane located on ABC half-transporter named adrenoleukodystrophy protein (ALDP). X-ALD is characterized by a highly variable clinical spectrum, including progressive cerebral type, adrenomyeloneuropathy, and addison-only phenotype. No genotype/phenotype correlation has been established. Thus, unidentified modifier genes and other co-factors are speculated to modulate the phenotypic variation and disease severity. Recent advanced sequencing methods and reprogramming technologies not only offer an affordable and applicable approach to investigate the pathophysiological mechanisms of adrenoleukodystrophy, but also provide means to develop therapy. A causal therapy of X-ALD is lacking. Lorenzo's oil therapy is recommended for asymptomatic boys, but the longest study found that the oil was not beneficial at all to symptomatic X-ALD patients. Hematopoietic stem cell therapy has a relevant chance of success when performed during this early stage of cerebral type X-ALD. Recently, it has been insisted that lentiviral-mediated gene therapy of hematopoietic stem cells can provide clinical benefits in X-ALD. This review describes current knowledge on the clinical presentation, pathogenesis, diagnosis and management of X- ALD.

• Mass Spectrometry Letters
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• v.12 no.4
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• pp.126-130
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• 2021

The mass spectrometry (MS) provides the mass-to-charge ratios of atoms, molecules, stable/metastable complexes, and their fragments. I have taken a long journey with MS to address outstanding issues and problems by experiments and theory and gain insights into underlying principles in chemistry. By looking through the mass-to-charge ratio, I have studied thermochemical problems in silicon chemistry, the infrared multiphoton dissociation spectroscopy of organometallic intermediates, unimolecular dissociations of halotoluene radical cations, and the kinetics of association/dissociation of alkali halide triple ions with Lewis bases. Various MS platforms have been used to characterize non-covalent interactions between porphyrins and fullerenes and those between the group IIB ions and trioctylchalcogenides, and to examine the binding of the group IA, IIA and porphyrin ions to G-quadruplex DNA. Recently, I have focused on mass-balanced H/D isotope dipeptide tags for MS-based quantitative proteomics, a simple chemical modification method for MS-based lipase assay, and the kinetics and dynamics of energy-variable collision-induced dissociation of chemically modified peptides. Now, I see an important role of MS in global issues in the post-COVID era, as the society demands high standards for indoor air quality to contain the airborne-pathogen transmission as well as in-situ monitoring and tracking of carbon emissions to reduce global warming.

• Geomechanics and Engineering
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• v.33 no.4
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• pp.401-414
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• 2023

Organic clays are ideal habitat for flora and fauna. From a geotechnical perspective, organic clays are soft, weak, variable, heterogeneous and flocculated. Portland cement is a universally common stabiliser. However, some organic acids in soil inhibit full hydration and expose cementation products to rapid dissolution. This paper investigates scopes for use of C₃S cement to enable durable cementation. Prospects of using PP fibre alongside with C₃S cement, scopes for partial replacement of C₃S cement with a plant-based nanosilica and evolution of binders are then investigated. Binding mixtures here mimic the natural functions of rhizoliths, amorphous phases, and calcites. Testing sample population include natural and fibre-reinforced clays, compact mixes of clay - C₃S cement, clay - nanobiosilica, and clay, C₃S cement and nanobiosilica. Benefits and constraints of C₃S cement and fibres for retaining the naturally flocculated structure of organic clays are discussed. Nanobiosilica provides an opportunity to cut the C₃S content, and to transition of highly compressive organic clays into an engineered, open-structured medium with >0.5 MPa compressive strength across the strains spanning from peak to 1.5-times peak.

• Korean Journal of Environmental Agriculture
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• v.24 no.4
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• pp.379-385
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• 2005

This study was conducted to find out a useful bioremediation technology for heavy metal contaminated soil and water. We isolated strain CPB from heavy metal contaminated soil and evaluated the tolerance level and adsorption capacity of strain CPB to heavy metals (Strain is not determined yet). Strain CPB showed variable tolerance limit to different kinds heavy metal or concentrations of heavy metals. The growth of strain CPB was significantly inhibited by mixed heavy metals (Cd+Cu+Pb+Zn) than that of by single heavy metal. Strain CPB showed high binding capacity with Pb (Pb>Cd>Cu>Zn). In general, strain CPB showed high uptake of heavy metals such as Pb, Cd and Cu. It was observed that the capacity of heavy metal uptake from mixture of heavy metals was reduced in comparison with single heavy metal treatment. But total contents of heavy metal bound with cell in mixed heavy metal showed higher than in single heavy metal treatment. Heavy metal adsorption in cells was affected by several external factors, such as temperature and pH etc.. The optimum temperature and pH of the adsorption of heavy metal into cells were ca. $25{\sim}35^{\circ}C$ and pH ca. $5{\sim}7$, respectively. A large number of the electron dense particles were found mainly on the cell wall and cell membrane fractions, which was determined by transmission electron microscope. Energy dispersive X-ray spectroscopy revealed that the electron dense particles were the heavy metal complexes the substances binding with heavy metals.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.34 no.5
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• pp.550-555
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• 2001

Insulin-like growth factor-I (IGF-I) is a mitogenic peptide with a molecular mass of 7 kDa. It is produced mainly in the liver and has important functions in the regulation of development and somatic growth. Moreover, Serum IGF-I concentration is regulated by the quantity and the nutritional quality of dietary protein. To determine the IGF-I level in Korean rockfish, Sabastes schlegeli, were fed four experiment diets that contained different protein quantities, namely $30\%,\;40\%,\;50\%\;and\;60\%$ for 70 days. Weight gain of the fish increased depending dietary protein quantity, Also, IGF-I concentrations increased according to dietary protein quantity, Feeding experiments were conducted to examine the effects of dietary protein sources on the serum IGF-I level in Korean rockfish, Fish meal (CO), soybean meal (SM), corn-gluten meal (CGM), meat meal (MM) and feather meal (FM) were used as variable protein sources of the formulated diet. IGF-I concentrations of the CO and MM groups ($277.7\pm23.2,\;291.5\pm41.2\;ng/mL$) were higher than those of the CGM and FM groups ($208.9\pm21.3,\;217.2\pm38.2\;ng/mL$). And IGFBP-3 levels by western blot analysis increased in good protein diets such as in the CO and MM groups. In conclusion, IGF-I may be a sensitive indicator the protein metabolism in fish as well as mammalian.

• Journal of Technologic Dentistry
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• v.33 no.4
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• pp.349-357
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• 2011

Purpose: In this study, for the reasons of observing the changes when using bonding agent with Ni-Cr alloy and Co-Cr alloy and using VM13 and Vintage MP ceramic which have the disparity in coefficient of thermal expansion, it is carried out to evaluate the characteristics of the bonding agent through the analysis of the interface between metal and ceramic and the analysis of bond strength by variable. Methods: The surface treatment was performed on the two kinds of alloy(Ni-Cr alloy and Co-Cr alloy) specimens, which were sandblasted and were treated with bonder application. The metal-ceramic interfaces were analyzed with EPMA in order to ionic diffusion, and the shear test was performed. Results: As a result of observation of metal-ceramic interfacial properties, it was observed that Cr atoms were spread from the alloy body to the ceramic floor in the specimen of Group B. It was also seen that Cr, W atoms were spread from the alloy body to the ceramic floor in the specimen of Group S. In consequence of observing Shear bond strength, it was calculated that the specimen of BSV was 27.75(${\pm}11.21$)MPa, BSM was 27.02(${\pm}5.23$)MPa, BCV was 30.20(${\pm}5.99$)MPa, BCM was 27.94(${\pm}10.76$)MPa, SSV was 20.83(${\pm}2.58$)MPa, SSM was 23.98(${\pm}3.94$)MPa, SCV was 32.32(${\pm}4.68$)MPa, and SCM was 34.54(${\pm}10.63$)MPa. Conclusion: In the metal-ceramic interface of Bellabond plus sample group, diffusion of Cr atoms was incurred and diffusion of C Cr atoms and W atoms in the sample group of $Starloy{(R)}\;C$ was observed. Using bonding agent showed the higher bond strength than using the sand blasting treatment. In the Bellabond plus alloys, the specimen group with the use of binding materials showed higher shear bond strength, but didn't show statistically significant differences (p>0.05). In the $Starloy{(R)}\;C$ alloys, the specimen group with the use of binding materials showed higher shear bond strength and statistically significant differences(p<0.05). In terms of VM13 ceramic, it was in the Bellabond plus alloys that the high shear bond strength was showed, but there's no statistically significant differences(p>0.05). In terms of Vintage MP ceramic, it was in the $Starloy{(R)}\;C$ alloys that the high shear bond strength was showed and statistically significant differences(p<0.05). Metal-ceramic to fracture of the shear strength measurements and an analysis of all aspects of military usage fracture of the composite, respectively.

• Journal of Microbiology and Biotechnology
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• v.29 no.4
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• pp.651-657
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• 2019

Although smallpox was eradicated in 1980, it is still considered a potential agent of biowarfare and bioterrorism. Smallpox has the potential for high mortality rates along with a major public health impact, eventually causing public panic and social disruption. Passive administration of neutralizing monoclonal antibodies (mAbs) is an effective intervention for various adverse reactions caused by vaccination and the unpredictable nature of emerging and bioterrorist-related infections. Currently, vaccinia immune globulin (VIG) is manufactured from vaccinia vaccine-boosted plasma; however, this production method is not ideal because of its limited availability, low specific activity, and risk of contamination with blood-borne infectious agents. To overcome the limitations of VIG production from human plasma, we isolated two human single-chain variable fragments (scFvs), (SC34 and SC212), bound to vaccinia virus (VACV), from a scFv phage library constructed from the B cells of VACV vaccine-boosted volunteers. The scFvs were converted to human IgG1 (VC34 and VC212). These two anti-VACV mAbs were produced in Chinese Hamster Ovary (CHO) DG44 cells. The binding affinities of VC34 and VC212 were estimated by competition ELISA to $IC_{50}$ values of $2{\mu}g/ml$ (13.33 nM) and $22{\mu}g/ml$ (146.67 nM), respectively. Only the VC212 mAb was proven to neutralize the VACV, as evidenced by the plaque reduction neutralization test (PRNT) result with a $PRNT_{50}$ of ~0.16 mg/ml (${\sim}1.07{\mu}M$). This VC212 could serve as a valuable starting material for further development of VACV-neutralizing human immunoglobulin for a prophylactic measure against post-vaccination complications and for post-exposure treatment against smallpox.

• Journal of Korean Academy of Fundamentals of Nursing
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• v.9 no.1
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• pp.113-122
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• 2002

Purpose: The purpose of this study was to identify characteristics of serum ferrum, TIBC and ferritin's circadian rhythm in normal adults and to prepare a standard to determine the examination material extraction time. Method: Nine women and ten men made up the convenience sample for this study they were from the staff of D university hospital and students in D medical School located in K city who met the qualifications for inclusion in the sample. The value of serum ferrum, TIBC and circadian rhythm were calculated as follows : First. each variable's amplitude. the acrophase and average were measured for a 24 hour cycle using the cosinor method, and then each person's rhythm was analyzed. Results: There were significant serum iron circadian rhythm for both men and women (p<.05). For the men, mesor was $105.91{\mu}g/dl$. amplitude was $29.52{\mu}g/dl$, and the acrophase was 9.76 hour. For the women, mesor was $108.17{\mu}g/dl$, amplitude was $28.09{\mu}g/dl$, and the acrophase was 11.42 hour The rhythm change of TIBC was only significant for the women (p<.05), mesor was 383.39mg/dl, amplitude was 60.29mg/dl. and the acrophase was 14.93hour. As for the circadian rhythm of the ferritin, there are no diurnal variation in either sex, men were between 134.0ng/ml and 137.4ng/ml, and women, between 29.1ng/ml and 30.1ng/ml. Conclusion: To help diagnose the boundary line between normal or deficiency in iron, measurement should be carried out at a fixed time in the morning and evening, or a more proper time would be in the afternoon at the time when the width of amplitude is the least.

• Asian-Australasian Journal of Animal Sciences
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• v.24 no.7
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• pp.1019-1025
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• 2011

Toll-like receptors (TLRs) play an important role in the recognition of invading pathogens and the modulation of innate immune responses in mammals. The TLR4 and TLR7 are well known to recognize the bacterial lipopolysaccharide (LPS) and single stranded (ssRNA) ligands, respectively and play important role in host defense against Gram-negative bacteria and ssRNA viruses. In the present study, coding exon fragments of these two TLRs were identified, cloned, sequenced and analyzed in terms of insertion-deletion polymorphism, within bovine TLRs 4 and 7, thereby facilitating future TLR signaling and association studies relevant to bovine innate immunity. Comparative sequence analysis of TLR 4 exons revealed that this gene is more variable, particularly the coding frame (E3P1), while other parts showed percent identity of 95.7% to 100% at nucleotide and amino acid level, respectivley with other Bos indicus and Bos taurus breeds from different parts of the world. In comparison to TLR4, sequence analysis of TLR7 showed more conservation among different B. indicus and B. taurus breeds, except single point mutation at 324 nucleotide position (AAA to AAM) altering a single amino acid at 108 position (K to X). Percent identity of TLR7 sequences (all 3 exons) was between 99.2% to 100% at nucleotide and amino acid level, when compared with available sequence database of B. indicus and B. taurus. Simple Modular Architecture Research Tool (SMART) analysis showed variations in the exon fragments located in the Leucine Rich Repeat (LRR) region, which is responsible for binding with the microbial associated molecular patterns and further, downstream signaling to initiate anti-microbial response. Considering importance of TLR polymorphism in terms of innate immunity, further research is warranted.

• Korean Journal of Cognitive Science
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• v.10 no.4
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• pp.71-83
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• 1999

Conventional symbolic inference systems lack flexibility because they do not well reflect flexible semantic structure of knowledge and use symbolic logic for their basic inference mechanism. For solving this problem. we have recently proposed the 'Connectionist Semantic Network(CSN)' as a model for flexible knowledge representation and inference based on neural networks. The CSN is capable of carrying out both approximate reasoning and commonsense reasoning based on similarity and association. However. we have difficulties in representing general and structured high-level knowledge and variable binding using the connectionist framework of the CSN. In this paper. we propose a hybrid system called SymCSN(Symbolic CSN) that combines a symbolic module for representing general and structured high-level knowledge and a connectionist module for representing and learning low-level semantic structure Simulation results show that the SymCSN is a plausible model for human-like flexible knowledge representation and inference.