• Journal of Life Science
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• v.31 no.10
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• pp.913-921
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• 2021

Limb-girdle muscular dystrophy (LGMD) which is characterized by progressive muscle weakening of the hip and shoulder shows both dominant and recessive inheritances with many pathogenic genes including TTN. This study performed to identify genetic causes of a male patient with late onset (45 years old) autosomal recessive LGMD and atrial flutter. By application of the whole exome sequencing, we identified bi-allelic variants of TTN gene in the patient. One allele had a single missense variant of [c.24124G>T (p.V8042F)], while the other allele consisted of three missense variants of [c.29222G>C (p.R9741P) + c.67490A>G (p.H22497R) + c.75376C>T (p.R25126C)]. The p.V8042F allele was transmitted from his mother, while the other haplotype allele was putatively transmitted from his father. His two unaffected sons had only the p.R9741P. These variants have been not reported or rarely reported in the public human genome databases (1,000 Genome, gnomAD, and KRGDB). Most variants were located in the highly conserved immunoglobulin or fibronectin domains and were predicted to be pathogenic by the in silico analyses. The TTN giant protein plays a key role in muscle assembly, force transmission at the Z-line, and maintenance of resting tension in the I-band. In conclusion, we think that these bi-allelic compound heterozygous mutations may play a role as the genetic causes of the LGMD phenotype.

• Journal of Life Science
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• v.31 no.9
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• pp.818-826
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• 2021

5-Aminolevulinic acid phosphate (5-ALA-p) is a substance obtained by eluting 5-ALA (a natural delta amino acid) with aqueous ammonia, adding phosphoric acid to the eluate, and then adding acetone to confer properties suitable for use in photodynamic therapy applications. However, its pharmacological efficacy, including potential mechanisms of antioxidant and anti-inflammatory reactions, remains unclear. This study aimed to investigate the effects of 5-ALA-p on oxidative and inflammatory stresses in lipopolysaccharide (LPS)-stimulated RAW 264.7 cells. Our data showed that 5-ALA-p significantly inhibited excessive phagocytic activity via LPS and attenuated oxidative stress in LPS-treated RAW 264.7 cells. Furthermore, 5-ALA-p improved mitochondrial biogenesis reduced by LPS, suggesting that 5-ALA-p restores mitochondrial damage caused by LPS. Additionally, 5-ALA-p significantly suppressed the release of nitric oxide (NO) and pro-inflammatory cytokines, such as tumor necrosis factor α (TNF-α), interleukin (IL)-1β, and IL-6, which are associated with the inhibition of inducible NO synthase and respective cytokine expression. Furthermore, 5-ALA-p reduced the nuclear translocation of nuclear factor-kappa B (NF-κB) and inhibited phosphorylation of mitogen-activated protein kinases (MAPKs), indicating that the anti-inflammatory effect of 5-ALA-p is mediated through the suppression of NF-κB and MAPK signaling pathways. Based on these results, 5-ALA-p may serve as a potential candidate to reduce inflammation and oxidative stress.

• Korean Journal of Heritage: History & Science
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• v.54 no.4
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• pp.22-35
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• 2021

Recently, as the climate changes rapidly and the prevalence of airborne fine particulate matter increases, the pattern of pollutants in the atmospheric environment is also changing. Therefore, the importance of studying the stability of pigments used in colored cultural properties is emerging. Hoecheong is an inorganic blue glass pigment called smalt; it is made by using cobalt as a coloring element in potash glass, and was widely used in colored cultural assets, such as murals and paintings. In this study, we collected three other hoecheong pigments to analyze their properties. The percentage of Co and K contained are different according to the manufacturer, and the smalt-3 sample has a lower cobalt content (15.1 wt.%) and higher potassium content (29.6 wt.%). After this analysis, colored specimens were prepared. Prepared specimens were exposed to ultra-violet rays, CO₂/NO₂, and NaCl, which are known to have the greatest influence on the stability of pigments. We found that factors affecting the color stability were NO₂ gas, ultra-violet rays, and water-soluble salts (NaCl). Among them, NO₂ has the most severe impact on color change of the pigments. Results of the component analysis showed that the color change depends on the potassium and cobalt content of the hoecheong pigment. Among the specimens, smalt-3 showed the most vulnerability after exposure to NO₂ gas and water-soluble salts. Pigment film stability is affected by watersoluble salts, giving rise not only to color change, but also weakening the physical properties of the film. However, there was no significant change in composition and color after exposure to CO₂ gas. In conclusion, we found that hoecheong pigments underwent color change and increased instability of the coating film when exposed to any of the atmospheric environmental factors used in this study, except for CO₂.

• Journal of Korean Philosophical Society
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• v.137
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• pp.81-108
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• 2016

In Medieval and Christian history, disputation between Augustine and Pelagius originated from different perspectives regarding the free-will of humans and the grace of God. This debate has continued throughout the Middle-Ages and the Reformation to date. In this regard, the purpose of this article is to investigate the implication of opposition and disputation between Augustine and Pelagius on today's society. Overemphasis on human capacity and free-will leads to humanism, while the arguments supporting the grace of God can encourage evasion of the ethical obligations of humans. Augustine and Pelagius had opposing views on this subject. Pelagius emerged in the Roman Empire, emphasizing the ethical responsibility of humans with free-will. On the other hand, Augustine argued that human nature and free-will had been damaged. With a pessimistic outlook on human capacity, he emphasized the grace of God. Shocked by the self-indulgence and ethical negligence of the Church of Rome which was the center of Christian nations at that time, Pelagius strongly urged Christians of the Roman Church to take moral responsibility. However, the theology of Augustine justified the status quo of the Roman Church while Pelagius focused on the necessity of an ethical life of humans based on free-will. Viewing Pelagius' strict moralism as a humanist view that emphasizes human capacity and action, Augustine showed strong opposition to such a view and instead emphasized God's grace. Pelagius was condemned as a heretic as his belief in free-will did not follow Augustine's Doctrine of Grace. However, Pelagianism penetrated Christian history and still poses a threat to Christianity to date. Societal corruption and depravity is still prevalent in today's society as it was in the time of Pelagius. South Korea for instance is exposed to serious moral corruption and a lack of social responsibility as shown in the sinking of the MV Sewol in April 2014. For those reasons, Christian society emphasizes Christian ethics and requires an honest leader who will set an example of Christian life. In this light, I want to examine the implications of disputation between Augustine and Pelagius on today's society.

• Journal of Navigation and Port Research
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• v.42 no.6
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• pp.507-517
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• 2018

The probability of T/S cargo volume to decrease is the most notable problem associated with inappropriate mix of terminal operators in Busan port. Other problems include, the deterioration of the national carriers' competitiveness from non-operation of own terminal, excessively high proportion of financial operators in the engagement of operation which may result in their passiveness in timely investment, additional cost burden to carriers' in the handling inter-terminal T/S cargo transportation and inefficiency in terminal operation by the multiplicity of operators proved to be same recognized as so through the analysis. Therefore, in order to provide solutions for the problems and to strengthen Busan port's competitiveness, this research suggests the restructuring of operators mix as follows. To achieve sustainable growth of T/S cargo, global carriers' participation in terminal operation should be of utmost priority. To enhance the operational efficiency, the operators should be integrated. Similarly, the integration of operators will play a key role in verifying that national carriers' own terminal operation is an important factor in raising its competence. Finally, BPA's active engagement in the entire operation of port is also critical in public-oriented operation of the port. Whereas in the interactive analysis by taking the merits of Busan port into consideraion, global carrier's participation in operation, integration of operators and BPA's engagement in operation proved to contribute to the increase of T/S cargo and strengthening of operational efficiencies of Busan port.

• Journal of Life Science
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• v.29 no.7
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• pp.800-808
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• 2019

Charcot-Marie-Tooth disease (CMT) is a group of rare peripheral neuropathies characterized by progressive muscle weakness and atrophy and areflexia in the upper and lower extremities. The most common subtype of CMT is CMT1A, which is caused by a tandem duplication of the PMP22 gene in the 17p12 region. Patients with CMT1A show a loose genotype-phenotype correlation, which suggests the existence of secondary genetic or association factors. Recently, polymorphisms of rs71428439 (n.83A>G) and rs2292832 (n.86T>C) in the MIR149 have been reported to be associated with late onset and mild phenotypic CMT1A severity. The aim of this study was to examine the intrafamilial heterogeneities of clinical phenotypes according to the genotypes of these two SNPs in MIR149. For this study, we selected 6 large CMT1A families who showed a wide range of phenotypic variation. This study suggested that both SNPs were related to the onset age and severity in the dominant model. In particular, the AG+GG (n.83A>G) and TC+CC genotypes (n.86T>C) were associated to late onset and mild symptoms. Motor nerve conduction velocity (MNCV) was not related to the MIR149 genotypes. These results were consistent with the previous studies. Therefore, we suggest that the rs71428439 and rs2292832 variants in MIR149 may serve as genetic modifiers of CMT1A intrafamilial phenotypic heterogeneity, as they have a role in the unrelated patients. This is the first study to show an association using large families with variable clinical CMT1A phenotypes. The results will be helpful in the molecular diagnosis and treatment of patients with CMT1A.

• The Korean Journal of Archival Studies
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• no.22
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• pp.37-76
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• 2009

This paper aims-through comparative research on two organizations and use of political process theory-to analyze the historical development of, current issues related to and the characteristics of the new transformation of the Freedom of Information Movements (FOIMs) in South Korea. In the ten years since the Freedom of Information Act (FOIA) took effect in 1998, Korean FOIMs have developed along the following course: 'emergence' (1998), 'expansion and extension' (1999-2004), 'institutionalization and retro-institutionalization-' (2005-2008). Specifically, in the early stage of FOIMs, the Freedom of Information (FOI) department of the People's Solidarity for Participatory Democracy, established in 1998, had led the FOI movement by initiating reform of the FOI institution and advocating an end to old practices. Paradoxically, however, following the institutional progress of FOI under the Roh Moo Hyun government, the vitality of FOIMs seemed to be weakening. And under the Lee Myung Bak government, which is showing regression in both the FOI institution and practices, the 'dual transformation' of the FOIMs is being led not by old groups but by new ones. The Center for Freedom of Information and Transparent Society(CFOI), which was founded in 2008, has journalists, researchers of archival studies, citizens, lawyers and nongovernmental activists as members. Through its blog style Homepage, countless reports are becoming "open to the public" and "share with the public." And its various civic education programs are interactive bridges which enable mutual communication between the Center and citizens. CFOI is expanding the FOI movement in different ways than the traditional activists such as the FOI department of the PSPD department, which worked through methods such as policy proposals, disclosing information litigation, comments and public statements, and hosting forums. CFOI is leading the 'dual process of transformation' of FOIMs, namely the transformation from an 'advocacy' movement to an 'empowerment' movement and transformation of the FOI movement's framework from "open to the public" to "share with the public."

• The Korean Journal of Archival Studies
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• no.19
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• pp.153-192
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• 2009

Appearing newly on June 1894, Gaboh regime enforced modern reformation policy. In light of archives' management, it was totally different from before. Government established individual department of record management in every division and proclaimed a legislative bill which was stipulated about record management process. They modified archives' form including peculiar declaration of the name of an era and use together with Korean and Chinese. Also they tried to conserve the original copy of the archives. As King Gojong announced the Great Han Empire(Taehan Cheguk, 大韓帝國) on October 1897, he reinforced Gaboh regimes' weakened royal authority and enforced reformation policy which was designed for himself. First he abolished the administration which restricted royal authority, and established new department called Euijungbu(議政府). To restrain the royal power, he separated the Royal House and government and reinforced Gungnaebu(宮內府). In addition, King Gojong enforced the policy which he can manage directly about troops, policies, and finances. Consequently, He established Wonsubu(元帥府), Kyungbu(警部), and made direct belonging of an emperor. Also, department called Naejangwon(內藏院) tried to levy many kinds of taxes directly to build up the financial foundation under the emperor. The record management system of Great Han Empire succeeded to that of Gaboh regimes Times'. First, government and powerful organization directly under the emperor set up the department of record management. Euijungbu (議政府) and governmental department, of course, Gungnaebu(宮內府), Wonsubu(元帥府), Kyungbu(警部), Tongshinwon(通信院), Jikyeahmun(地契衙門) which support the right of an emperor established document division and record division individually. To carry out government's service effectively and systematically, it was considered effective to divide record management department. Moreover, despite the difference between the divisions, they were separated into current record division and non current record division. Generally, document department took charge of acceptance, sending and crafting of current document and archives department was eligible for preservation and compilation of major document and eternal conservation document. This seems to consider life cycle of the record and keep the evaluation of record in mind. Finally, perception for the record management has revealed to modern configuration.

• Journal of Climate Change Research
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• v.9 no.4
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• pp.423-433
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• 2018

Particulate matter air pollution is a serious problem affecting human health and visibility. The variations in $PM_{10}$ concentrations are influenced by not only local emission sources, but also atmospheric circulation conditions. In this study, we investigate the temporal features of $PM_{10}$ concentrations in South Korea and the atmospheric circulation patterns associated with high concentration episodes of $PM_{10}$ during winter (December-January-February) 2001-2016. Based on those analyses, a Korea Particulate matter Index (KPI) is developed to represent the large-scale atmospheric pattern associated with high concentration episodes of $PM_{10}$. The atmospheric patterns are characterized by persistent high-pressure anomalies, weakened lower-level north-westerly anomalies, and northward shift of the upper-level meridional wind anomalies near the Korean Peninsula. To evaluate the change in occurrence of high concentration episodes of $PM_{10}$ under a possible future warmer climate, we apply KPI analysis to CMIP5 climate simulations. Here, historical and two representative concentration pathway (RCP) scenarios (RCP 4.5 and RCP 8.5) are used. It is found that the occurrence of atmospheric conditions favorable for high $PM_{10}$ concentration episodes tends to increase over South Korea in response to climate change. This suggests that large-scale atmospheric circulation changes under future warmer climate can contribute to increasing high $PM_{10}$ concentration episodes in South Korea.

• International Area Studies Review
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• v.22 no.3
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• pp.221-251
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• 2018

Two-tier wage systems mean the dual wage systems that the new wage system require the new future employees to get much lower wage level, compared to the level of wages for existing employees under the existing wage system. While it allows employers to benefit from low-cost advantages, the two-tier wage systems is definitely a type of wage discrimination by the collusion between management and trade unions in that it forces the new future employee to accept the low wages. Also it reflects extreme collectivism which old union members try to keep having their jobs and wages at the sacrifice of future members' wages. The two-tier wage systems had been introduced by airplane industry in 1980s and introduced again in Big Three auto companies in 2007. The purpose of this paper is to examine the history, contents, and details of two-tier wage system in the United States and to think of the possibility of recurrence of the systems in the context of Korean auto industry in the near future. The tentative findings from this paper implies that the two-tier wage systems will likely happen in Korean auto industry because the collusion between trade unions and management are found easily and the degree of extreme collectivism favored by the old permanent union members is seriously high. It is time for trade unions to go back to their original ideals and purposes and to revitalize solidarity among workers.