• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• The Journal of Art Theory & Practice
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• no.5
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• pp.217-229
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• 2007

Many historical research and facet about modern art in Indonesia which formulating background of contemporary Indonesian Art. Indonesian art critic Sanento Yuliman states that Modern art has been rapidly developing in Indonesia since the Indonesian Independence in 1945. Modern Art is a part of the super culture of the Indonesian metropolitan and is closely related to the contact between the Indonesian and Western Cultures. Its birth was part of the nationalism project, when the Indonesian people consists of various ethnics were determined to become a new nation, the Indonesian nation, and they wished for a new culture, and therefore, a new art. The period 1960s, which was the beginning of the creation and development of the painters and the painters associations, was the first stage of the development of modern art in Indonesia. The second stage showed the important role of the higher education institutes for art. These institutes have developed since the 1950s and in the 1970s they were the main education institutes for painters and other artists. The artists awareness of the medium, forms or the organization of shapes were encouraged more intensely and these encouraged the exploring and experimental attitudes. Meanwhile, the information about the world's modern art, particularly Western Art; was widely and rapidly spread. The 1960s and 1970s were marked by the development of various abstractions and abstract art and the great number of explorations in various new media, like the experiment with collage, assemblage, mixed media. The works of the Neo Art Movement-group in the second half of the 1970s and in the 1980s shows environmental art and installations, influenced by the elements of popular art, from the commercial world and mass media, as well as the involvement of art in the social and environmental affairs. The issues about the environment, frequently launched by the intellectuals in the period of economic development starting in the 1970s, echoed among the artists, and they were widened in the social, art and cultural circles. The Indonesian economic development following the important change in the 1970s has caused a change in the life of the middle and upper class society, as has the change in various aspects of a big city, particularly Jakarta. The new genre emerged in 1975 which indicates contemporary art in Indonesia, when a group of young artists organized a movement, which was widely known as the Indonesian New Art Movement. This movement criticized international style, universalism and the long standing debate on an east-west-dichotomy. As far as the actual practice of the arts was concerned the movement criticized the domination of the art of painting and saw this as a sign of stagnation in Indonesian art development. Based on this criticism 'the movement' introduced ready-mades and installations (Jim Supangkat). Takes almost two decades that the New Art Movement activists were establishing Indonesian Installation art genre as contemporary paradigm and influenced the 1980's gene ration like, FX Harsono, Dadang Christanto, Arahmaiani, Tisna Sanjaya, Diyanto, Andarmanik, entering the 1990's decade as "rebellion period" ; reject towards established aesthetic mainstream i.e. painting, sculpture, graphic art which are insufficient to express "new language" and artistic needs especially to mediate social politic and cultural situation. Installation Art which contains open possibilities of creation become a vehicle for aesthetic establishment rejection and social politics stagnant expression in 1990s. Installation art accommodates two major field; first, the rejection of aesthetic establishment has a consequences an artists quest for medium; deconstruction models and cross disciplines into multi and intermedia i.e. performance, music, video etc. Second aspect is artists' social politic intention for changes, both conclude as characteristics of Indonesian Installation Art and establishing the freedom of expression in contemporary Indonesian Art until today.

• Journal of Marine Life Science
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• v.8 no.2
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• pp.104-114
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• 2023

As plastic usage increases globally, the amount of plastic waste entering the marine environment is steadily rising. Microplastics, in particular, can be ingested by marine organisms and accumulated in their digestive tracts, causing harmful effects on their growth and reproduction. Cytochrome P450 (CYP) enzymes are known to metabolize various environmental pollutants as detoxification enzymes, but their role in crustaceans is not well understood. In this study, sequences of nine CYP genes (CYP370A4, CYP370C5 from clan 2; CYP350A1, CYP350C5, CYP361A1 from clan 3; CYP4AN-like, CYP4AP2, CYP4AP3, CYP4C33-like1 from clan 4) were analyzed using conserved domains in the brackish water flea Diaphanosoma celebensis. Additionally, after exposure to three different sizes of polystyrene beads (0.05-, 0.5-, 6-㎛ PS beads; 0.1, 1, and 10 mg/L) for 48 hours, the expression of these nine CYP genes were investigated using real-time reverse transcription polymerase chain reaction (RT-PCR). The results showed that all CYP genes possessed conserved motifs, indicating that D. celebensis CYP has evolutionarily conserved functions. Among these CYP genes, the expression of CYP370C5, CYP360A1, and CYP4C122 showed a significant increase after exposure to 0.05-㎛ PS beads, suggesting their involvement in PS metabolism. This research will contribute to understanding the molecular mode of actions of microplastics on marine invertebrates.

• Journal of Life Science
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• v.19 no.11
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• pp.1529-1537
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• 2009

To elucidate further the antitumor effects of a natural L-arginine analogue, L-canavanine, the mechanism underlying apoptogenic activity of L-canavanine and its modulation by protein tyrosine kinase $p56^{lck}$ was investigated in human Jurkat T cells. When the cells were treated with 1.25 to 2.5 mM L-canavanine for 36 h, several apoptotic events including mitochondrial membrane potential (${\Delta\Psi}m$) loss, activation of caspase-9, -3, -8, and -7, poly (ADP-ribose) polymerase (PARP) degradation, and DNA fragmentation were induced without alteration in the levels of Fas or FasL. These apoptotic changes were more significant in $p56^{lck}$-deficient Jurkat clone JCaM1.6 than in $p56^{lck}$-positive Jurkat clone E6.1. The L-canavanine-induced apoptosis observed in $p56^{lck}$-deficient JCaM1.6 cells was significantly reduced by introducing $p56^{lck}$ gene into JCaM1.6 cells by stable transfection. Treatment of JCaM1.6/lck cells with L-canavanine caused a transient 1.6-fold increase in the kinase activity of $p56^{lck}$. Both FADD-positive wild-type Jurkat T cell clone A3 and FADD-deficient Jurkat T cell clone I2.1 exhibited a similar susceptibility to the cytotoxicity of L-canavanine, excluding involvement of Fas/FasL system in triggering L-canavanine-induced apoptosis. The L-canavanine-induced apoptotic sub-$G_1$ peak and activation of caspase-3, -8, and -7 were abrogated by pan-caspase inhibitor (z-VAD-fmk), whereas L-canavanine-induced activation of caspase-9 was not affected. These results demonstrated that L-canavanine caused apoptosis of Jurkat T cells via the loss of ${\Delta\Psi}m$, and the activation of caspase-9, -3, -8, and -7, leading to PARP degradation, and that the $p56^{lck}$ kinase attenuated the ${\Delta\Psi}m$ loss and activation of caspases, and thus contributed as a negative regulator to L-canavanine-induced apoptosis.