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Charcot-Marie-Tooth 1A Concurrent with Schwannomas of the Spinal Cord and Median Nerve
Kwon, Joo Young,Chung, Ki Wha,Park, Eun Kyung,Park, Sun Wha,Choi, Byung-Ok The Korean Academy of Medical Sciences 2009 JOURNAL OF KOREAN MEDICAL SCIENCE Vol.24 No.4
<P>We identified Charcot-Marie-Tooth disease type 1A (CMT1A) in a family with schwannomas in the spinal cord and median nerve. The CMT1A in this family showed an autosomal dominant pattern, like other CMT patients with <I>PMP22</I> duplication, and the family also indicated a possible genetic predisposition to schwannomas by 'mother-to-son' transmission. CMT1A is mainly caused by duplication of chromosome 17p11.2-p12 (<I>PMP22</I> gene duplication). A schwannoma is a benign encapsulated tumor originating from a Schwann cell. A case of hereditary neuropathy with liability to pressure palsies (HNPP) concurrent with schwannoma has been previously reported. Although it seems that the co-occurrence of CMT1A and schwannomas in a family would be the result of independent events, we could not completely ignore the possibility that the coincidence of two diseases might be due to a shared genetic background.</P>
Kwon, Hye-Ok,Kim, Hee-Young,Park, Yu-Mi,Seok, Kwang-Seol,Oh, Jeong-Eun,Choi, Sung-Deuk Elsevier 2017 Environmental pollution Vol.220 No.1
<P><B>Abstract</B></P> <P>A nationwide emission estimate of perfluoroalkyl substances (PFASs) from wastewater treatment plants (WWTPs) is required to understand the source–receptor relationship of PFASs and to manage major types of WWTPs. In this study, the concentrations of 13 PFASs (8 perfluorocarboxylic acids, 3 perfluoroalkane sulfonates, and 2 intermediates) in wastewater and sludge from 81 WWTPs in South Korea were collected. The emission pathways of PFASs were redefined, and then the national emission of PFASs from WWTPs was rigorously updated. In addition to the direct calculations, Monte Carlo simulations were also used to calculate the likely range of PFAS emissions. The total (Σ<SUB>13</SUB>PFAS) emission (wastewater + sludge) calculated from the direct calculation with mean concentrations was 4.03 ton/y. The emissions of perfluorooctanoic acid (PFOA, 1.19 ton/y) and perfluorooctane sulfonate (PFOS, 1.01 ton/y) were dominant. The Monte Carlo simulations suggested that the realistic national emission of Σ<SUB>13</SUB>PFASs is between 2 ton/y and 20 ton/y. Combined WWTPs treating municipal wastewater from residential and commercial areas were identified as a major emission source, contributing 65% to the total PFAS emissions. The Han and Nakdong Rivers were the primary contaminated rivers, receiving 89% of the total PFAS discharge from WWTPs. The results and methodologies in this study can be useful to establish a management policy for PFASs.</P> <P><B>Graphical abstract</B></P> <P>[DISPLAY OMISSION]</P>
Kwon, SooJin,Ki, Soo Mi,Park, Sang Eon,Kim, Min-Jeong,Hyung, Brian,Lee, Na Kyung,Shim, Sangmi,Choi, Byung-Ok,Na, Duk L,Lee, Ji Eun,Chang, Jong Wook Elsevier 2016 Molecular therapy Vol.24 No.9
<P>The role of Wharton's jelly-derived human mesenchymal stem cells (WJ-MSCs) in inhibiting muscle cell death has been elucidated in this study. Apoptosis induced by serum deprivation in mouse skeletal myoblast cell lines (C2C12) was significantly reduced when the cell lines were cocultured with WJ-MSCs. Antibody arrays indicated high levels of chemokine (C motif) ligand (XCL1) secretion by cocultured WJ-MSCs and XCL1 protein treatment resulted in complete inhibition of apoptosis in serum-starved C2C12 cells. Apoptosis of C2C12 cells and loss of differentiated C2C12 myotubes induced by lovastatin, another muscle cell death inducer, was also inhibited by XCL1 treatment. However, XCL1 treatment did not inhibit apoptosis of cell lines other than C2C12. When XCL1-siRNA pretreated WJ-MSCs were cocultured with serum-starved C2C12 cells, apoptosis was not inhibited, thus confirming that XCL1 is a key factor in preventing C2C12 cell apoptosis. We demonstrated the therapeutic effect of XCL1 on the zebrafish myopathy model, generated by knock down of a causative gene ADSSL7. Furthermore, the treatment of XCL1 resulted in significant recovery of the zebrafish skeletal muscle defects. These results suggest that human WJ-MSCs and XCL1 protein may act as promising and novel therapeutic agents for treatment of myopathies and other skeletal muscle diseases.</P>
Analysis of Recent Accidents and Regulating Activities for the Hazardous Materials in Korea
Kwon, Kyung-Ok,Kim, Young-Eun The Korean Institute of GAS 2011 한국가스학회지 Vol.15 No.1
The systems in Korea regarding manufacture, storage, transport and use of hazardous materials are regulated by the related laws and ordinances. The number of accident from hazardous materials has recently decreased but the size of accident has increased according to the hazardous substances are greatly consumed and delivered. The results of analysis showed that most of accidents are caused by human problems and occurred frequently at unauthorized facilities. It is suggested that workers should be trained more and the strict regulation on unauthorized facilities is needed to reduce the accidents caused by hazardous materials.
( Eun-ok Choi ),( Eun-ju Cho ),( Jin-woo Jeong ),( Cheol Park ),( Su-hyun Hong ),( Hye-jin Hwang ),( Sung-kwon Moon ),( Chang Gue Son ),( Wun-jae Kim ),( Yung Hyun Choi ) 한국응용약물학회 2017 Biomolecules & Therapeutics(구 응용약물학회지) Vol.25 No.2
Baicalein, a natural flavonoid obtained from the rhizome of Scutellaria baicalensis Georgi, has been reported to have anticancer activities in several human cancer cell lines. However, its antimetastatic effects and associated mechanisms in melanoma cells have not been extensively studied. The current study examined the effects of baicalein on cell motility and anti-invasive activity using mouse melanoma B16F10 cells. Within the noncytotoxic concentration range, baicalein significantly inhibited the cell motility and invasiveness of B16F10 cells in a concentration-dependent manner. Baicalein also reduced the activity and expression of matrix metalloproteinase (MMP)-2 and -9; however, the levels of tissue inhibitor of metalloproteinase-1 and -2 were concomitantly increased. The inhibitory effects of baicalein on cell motility and invasiveness were found to be associated with its tightening of tight junction (TJ), which was demonstrated by an increase in transepithelial electrical resistance and downregulation of the claudin family of proteins. Additionally, treatment with baicalein markedly reduced the expression levels of lipopolysaccharide-induced phosphorylated Akt and the invasive activity in B16F10 cells. Taken together, these results suggest that baicalein inhibits B16F10 melanoma cell migration and invasion by reducing the expression of MMPs and tightening TJ through the suppression of claudin expression, possibly in association with a suppression of the phosphoinositide 3-kinase/Akt signaling pathway.