SLC9A6 -related developmental and epileptic encephalopathy with spike-and-wave activation in sleep: A case report

Hye Ri Bae,김영옥 대한의학유전학회 2022 대한의학유전학회지 Vol.19 No.2

The gene encoding solute carrier family 9 member 6 (SLC9A6) on Xq26.3 is associated with Christianson syndrome (CS) mimicking Angelman syndrome. In CS, developmental and epileptic encephalopathy (DEE) appears in about 20%, and DEE with spike-and-wave activation in sleep (SWAS) is reported only in several cases. A 10-year-old boy with DEE showed multi-drug resistant focal seizures from 6 months of age. He had progressive microcephaly, regression, global developmental delay without speech, hyperkinesia, and truncal ataxia; he had a long thin face, esotropia, and happy demeanor. Brain magnetic resonance imaging demonstrated cerebellar atrophy. Electroencephalogram at 7.5 years of age showed nearly continuous dif-fuse paroxysms in slow wave sleep. The seizures were responsive to corticosteroids for a while. Trio whole exome sequencing exhibited a likely pathogenic variant of SLC9A6 in the proband and his asymptomatic mother: c.1194dup (p.Leu399AlafsTer12). This is a rare case report of CS with DEE-SWAS in a Korean patient.

[P10-08] Screening of Antioxidant and AChE Inhibitor from Korean Plants in PC12 Cells

Hye Ri Bae,Mi Jeong Kim,Soo Jung Choi,Hye Kyung Kim,Hong Yon Cho,Bum Shik Hong,Dong-Hoon Shin 한국식품영양과학회 2006 한국식품영양과학회 학술대회발표집 Vol.2006 No.10

독일 정치교육을 통한 한국 시민교육 고찰 : 독일연방정치교육원의 웹페이지 내용분석을 중심으로

배혜리(Bae, Hye Ri),신수빈(Shin, Subin),김영환(KIM, YoungHwan) 학습자중심교과교육학회 2022 학습자중심교과교육연구 Vol.22 No.2

목적 본 연구의 목적은 독일 정치교육의 중심인 연방정치교육원의 정치교육 동향을 파악하고 이에 터해 한국 시민교육에 주는 시사점을 제시하는 것이다. 방법 이를 위해 독일연방정치교육원의 정치교육 전반을 가장 잘 파악할 수 있는 연방정치교육원의 웹페이지를 내용분석했다. 이 사이트는 다양한 형태와 폭넓은 주제의 자료들이 꾸준히 업데이트되고 있어 과거의 간행물뿐만 아니라 최신 기사와 콘텐츠까지 무료로 이용할 수 있으며, 2-3년마다 독일연방정치교육원의 현황과 주요 활동, 주안점 등을 포함한 연간활동 보고서를 발간하고 있다. 결과 독일연방정치교육원은 규범적, 실천적, 합헌적 정치교육을 시행하고 관리하는 중요한 역할을 담당하고, 시민들의 역량을 기르고 정치와 역사, 사회에 관심을 가지고 참여하도록 독려하는 역할을 오랫동안 훌륭하게 수행해오고 있다. 사명이 정치적 이슈에 대한 이해와 민주적 의식을 증진하고 정치적 참여를 위한 태세를 강화하는 것으로 독립적인 운영과 체계적인 활동으로 정파적⋅학술적 균형을 유지하며, 여러 매체와 방법을 이용하여 다양한 사회적 배경과 연령대의 대상에 맞춰 비판적이고 실천적인 역량을 기르기 위한 정치교육 활동을 시행하고 있다. 결론 도출된 시사점은 다음과 같다. 첫째, 시민성의 개발을 위해 체계적이고 다원적인 교육을 이끌어갈 수 있는 시민교육 기관과 체제가 필요하다. 둘째, 시민교육의 대상을 넓히고 민주시민으로서 필요한 실천적 역량을 기를 수 있는 교육내용을 교육대상에 맞게 구성해야 한다. 셋째, 시민의 정치적⋅사회적 참여 능력을 기르기 위해 참여와 실천을 목적으로 하는 교육 활동과 기회를 제공해야 한다. Objectives The purpose of this study is to explore and identify implications on the civic education of Korea based on an analysis of the directionality and trend of German political education. Methods This study analyzes the contents of the website of the Federal Agency for Civic Education(Bundeszentrale für politische Bildung), which provides comprehensive and representative information about the political education of Germany. The website has been updating not only past publications but also latest articles and contents, and regularly publishes annual activity reports every two to three years available for free. Results The Federal Institute of Political Education has long played an important role in implementing and managing normative, practical, and constitutional political education, and encouraging citizens to develop their capabilities and participate with interest in politics, history, and society. Its mission is to promote understanding of political issues and democratic awareness and to strengthen posture for political participation. The institute maintains a political and academic balance through independent operation and systematic activities, and implements political education activities to develop critical and practical capabilities of citizens from various social backgrounds and age groups. Conclusions Following implications can be derived for the civic education of Korea. First, a systematic and multi disciplinary education system is needed. Second, citizens of various statuses and classes should be considered as subjects of civic education and contents that are necessary and suitable for each target group should be organized in educational projects and curriculum. Third, in order to foster citizens political and social participation, educational activities should be practical and provide citizens sufficient opportunities to participate in political and social activities.

HCV : PE-107 ; EGR and WT1 control the differentiation of BM-derived mesenchymal stem cells into hepatic with mesenchymal to epithelial transition

( Hye-lim Kim ),( Jung Hoon Cha ),( Na Ri Park ),( Won Hee Hur ),( Si Hyun Bae ),( Jong Young Choi ),( Seung Kew Yoon ) 대한간학회 2012 춘·추계 학술대회 (KASL) Vol.2012 No.1

Background: Epithelial-to-mesenchymal transition (EMT) drives mammary epithelial cells to de-differentiate into mammary stem cells which are mesenchymal-like. MET and the reverse process, epithelial mesenchymal transition (EMT), both occur in normal tissue, including gastrulating and regenerating tissue. MET in hepatic stem cells is important to multiple processes, including hepatic differentiation, liver development and regeneration. In this study, We investigated regulator of EMT/MET process in the hepatic differentiation. Methods: Based on the 2 step protocol, bone marrow-derived mesenchymal stem cells were cultured for hepatic differentiation. Pas staining and Urea production test were performed to verify the functionality of hepatic differentiation. To search for novel proteins in MSC before and after differentiation, protein/ membrane array was performed. EMT/ MET markers were identified during the hepatic differentiation of MSC by Western blot. Results: During differentiation proceeds, from day 1 to day 28, stem cells change in shape from spindle to polygon with larger in size and tighter intercellular connections. Intracellular glycogen and urea production was significantly increased from day 1 to day 28. The mRNA expression of liver-specific genes was up-regulated. In order to study key regulators in hepatic differentiation, we performed protein/membrane array. Novel factors of EGR1, EGR2 and WT1 were significantly in differentiated MSCs increased during the culture compared to undifferentiated MSCs. The induction of MET markers, E-cadherin and CK18 and the reduction of EMT markers, vimentin and N-cadherin, were exhibited during hepatic differentiation. Conclusions: We identified the novel factors of EGR and WT1 which were expected to play a role in hepatic differentiation. In the future, the acceleration of differentiation by novel factors and EMT/MET process should be studied further.

Basic, Research : EGR1 Control the Differentiation of BM-derived Mesenchymal Stem Cells into Functional Hepatocyte through Mesenchymal-Epithelial Transition

( Hye Lim Kim ),( Si Hyun Bae ),( Jung Hoon Cha ),( Na Ri Park ),( Jong Young Choi ),( Seung Kew Yoon ) 대한간학회 2013 춘·추계 학술대회 (KASL) Vol.2013 No.1

Background: Mesenchymal-Epithelial transition (MET) in hepatic stem cells is important to multiple processes, including hepatogenic differentiation and liver development, regeneration. Early growth response (EGR) 1, transcription factor of early growth response gene family, is induced in the responses to a number of growth and differentiation factors. The aim of this study is to investigate that EGR1 acts as a key regulator of EMT/MET in the process of hepatogenic differentiation from bone marrow-derived mesenchymal stem cells (BM-MSCs). Methods: Pas staining and Urea production test were performed to verify the functionality of hepatogenic differentiation. To search for novel proteins in BM-MSCs before and after differentiation, protein/DNA array was performed. Immunohistochemistry were identified the enhanced expression of EGR1 and MET markers, accompanied by decreased expression of EMT markers. To determine the effect of EGR1, BMMSCs were infected by lenti-shEGR1. To determine the role of EGR1 in BM-MSCs which were infected by lenti-shEGR1, MET/EMT markers during the hepatogenic differentiation were identified by immunoblotting, immuno- fluorescence. Results: During differentiation proceeds, from day 1 to day 28, stem cells change in shape from spindle to polygon with larger size and tighter intercellular connections. Intracellular glycogen and urea production was significantly increased from day 1 to day 28. The mRNA expression of liver-specific genes was upregulated. In order to study key regulator in hepatogenic differentiation, we performed protein/DNA array. EGR1 in differentiated BM-MSCs were significantly increased during the culture compared to undifferentiated BM-MSCs. In contrast, downregulation of EGR1 by lenti-shRNA reduced hepatogenic differentiation and increased the expression of EMT related genes in associaton with the decreased expression of MET related gene. Conclusions: In this study, we identified novel factors in the process of hepatogenic differentiation through inducing the MET process. Our study suggests that EGR1 were expected to the “transcription factor therapy” as novel strategy in future clinical treatment.

Basic, HCC basic : PE-107 ; EGR and WT1 control the differentiation of BM-derived mesenchymal stem cells into hepatic with mesenchymal to epithelial transition

( Hye Lim Kim ),( Jung Hoon Cha ),( Na Ri Park ),( Won Hee Hur ),( Si Hyun Bae ),( Jong Young Choi ),( Seung Kew Yoon ) 대한간학회 2012 춘·추계 학술대회 (KASL) Vol.2012 No.-

Background: Epithelial-to-mesenchymal transition drives mammary epithelial cells to de-differentiate into mammary stem cells which are mesenchymal-like. MET and the reverse process, epithelial mesenchymal transition (EMT), both occur in normal tissue, including gastrulating and regenerating tissue. MET in hepatic stem cells is important to multiple processes, including hepatic differentiation, liver development and regeneration. In this study, We investigated regulator of EMT/MET process in the hepatic differentiation. Methods: Based on the 2 step protocol, bone marrow-derived mesenchymal stem cells were cultured for hepatic differentiation. Pas staining and Urea production test were performed to verify the functionality of hepatic differentiation. To search for novel proteins in MSC before and after differentiation, protein/ membrane array was performed. EMT/ MET markers were identified during the hepatic differentiation of MSC by Western blot. Results: During differentiation proceeds, from day 1 to day 28, stem cells change in shape from spindle to polygon with larger in size and tighter intercellular connections. Intracellular glycogen and urea production was significantly increased from day 1 to day 28. The mRNA expression of liver-specific genes was up-regulated. In order to study key regulators in hepatic differentiation, we performed protein/membrane array. Novel factors of EGR1, EGR2 and WT1 were significantly in differentiated MSCs increased during the culture compared to undifferentiated MSCs. The induction of MET markers, E-cadherin and CK18 and the reduction of EMT markers, vimentin and N-cadherin, were exhibited during hepatic differentiation. Conclusions: We identified the novel factors of EGR and WT1 which were expected to play a role in hepatic differentiation. In the future, the acceleration of differentiation by novel factors and EMT/MET process should be studied further.

SSD1과 상호작용하는 유전자

성혜란, 배윤진, 송나리, 이종길, 송석길 충북대학교 약품자원개발연구소 2006 약학논문집 Vol.21 No.-

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Anticoagulant and Antiplatelet Activities of Artemisia princeps Pampanini and Its Bioactive Components

Ri Ryu,Un Ju Jung,Hye-jin Kim,Wonhwa Lee,Jong-Sup Bae,Yong Bok Park,Myung-Sook Choi 한국식품영양과학회 2013 Preventive Nutrition and Food Science Vol.18 No.3

Artemisia princeps Pampanini (AP) has been used as a traditional medicine in Korea, China and Japan and reported to exhibit various beneficial biological effects including anti-inflammatory, antioxidant, anti-atherogenic and lipid lowering activities; however, its antiplatelet and anticoagulant properties have not been studied. In the present study, we evaluated the effects of an ethanol extract of Artemisia princeps Pampanini (EAP) and its major flavonoids, eupatilin and jaceosidin, on platelet aggregation and coagulation. To determine the antiplatelet activity, arachidonic acid (AA)-, collagen-and ADP (adenosine diphosphate)-induced platelet aggregation were examined along with serotonin and thromboxane A₂ (TXA₂) generation in vitro. The anticoagulant activity was determined by monitoring the activated partial thromboplastin time (aPTT) and prothrombin time (PT) in vitro. The data showed that EAP and its major flavonoids, eupatilin and jaceosidin, significantly reduced AA-induced platelet aggregation and the generation of serotonin and TXA₂, although no significant change in platelet aggregation induced by collagen and ADP was observed. Moreover, EAP significantly prolonged the PT and aPTT. The PT and/or aPTT were significantly increased in the presence of eupatilin and jaceosidin. Thus, these results suggest that EAP may have the potential to prevent or improve thrombosis by inhibiting platelet activation and blood coagulation.

Roll-to-roll production of 30-inch graphene films for transparent electrodes

Bae, Sukang,Kim, Hyeongkeun,Lee, Youngbin,Xu, Xiangfan,Park, Jae-Sung,Zheng, Yi,Balakrishnan, Jayakumar,Lei, Tian,Ri Kim, Hye,Song, Young Il,Kim, Young-Jin,Kim, Kwang S.,Ö,zyilmaz, Barbaros,Ahn, J Springer Science and Business Media LLC 2010 Nature nanotechnology Vol.5 No.8

Mechanical and Biological Characteristics of Reinforced 3D Printing Filament Composites with Agricultural By-product

( Hye-been Kim ),( Yu-ri Seo ),( Kyeong-je Chang ),( Sang-bae Park ),( Hoon Seonwoo ),( Jin-woo Kim ),( Jangho Kim ),( Ki-taek Lim ) 한국산업식품공학회 2017 산업 식품공학 Vol.21 No.3

Scaffolds of cell substrates are biophysical platforms for cell attachment, proliferation, and differentiation. They ultimately play a leading-edge role in the regeneration of tissues. Recent studies have shown the potential of bioactive scaffolds (i.e., osteo-inductive) through 3D printing. In this study, rice bran-derived biocomposite was fabricated for fused deposition modeling (FDM)-based 3D printing as a potential bone-graft analogue. Rice bran by-product was blended with poly caprolactone (PCL), a synthetic commercial biodegradable polymer. An extruder with extrusion process molding was adopted to manufacture the newly blended “green material.” Processing conditions affected the performance of these blends. Bio-filament composite was characterized using field emission scanning electron microscopy (FE-SEM) and energy dispersive X-ray spectroscopy (EDX). Mechanical characterization of bio-filament composite was carried out to determine stress-strain and compressive strength. Biological behaviors of bio-filament composites were also investigated by assessing cell cytotoxicity and water contact angle. EDX results of bio-filament composites indicated the presence of organic compounds. These bio-filament composites were found to have higher tensile strength than conventional PCL filament. They exhibited positive response in cytotoxicity. Biological analysis revealed better compatibility of r-PCL with rice bran. Such rice bran blended bio-filament composite was found to have higher elongation and strength compared to control PCL.