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A Novel COL4A1 Mutation in a Neonate with Intrauterine Intraventricular Hemorrhage and Porencephaly
Jihyun Noh,Euiseok Jung,Ah Young Jung,Beom Hee Lee,Byong Sop Lee,Ellen Ai-Rhan Kim,Ki-Soo Kim 대한신생아학회 2020 Neonatal medicine Vol.27 No.1
Collagen type IV alpha 1 (COL4A1) plays an important role in construction of the basement membranes of all human tissues, especially vessels. Mutations in COL4A1 lead to various multisystemic dysfunctions, including hereditary porencephaly, hemorrhagic stroke, hemiplegia, cerebral small vessel disease, and nephropathy. In this study, we describe a neonatal case featuring a novel de novo COL4A1 mutation, manifesting as fetal intraventricular hemorrhage and porencephaly. This patient is one of the youngest to have been diagnosed with the most severe phenotype. Our experience may assist clinicians in the diagnosis and management of this extremely rare genetic condition.
Noh, Jihyun,Lee, Eun-sung,Chung, Jun-mo Blackwell Publishing Ltd 2009 Journal of Neurochemistry Vol.109 No.5
<P>Abstract</P><P>Neu2000 [NEU, 2-hydroxy-5-(2,3,5,6-tetrafluoro-4-trifluoromethyl-benzylamino)-benzoic acid], a derivative of sulfasalazine, attenuates NMDA-induced neuronal toxicity. Here we investigated the effects of NEU on the NMDA receptor (NMDAR) using whole-cell patch clamp technique to determine the molecular mechanisms underlying its neuroprotective role. NEU reversibly suppressed NMDA responses in an uncompetitive manner with fast binding kinetics. Its inhibition of NMDAR activity depended on both the concentration and the use of agonist but not on the membrane potential. NEU accelerated NMDA desensitization without affecting the binding affinity of NMDAR for its agonists and stabilized the closed state of NMDAR. Therefore, NEU should effectively alleviate disorders that are a result of glutamate excitoxicity with fewer side effects because it is a low-affinity gating modifier that antagonizes NMDAR in an uncompetitive manner. Moreover, in the presence of ifenprodil (an NR2B antagonist) but not NVP-AAM077 [(<I>R</I>)-[(<I>S</I>)-1-(4-bromo-phenyl)-ethylamino]-(2,3-dioxo-1,2,3,4-tetrahydro-quinoxalin-5-yl)-methyl]-phosphonic acid, an NR2A antagonist], the extent of NEU block was decreased, suggesting that NEU is an NR2B-specific antagonist.</P>
Functional group effects on a metal-organic framework catalyst for CO<sub>2</sub> cycloaddition
Noh, Jinmi,Kim, Youngik,Park, Hyojin,Lee, Jihyun,Yoon, Minyoung,Park, Myung Hwan,Kim, Youngjo,Kim, Min THE KOREAN SOCIETY OF INDUSTRIAL AND ENGINEERING 2018 JOURNAL OF INDUSTRIAL AND ENGINEERING CHEMISTRY -S Vol.64 No.-
<P><B>Abstract</B></P> <P>A variety of metal-organic frameworks (MOFs) have been reported as efficient catalysts for CO<SUB>2</SUB> fixation reactions, such as cycloaddition to cyclic carbonates. The permanent porosity of the frameworks and the Lewis acidity of the MOF metal sites have been considered as the major contributors to the catalytic activity in the cycloaddition of CO<SUB>2</SUB>. In this study, we have, instead, focused on the effects of the organic functional groups for effective catalytic ability. A total of eight different functionalized Zr-based MOFs were tested. It was revealed that the non-functionalized pristine UiO-66 MOF (UiO=University of Oslo) showed the best conversion at low temperature (77% at 50°C), whereas the hydroxy-functionalized UiO-66-OH MOF displayed the best conversion at high temperature (91% at 140°C). The Zr-MOF could be recycled up to four times without a significant decrease in the reactivity.</P> <P><B>Highlights</B></P> <P> <UL> <LI> The organic functional group effects on MOF-catalyzed CO<SUB>2</SUB> cycloaddition have been examined. </LI> <LI> Non-functionalized Zr-based MOF, UiO-66 showed the best catalytic activity at 50°C. </LI> <LI> The hydroxy-functionalized UiO-66-OH performed cyclic organic carbonate synthesis efficiently at 140°C. </LI> <LI> Various epoxides were successfully converted to cyclic organic carbonates, and the Zr-MOF catalyst could be recycled by simple centrifugation. </LI> </UL> </P> <P><B>Graphical abstract</B></P> <P>[DISPLAY OMISSION]</P>
순천만 IT 융합형 콘텐츠 개발과 고등학생 대상 진로 캠프 운영
윤지현(Jihyun Yoon),전영국(Youngcook Jun),노광흔(Kwangheun Noh) 한국컴퓨터교육학회 2021 한국컴퓨터교육학회 학술발표대회논문집 Vol.25 No.2(A)
이 연구는 대학생들이 IT를 사용하여 순천만의 다양한 주제와 접목하는 융합형 프로젝트를 수행하는데 관련되는 콘텐츠를 개발한 후에 고등학생 대상으로 진로 캠프에 적용해 보는 사례를 다루었다. 순천만의 정원에 있는 꽃을 모티브로 하여 아두이노 보드를 사용하여 움직이면서 조명으로 사용할 수 있는 콘텐츠를 제작하였다. 이를 토대로 K시의 J 고등학교에 재학 중인 1-2학년 학생 26명이 2020년 12월에 2주에 걸쳐 움직이는 꽃을 주제로 한 코딩 및 진로 캠프 프로그램에 참여하였다. 고등학생들은 먼저 IT 분야에서 진행되는 진로 동향을 소개 받고 순천만 정원의 꽃과 관련되는 아이디어를 디자인 한 후에 아두이노 보드를 사용하여 구현해 보는 일련의 활동을 전개하였다. 설문지 자료를 분석하여 만족도 조사를 하였으며 향후에 진로 캠프에서 보완해야 할 부분을 도출하였다.
노영숙(Noh, Yeongsuk),장지현(Jang, Jihyun) 충북대학교 국제개발연구소 2018 사회적경제와 정책연구 Vol.8 No.1
본 연구는 충북지역을 사례로 지역사회에 기반한 사회적기업의 딜레마를 연구하였다. 본 연구를 위한 자료는 한국사회적기업진흥원, 충북도청등에서 생산한 자료를 분석 · 활용하였다. 충북지역의 사회적기업의 유형은 일자리제공형 58개(69.0%), 기타형 10개(11.9%), 혼합형 9개(10.7%), 사회서비스제공형 4개(4.8%), 지역사회공헌형 3개(3.6%)로 파악되었으며, 서비스분야는 청소, 환경, 복지 분야 중심이었다. 연구결과, 사회적기업의 딜레마 및 타개책을 정리하면 다음과 같다. 첫째, 사회적기업의 서비스분야가 특정업종에 한정되어 있어, 지역민을 동참시킬 수 있는 업종으로 다양화할 필요가 있다. 둘째, 정부지원을 받기 위한 수단으로 관련제도를 이용하고 있는데, 사회적 가치를 공유하는 것에 중점을 두는 인식전환이 필요하다. 셋째, 자본의 영세성 및 인적 구성의 제한 등 내재적 한계성이 있기 때문에 정부지원기간 중 경제적·사회적 인프라를 구축해야 지속적인 영업활동이 가능하다. 넷째, 사회적기업이 지속적으로 유지 · 발전될 수 있도록 엄격한 심사, 운영상 지도, 편법 운영시 행정제재 등을 강화할 필요가 있다. In this study, we studied the dilemma of social enterprises based on the community, in the case of the Chungbuk area. The material for this research analyzed and utilized materials produced at Korea Social Enterprise Promotion Agency, Chungcheongbuk-do and others. Types of social enterprises in Chungcheongbuk-do area are 58 offer types (69.0%), 10 other types (11.9%), 9 mixed types (10.7%), 4 social services type (4.8%), Three community contribution types (3.6%), and the service field was mainly in the clean, environmental, and welfare fields. The results of the research, the dilemma of social enterprises and the breakthrough plan are summarized as follows. First, the service field of social enterprises is limited to specific industries, and it is necessary to diversify to the industries where local residents can participate. Secondly, although we use related systems as a means to get government support, it is necessary to change the recognition which focuses on sharing social value. Third, since there is an implicit limit such as limitation of human capital and restrictions on capital, continuous marketing activities are possible while building the economic and social infrastructure during the government`s support period. Fourth, it is necessary to strengthen strict examination, operational maps, shortcut management city administration sanctions, etc. so that social enterprises can be sustainably maintained and developed.
BMB Reports : Targeted disruption of EBNA1 in EBV-infected cells attenuated cell growth
( Ka Won Noh ),( Jihyun Park ),( Myung Soo Kang ) 생화학분자생물학회(구 한국생화학분자생물학회) 2016 BMB Reports Vol.49 No.4
Epstein Barr virus (EBV)-encoded nuclear antigen-1 (EBNA1) plays a pivotal in an EBV episome replication and persistence. Despite considerable attempts, there are no EBV drugs or vaccines. We attempted to eradicate EBV episomes by targeting EBNA1 using the transcription activator-like effector nucleases (TALEN) (E1TN). E1TN-mediated transient knockout (KO) of EBNA1 reduced EBNA1 expression, and caused significant loss of EBV genomes and progressive death of EBV-infected cells. Furthermore, when a mixture of EBV-infected Burkitt`s lymphoma (BL) cells and EBV-negative BL cells was targeted by E1TN, EBV-negative cells were counter-selected while most EBV-infected cells died, further substantiating that EBNA1 KO caused selective death of EBV-infected cells. TALEN-mediated transient targeting of EBNA1 attenuated the growth of EBV-infected cells, implicating a possible therapeutic application of E1TN for EBV-associated disorders. [BMB Reports 2016; 49(4): 226-231]