• Title/Summary/Keyword: BPES

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Differential Activities of FOXL2 and Its Mutants on SF-1-Induced CYP19 Transcriptional Activation (SF-1을 매개한 CYP19의 전사활성에 미치는 FOXL2 야생형과 돌연변이형의 차별적 영향)

  • Park, Mi-Ra;Kim, Ah-Young;Na, Soon-Young;Kim, Hong-Man;Lee, Kang-Seok;Bae, Jee-Hyeon;Ko, Jeong-Jae
    • Development and Reproduction
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    • v.14 no.2
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    • pp.91-97
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    • 2010
  • FOXL2 is a winged-helix/forkhead (FH) domain transcription factor, and mutations in FOXL2 gene are responsible for blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). BPES is an autosomal dominant genetic disease. BPES type I patients exhibit both premature ovarian failure (POF) and eyelid malformation, while only the eyelid defect is observed in BPES type II. FOXL2-null ovaries showed a blockage of granulosa cell differentiation, suggesting that FOXL2 plays an essential role for proper ovarian folliculogenesis. Previously, we screened for FOXL2-interacting proteins and identified steroidogenic factor-1 (SF-1) which is known to be required for gonad development and transactivates steroidogenic enzymes including CYP19. In the present study, we demonstrated that FOXL2 transactivates CYP19 and stimulated the transcriptional activation of CYP19 induced by SF-1. In contrast, FOXL2 mutants found in BPES type I and II exhibited compromised abilities to enhance CYP19 induction mediated by SF-1. Thus, this study provides a functional difference between wild-type FOXL2 and its mutants which may aid to understand pathophysiology of BPES elicited by FOXL2 mutations.

Paper-Based Bipolar Electrochemistry

  • Renault, Christophe;Scida, Karen;Knust, Kyle N.;Fosdick, Stephen E.;Crooks, Richard M.
    • Journal of Electrochemical Science and Technology
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    • v.4 no.4
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    • pp.146-152
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    • 2013
  • We demonstrate that carbon electrodes screen-printed directly on cellulose paper can be employed to perform bipolar electrochemistry. In addition, an array of 18 screen-printed bipolar electrodes (BPEs) can be simultaneously controlled using a single pair of driving electrodes. The electrochemical state of the BPEs is read-out using electrogenerated chemiluminescence. These results are important because they demonstrate the feasibility of coupling bipolar electrochemistry to microfluidic paperbased analytical devices (${\mu}PADs$) to perform highly multiplexed, low-cost measurements.

Discovery of UBE2I as a Novel Binding Protein of a Premature Ovarian Failure-Related Protein, FOXL2 (조기 난소 부전증 유발 관련 단백질인 FOXL2의 새로운 결합 단백질 UBE2I의 발견)

  • Park, Mira;Jung, Hyun Sook;Kim, Hyun-Lee;Pisarska, Margareta D.;Ha, Hye-Jeong;Lee, Kangseok;Bae, Jeehyeon;Ko, Jeong-Jae
    • Development and Reproduction
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    • v.12 no.3
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    • pp.289-296
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    • 2008
  • BPES (Blepharophimosis/Ptosis/Epicanthus inversus Syndrome) is an autosomal dominant disorder caused by mutations in FOXL2. Affected individuals have premature ovarian failure (POF) in addition to small palpebral fissures, drooping eyelids, and broad nasal bridge. FOXL2 is a member of the forkhead family transcription factors. In FOXL2-deficient ovaries, granulosa cell differentiation dose not progress, leading to arrest of folliculogenesis and oocytes atresia. Using yeast two-hybrid screening of rat ovarian cDNA library with FOXL2 as bait, we found that small ubiquitin-related modifier (SUMO)-conjugating E2 enzyme UBE2I protein interacted with FOXL2 protein. UBE2I also known as UBC9 is an essential protein for processing SUMO modification. Sumoylation is a form of post-translational modification involved in diverse signaling pathways including the regulation of transcriptional activities of many transcriptional factors. In the present study, we confirmed the protein-protein interaction between FOXL2 and UBE2I in human cells, 293T, by in vivo immunoprecipitation. In addition, we generated truncated FOXL2 mutants and identified the region of FOXL2 required for its association with UBE2I using yeast-two hybrid system. Therefore, the identification of UBE2I as an interacting protein of FOXL2 further suggests a presence of novel regulatory mechanism of FOXL2 by sumoylation.

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The Effect of the Demand Forecast on the Energy Mix in the National Electricity Supply and Demand Planning (전력수급계획 수립시 수요예측이 전원혼합에 미치는 영향)

  • Kang, Kyoung-Uk;Ko, Bong-Jin;Chung, Bum-Jin
    • Journal of Energy Engineering
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    • v.18 no.2
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    • pp.114-124
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    • 2009
  • The Ministry of Knowledge and Economy (MKE) establishes the Basic Plan for Long-Term Electricity Supply and Demand(BPE) biannually, a governmental plan for the stable electricity supply. This study investigated the effects of the electric demand forecast on the energy mix. A simplified simulation model was developed, which replaces the WASP program developed by the KPX and verified by comparing both results. Three different electric demand scenarios were devised based upon the 2005 electric demand forecast: Proper, 5 % higher, and 5% lower. The simplified model calculates the energy mix for each scenario of the year 2005. Then it calculates the energy mix for the proper electric demand forecast of the year 2007 using the energy mixes of the three scenarios as the initial conditions, so that it reveals the effect of electric demand forecast of the previous BPE on the energy mix of the next BPE. As the proper electric demand forecasts of the year 2005 and 2007 are the same, there is no change in the previous and the next BPEs. However when the electric demand forecasts were 5% higher in the previous BPE and proper in the next BPE, some of the planned power plant construction in the previous BPE had to be canceled. Similarly, when the electric demand forecasts were 5% lower in the previous BPE and proper in the next BPE, power plant construction should be urgently increased to meet the increased electric demand. As expected the LNG power plants were affected as their construction periods are shorter than coal fired or nuclear power plants. This study concludes that the electric demand forecast is very important and that it has the risk of long term energy mix.