Human-to-mouse prion-like propagation of mutant huntingtin protein

Jeon, Iksoo,Cicchetti, Francesca,Cisbani, Giulia,Lee, Suji,Li, Endan,Bae, Jiwoo,Lee, Nayeon,Li, Ling,Im, Wooseok,Kim, Manho,Kim, Hyun Sook,Oh, Seung-Hun,Kim, Tae-Aug,Ko, Jung Jae,Aubé,, Benoit,O Springer Berlin Heidelberg 2016 Acta neuropathologica Vol.132 No.4

<P>Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder of the central nervous system (CNS) that is defined by a CAG expansion in exon 1 of the huntingtin gene leading to the production of mutant huntingtin (mHtt). To date, the disease pathophysiology has been thought to be primarily driven by cell-autonomous mechanisms, but, here, we demonstrate that fibroblasts derived from HD patients carrying either 72, 143 and 180 CAG repeats as well as induced pluripotent stem cells (iPSCs) also characterized by 143 CAG repeats can transmit protein aggregates to genetically unrelated and healthy host tissue following implantation into the cerebral ventricles of neonatal mice in a non-cell-autonomous fashion. Transmitted mHtt aggregates gave rise to both motor and cognitive impairments, loss of striatal medium spiny neurons, increased inflammation and gliosis in associated brain regions, thereby recapitulating the behavioural and pathological phenotypes which characterizes HD. In addition, both in vitro work using co-cultures of mouse neural stem cells with 143 CAG fibroblasts and the SH-SY5Y human neuroblastoma cell line as well as in vivo experiments conducted in newborn wild-type mice suggest that exosomes can cargo mHtt between cells triggering the manifestation of HD-related behaviour and pathology. This is the first evidence of human-to-mouse prion-like propagation of mHtt in the mammalian brain; a finding which will help unravel the molecular bases of HD pathology as well as to lead to the development of a whole new range of therapies for neurodegenerative diseases of the CNS.</P><P><B>Electronic supplementary material</B></P><P>The online version of this article (doi:10.1007/s00401-016-1582-9) contains supplementary material, which is available to authorized users.</P>

고전압 BCDMOS 공정을 이용한 조명용 Single-Chip LED Driver

전익수(Iksoo Jeon),김병용(Byeongyong Kim),박시홍(Shihong Park) 대한전기학회 2013 대한전기학회 학술대회 논문집 Vol.2013 No.4

Iconicity and conceptual metaphor in military hand signals

( Iksoo Kwon ),( Jung Eun Lee ),( Jinree Jeon ),( Young Eun Park ) 경희대학교 언어연구소 2016 언어연구 Vol.33 No.2

This paper explores military hand signals as a type of non-verbal language modality. We show that this type of sign language involves a construal process that relies on, first, iconic, and second, metaphoric relationships between the thing signed and the gesture. Although they are, unlike primary sign language, used among non-deaf people with very limited inventory and involve very little reciprocity, military hand signals still provide another indicator of human cognition and conceptualization based on iconicity and conceptual metaphor. This paper thoroughly discusses a few hand signal examples from a US Army ROTC field manual and non-fiction military movie/TV show clips and provides systematic explanations of how iconicity and metaphor guide interlocutors to grasp the meanings conveyed by the signals as a non-verbal modality. Based on the analyses, this paper addresses characteristics of military hand signals especially on their form-meaning relationship and their status in the typology of gestures in general. (Hankuk University of Foreign Studies) 5

3차원 개별요소해석 시의 초기 모델 및 재료 스케일 영향

전제성(Jeon Jesung),신동훈(Shin Donghoon),하익수(Ha Iksoo) 한국지반환경공학회 2011 한국지반환경공학회논문집 Vol.12 No.7

본 연구에서는 3차원 개별요소해석 코드인 Particle Flow Code, PFC<SUP>3D</SUP>(Itasca)를 이용, 조립재료의 실내 삼축압축시험에 대한 개별요소 수치 모델링을 수행하였으며, 해석 모델과 개별요소를 대상으로 다양한 상사 조건에 대한 개별요소 수치 모델링을 수행, 그 결과를 통해 각각의 스케일 조건이 최종 수치 모델링 결과에 미치는 영향을 분석하였다. 3차원 개별요소 수치 모델링은 기존 2차원 모델링 대비 별도의 간극률 환산 없이 정확한 초기 조건 구현이 가능했으며, 응력-변형 및 체적변화 거동, 강도정수 등에 있어 실내시험 결과와 유사한 수치 해석적 예측이 가능하였다. 해석 모델과 개별요소에 대한 다양한 상사비 조건별 수치 모델링 결과, 3차원 해석 시의 안정적 예측결과 도출 및 수치 시험실 활용에 대한 적정성을 확보하되, 해석시간 단축 및 해석 효율성 확보를 위해서는 해석 모델과 개별요소에 대한 적정 상사비 결정이 필요함을 알 수 있었다. 해석 모델의 크기와 개별요소의 입경크기를 변화시켜 개별요소 수치모델링을 수행한 결과, 대부분의 경우 전체적인 응력-변형 거동에 차이가 발생하였지만, 점착력과 내부마찰각의 강도정수는 Dmod/Dgmax < 10 조건에 유사한 결과를 보였으며, 개별요소 방법이 수치 시험실 기법을 이용한 강도정수산정에 효과적으로 적용될 수 있음을 확인할 수 있었다. Numerical simulations by three-dimensional Particle Flow Code(PFC<SUP>3D</SUP>, Itasca) considering distinct element method (DEM) were carried out for prediction of triaxial compression test with sand material. The effect of scale conditions for numerical model and distinct material on final prediction results was analyzed by numerical models under various scale conditions, and following observations were made from the numerical experiments. It is very useful to model the initial material condition without any porosity conversion from 2-D to 3-D DEM. Numerical experiments have shown that in all cases considered, 3D distinct element modeling could provide good agreement on stress-strain behavior, volume change and strength properties with laboratory testing results. It was important thing to assess reasonable scale ratio of numerical model and distinct elements for saving calculation time and securing calculation efficiency under condition with accuracy and appropriateness as numerical laboratory. As results of DEM simulations under various scale conditions, most of results show that shear strength properties as cohesion and internal friction angle are similar in condition of Dmod/Dgmax < 10. It shows that 3-D distinct element method could be used as efficient tool to assess strength properties by numerical laboratory technique.

CDMA 디지틀 셀룰라 시스템에서의 비터비 복호기 성능 분석

전인산(Insan Jeon),어익수(Iksoo Eo),임인기(Ingi Lim),연광일(Kwangil Yeon),김재석(Jaeseok Kim),이훈복(Hunbok Lee) 한국통신학회 1994 한국통신학회 학술대회논문집 Vol.1994 No.11

CDMA 단말기 모델 ASIC 설계

연광일(Kwangil Yeon),어익수(Iksoo Eo),전인산(Insan Jeon),임인기(Ingi Lim),김재석(Jaeseok Kim),이훈복(Hoonbok Lee) 대한전자공학회 1994 대한전자공학회 학술대회 Vol.1994 No.11

토종닭 종계 9개 조합의 체중 및 산란능력 비교 연구

김기곤(Kigon Kim),박병호(Byoungho Park),전익수(Iksoo Jeon),추효준(Hyojun Choo),차재범(Jaebeom Cha) 한국가금학회 2021 韓國家禽學會誌 Vol.48 No.4

본 연구는 토종닭 종계의 산란능력 검증을 위해 국립축산과학원 가금연구소의 토종닭 순계로 생산한 종계 9개 조합의 생산능력을 비교 및 분석하였다. 분석 결과, 16주 체중은 CY조합이 1,604.9 g으로 가장 높았고 DK 조합이 1,424.4 g으로 가장 낮았다. 종계 9개 조합의 시산일령은 118.6일로 나타났고 YD 조합이 111.6일로 가장 빠르고 DK 조합이 126일로 가장 느린 시산을 보였다. 일계 산란율은 CF, CK 및 DK 조합이 74% 이상으로 높게 나타났다. DK 조합은 시산일령이 느려 일계산란수가 114.4개로 낮았으나 높은 일계 산란율을 보여 다른 조합에 비해 산란지속성이 우수할 것으로 사료된다. 난중은 DK 조합이 20주령 43.9 g, 36주령 58.1g으로 모든 주령에서 월등히 무겁게 나타났다. 난질에서 난각색은 F와 K가 사용된 조합이 밝은 난각색을 보였고, 계란무게 형질은 DK와 CF 조합이 무겁게 나타났다. 난각 강도와 난각 두께는 DK 조합이 각각 3.8 kg/cm2, 0.38 mm로 우수하였다. 종합하면, CF 와 DK 조합이 산란능력 및 계란품질이 우수하게 나타났으며 부계로 Y 계통을 사용한 YC, YD 및 YK 조합은 대부분 형질의 성적이 저조하게 나타났다. The present study investigated production traits, including body weight, egg production, egg weight, and egg quality in Korean indigenous parent stocks. Parent stocks produced from a nine-combination association from five pure line strains (C, D, F, K, and Y) were used. The body weight analyses results showed that the CY combination had the highest body weight (1,604.9 g) and the DK combination had the lowest (1,424.4 g). The average age at first egg of the nine combinations was 118.6 days; however, that of the YD combination was 111.6 days, making it the fastest growing combination, whereas the DK combination was the slowest, at 126 days. Hen-day egg production was 74% or more for the CF, CK, and DK combinations. The DK combination hens showed excellent persistence in egg laying. Hen-housed egg production exhibited results similar to those of hen-day egg production. Egg weight was significantly higher in the DK combination than in the other combinations and was observed to increase from 20 (43.9 g) to 40 (58.1 g) weeks. The egg quality analyses results showed that the combinations based on the F and K strains had a bright eggshell color, with relatively high egg weights. Eggshell strength and thickness were the highest in the DK combination (3.8 kg/cm², 0.38 mm). In summary, the CF and DK combinations showed excellent egg production ability and egg quality, while the YC, YD, and YK combinations with the Y strain as the paternal strain exhibited poor performance.

미국 대선 후보 공개토론회에 나타난 손짓 언어

이정은(Lee, Jung-Eun),전진리(Jinree Jeon),박영은(Young-Eun Park),권익수(Iksoo Kwon) 담화·인지언어학회 2016 담화와 인지 Vol.23 No.2

This paper aims to explore metaphoric co-speech gestures of candidates in the U.S. Presidential Debate in 2015. We collected data from four full video clips of the U.S. Republican and Democratic presidential debates, and focused especially on significant types of gestures whose construals rely on the speaker’s metaphorical conceptualization of linguistic contents. The metaphors that this paper focuses on are ASSEMBLIES ARE CIRCLES, IDEAS ARE OBJECTS, and ACTIONS ARE MOTIONS. These analyses provide systematic explanations of how conceptual metaphors motivate interlocutors to represent and grasp meanings conveyed by the non-verbal modalities.

토종닭 순계 12계통과 성별에 따른 성장능력 비교 연구

김기곤(Kigon Kim),박병호(Byoungho Park),전익수(Iksoo Jeon),추효준(Hyojun Choo),함진주(Jinjoo Ham),박건(Keon Park),차재범(Jaebeom Cha) 한국가금학회 2021 韓國家禽學會誌 Vol.48 No.4

이 연구는 국립축산과학원 가금연구소에서 보유하고 있는 토종닭 순계 12계통의 육성기 성장능력을 파악하기 위해 생시(0주령)부터 16주령까지 2주 간격으로 체중, 증체량을 측정하고, 성장곡선을 추정하여 제시하였다. 각 주령별 체중에 대한 성별 효과와 계통 효과는 전 기간에서 각각 유의적인 차이를 보였으며 전 기간 체중은 수컷이 암컷보다 유의적으로 무겁게 나타났다. 계통별 체중 차이는 코니쉬 품종과 그 외 품종으로 구분되어 나타났고, 코니쉬 품종이 생시 체중을 제외하고 다른 품종에 비해 약 2배 정도 체중이 무거운 경향을 보였다. 증체량의 경우 생시부터 6주령까지 증체량이 빠르게 증가하는 경향과 이후 피크에 도달하고 12주령부터 14주령까지 기간에 증체량이 감소하는 경향은 모든 성별과 계통에서 공통적으로 나타났다. 그러나 성별과 계통에 따라 증체량의 피크 도달 시점과 횟수의 차이를 보였다. 주령별 체중 간 상관분석 결과, 토종닭 시장 출하 주령인 10주령과 8주령 체중에서 가장 높은 표현형 상관계수를 나타났다. 증체량과 증체량 간의 상관분석 결과는 0-2주령 증체량과 2~4주령 증체량 간 표현형 상관계수를 제외하고 —0.16에서 0.29로 낮은 표현형 상관을 보였다. 성장곡선 모형의 결정계수와 수정된 결정계수는 99.1∼99.9로 계통과 성별에 상관없이 모두 높은 적합도를 나타냈다. 그러나 각 모형별 적합도는 성별과 계통에 따라 차이가 나타냈다. 그러나 각 모형별 적합도는 성별과 계통에 따라 차이가 나타났다. 암컷의 성장 곡선은 D계통을 제외한 모든 계통에서 Von Betalanffy 모형이 가장 높은 적합도를 보였다. 반면에 수컷의 성장 곡선은 C계통을 제외한 모든 계통에서 Gompertz 모형이 가장 높은 적합도를 보였다. Logistic 모형은 모든 성별과 계통에서 모든 모형 중 가장 낮은 모형 적합도를 보였다. 성숙체중(α)의 경우 모든 성별과 계통에서 Von Bertalanffy, Gompertz, Logistic 모형 순으로 높게 나타났으며, 성장비(β)와 성숙률(γ)은 Logistic, Gompertz, Von Bertalanffy 모형 순으로 높게 나타났다. 성장곡선 모수인 α, β, γ는 암컷보다 수컷에서 높게 나타나는 경향을 보였다. 이 연구에서 수행한 가금연구소 보유 토종닭 순계 12계통의 육성기 성장 특성은 향후 토종닭 종계, 실용계를 생산하기 위한 교배조합 시험 설계와 순계의 개량방향, 그리고 사료 제한급이를 위한 기초자료로 활용될 것이다. This study aimed to identify the growth performance of Korean indigenous chicken pure-line by sex and twelve strains conserved in Poultry Research Institute, National Institute of Animal Science, Rural Development Administration. The effect of sex and strain on body weight was significantly different in every period, with males being heavier in all periods than females. In the case of biweekly weight gain, the tendency to increase rapidly from birth to six weeks old, and to decrease in the period from twelve to fourteen weeks old was common across all sex and strains. Depending on sex and strain, there were significant differences in age and the number of peaks. Regardless of sex and strain, the determination coefficient and adjusted determination coefficient showed high goodness of fit (99.1∼99.9%) to growth functions. However, for each model, the goodness-of-fit had variations by sex and strains. von Betalanffy function had the best fit to growth curves in all the female strains except strain D. On the other hand, Gompertz function had the best fit for all the male strains except strain C. Logistic function showed the lowest goodness-of-fit in all sex and strains. Mature weights were in the order of von bertalanffy, Gompertz, and Logistic models, while growth ratio and maturing rate followed the order of logistic, gompertz, and von bertalanffy functions. This information could be useful for Korean indigenous chicken management and designing crossbreeding tests and breeding programs.

PI3K/Akt and Stat3 signaling regulated by PTEN control of the cancer stem cell population, proliferation and senescence in a glioblastoma cell line

MOON, SEOK-HO,KIM, DAE-KWAN,CHA, YOUNG,JEON, IKSOO,SONG, JIHWAN,PARK, KYUNG-SOON Spandidos Publications 2013 International journal of oncology Vol.42 No.3

<P>Malignant gliomas are the most common primary brain tumor in adults. A number of genes have been implicated in glioblastoma including mutation and deletion of PTEN. PTEN is a regulator of PI3K-mediated Akt signaling pathways and has been recognized as a therapeutic target in glioblastoma. To achieve potent therapeutic inhibition of the PI3K-Akt pathway in glioblastoma, it is essential to understand the interplay between the regulators of its activation. Here, ectopic expression of PTEN in the U-87MG human glioblastoma-astrocytoma cell line is shown to result in the depletion of glioblastoma stem cells (GSCs) and to cause growth retardation and senescence. These effects are likely to be associated with PTEN-mediated cooperative perturbation of Akt and Stat3 signals. Using an in vivo rat model of glioblastoma, we showed that PTEN-overexpressing U-87MG cells failed to induce tumor formation, while untreated U-87MG cells did so. Furthermore, cells expressing the phosphorylated form of Stat3 were completely absent from the brain of rats implanted with PTEN-overexpressing U-87MG cells. Based on these results, PTEN appears to function as a crucial inhibitor of GSCs and as an inducer of senescence, suggesting that functional enhancement of the PTEN pathway will be useful to provide a therapeutic strategy for targeting glioblastoma.</P>